USMLE Step 2 CK Board Preparation: Diseases of the Blood

A healthy couple presents for an evaluation before traveling to the Dominican Republic; they leave in 1 month and will stay for 4 weeks. The CDC lists the Dominican Republic as a malaria-endemic area, but it is not considered to have a resistant strain of Plasmodium falciparum. What is the best advice for this couple?

"You should both take chloroquine weekly starting 1 week before travel."

72-year-old woman presents with lower back pain and headaches that have progressively worsened over the last 6 months. She has recently noticed that she is more easily fatigued. Aside from tenderness in the lumbar spine, her physical exam is unremarkable. X-rays of the spine reveal scattered lytic lesions throughout the vertebrae, and a skull X-ray reveals a diffuse, "moth-eaten" appearance. Results from a blood count reveal a moderate, normocytic anemia; other values are within normal limits. The laboratory reports the presence of rouleaux in the peripheral blood smear. What test would be most helpful in establishing the diagnosis?

A serum protein electrophoresis

42-year-old man presents with a 3-week history of headache, dizziness, and blurring of vision. He also notes increased tiredness and fatigue. Further questioning reveals that the patient has generalized pruritus, which is made worse after a hot shower. Physical examination reveals the following vital signs: temp. 36.9 C, BP 130/90 mmHg, pulse 78/min, and resp. 15/min. The patient is a well-built individual with a ruddy complexion; there is no scleral icterus and no lymphadenopathy. Abdominal examination reveals splenomegaly 3 inches below the left costal margin. Breath and heart sounds are normal. The patient does not smoke. Laboratory examination of the blood included the following tests and results: Pa O2: 98 mmHg Hematocrit: 61% WBC count: 13,000/microliter Platelet count: 800,000/microliter RBC count: 6,000,000/microliter with normal morphology Uric acid: 10.7 mg/dl Leukocyte alkaline phosphatase: Elevated If the erythropoietin level were found to be elevated, what would be the next step in management?

Abdominal CT scan

A 23-year-old man presents with history of progressive fatigue; it has been increasing over the past 3 months. He feels breathless on minimal exertion. His weight registers at 140 pounds; previously, he had been steady at 189 pounds. His reduced appetite has also been worrying him. On examination, he was found to have pallor, multiple petechiae, and ecchymosis, with diffusely swollen gums. Lab investigations reveal the following: Hemoglobin - 8g/dl Total white count - 75,000/cubic ml Platelet count - 25,000/cubic ml Peripheral Smear - myeloblasts >20% with peroxidase positive Auer rods He is diagnosed with AML, with t(15;17) M3 subtype. The main treatment modality that can be used for the patient's condition is also used for treating what other condition?

Acne

63-year-old man presents with bleeding gums and a general feeling of malaise. He states that he has been running a low-grade fever for the past few days, feels fatigued, and has bone and joint pain. He thought that he had the flu, but he became concerned when his gums began to bleed. Physical examination reveals a pale individual, mild petechiae on the extremities, gingival hyperplasia, and a palpable spleen. A CBC reveals pancytopenia and blasts with Auer rods on the differential. What is the most likely diagnosis?

Acute myelogenous leukemia

28-year-old male presents to the office with bilateral cervical lymphadenopathy of 2 cm with mild fever and sore throat for 1 week. He also complains of nausea and decreased appetite. On examination, there is hepatosplenomegaly and palatal petechiae. A new intern diagnoses him with streptococcal throat infection and prescribes antibiotics. The patient comes back the next day with a painless, itchy rash all over his body. The antibiotic that was most likely given to this patient is

Amoxicillin

A 56-year-old man presents to the outpatient clinic with increased fatigability, purpura, weight loss, bone pain, and pallor. CBC was done and revealed marked anemia, thrombocytopenia, and increased white blood cell count, and differential showed marked increase in plasma cells. CT head was done and showed multiple hypodense areas in the skull bone. A tissue biopsy was taken and stained positive with Congo red. What is the protein stained in the specimen?

Amyloid light chain (AL)

28-year-old woman receiving her first blood transfusion begins to experience flushing, tightness in her chest, and difficulty breathing within minutes of the start of the infusion. Her blood pressure drops rapidly, and the transfusion is immediately discontinued. Epinephrine is administered with good effect. Specimens sent to the lab show evidence of intravascular hemolysis. What is the most likely cause of such a reaction?

An ABO incompatibility

63-year-old man presents with fever and nosebleeds over the past several weeks; he also has the tendency to bruise easily. He has an oral temperature of 102° F. Physical exam is significant for splenomegaly palpable 6 cm below the left costal margin. No lymphadenopathy is present. Ecchymoses and petechiae are evident across the abdomen and around the knees and elbows. Results from a peripheral blood count reveal marked leukocytosis (WBC = 65 x 109/L) with the presence of large numbers of myeloblasts, mild anemia, and a platelet count of 43 x 109/L. What is the most likely diagnosis?

An acute neoplastic disorder

A 35-year-old man with known stage 3 chronic kidney disease due to diabetes presents with fatigue. His blood sugars are acceptable; his mood is euthymic, and he is sleeping and eating well. He does not smoke, and he has no known toxic environmental exposures. Work up shows normochromic normocytic anemia, with a hemoglobin of 11 g/dl, which is decreased from 12 g/dl 6 months ago. Medications include lisinopril, furosemide, and insulin. Iron 50 ug/dl (normal 50-150 ug/dl) Ferritin 200 ng/ml Ratio soluble transferrin receptor/log ferritin Reduced Reticulocytes 0.25% Platelets Normal Total white blood cell count 5x103/mm3 B12 levels, serum Normal What is the most likely diagnosis?

Anemia of chronic disease

A 20-month-old boy presents for a routine visit. He is eating well and drinking at least a bottle of cow's milk daily, which he has done since age 8 months. His history and physical are normal except for mild pallor. Urinalysis is normal. Result Normal Values Hemoglobin 9.5 10.5-14.0 g/dL RDW 52 38.5-49.0% MCV 72 76.0-90.0 fl Platelets 280,000 150,000-450,000/μL WBC 6.7 5000-14,500/μL What additional finding might you expect in this child?

Anisocytosis

65-year-old man develops fever, hoarseness, facial edema, and pruritus during transfusion. The transfusion is performed because of progressive anemia after pancreatic surgery 3 days ago. Since the surgery, the patient has been hemodynamicaly unstable and febrile (up to 38 C). When his Hgb dropped to 7 g/dL and Hctto 15.8%, transfusion of packed RBCs was ordered. Now, physical examination reveals fever (38 C), facial edema, local erythema, and morbilliform rash with wheezing. Post-transfusion blood specimen did not show hemolysis, and direct Coomb's test is negative. What is the most probable cause of his condition?

Antibodies to plasma proteins

24-year-old woman notices that she has a bruising tendency. She frequently has numerous small bruises and purple blotches on her skin. She hates having dental work because of the associated bleeding. On physical exam, you note that she has numerous petechiae. Her lab results are shown in the chart. TEST RESULTS REFERENCE RANGE Hematocrit 42 40-50 (male)36-44 (female) Hemoglobin 14 gm/dL 13.8-17.2 gm/dL (male)12.1-15.1 gm/dL (female) Platelets 41,000/mm3 130,000-400,000/mm3 PT 11.5 seconds 10.8-13.0 seconds PTT 30 seconds 25-41 seconds RBC 4.8 x 106/µl 4.5-5.7 x 106/µL (male)3.9-5.0 x 106/µL (female) The patient's condition is most likely to be characterized by what?

Antiplatelet IgG

37-year-old woman presents with fatigue, easy bruising, and menorrhagia. She states that she was in good health up until about 3 weeks ago, which is when she began feeling tired. She noticed that her last menstrual period lasted 14 days, which was about 8 days longer than usual. She states that she has no significant medical history, and she denies recent travel or illness. Physical exam reveals a thin, pale woman in mild physical distress. She is tachycardic and tachypneic. Laboratory analysis of her blood shows significant decreases in red/white blood cells and in platelets. A urinalysis is within normal limits and a bone marrow biopsy shows a decrease in red blood cells. What is the most likely cause of this patient's symptoms?

Aplastic anemia

14-year-old female is brought to the Family Medicine Clinic presenting with heavy menses and occasional epistaxis. The mother reports that this is common among the women in their extended family but not investigated until now. Lab values in the preliminary workup include a set of abnormal closure times in the PFA-100 bleeding test device (but not indicative of aspirin effect), a normal PT, slightly prolonged aPTT, normal platelet count, normal fibrinogen, and Hematocrit=32%. A von Willebrand's workup reveals 30% antigen level, 25% ristocetin cofactor activity level, and 40% Factor VIII clotting activity. The patient states that she wants analgesic relief from the discomfort accompanying menstruation and desires an intervention to reduce the episodic bleeding. What is the best set of recommendations you can make to this patient?

Avoid aspirin; use DDAVP (desmopressin) nasal spray at first sign of any excessive bleeding

A 30-year-old developmentally delayed man presents for elective surgery. His past medical history is significant for Pyrimidine-5'-Nucleotidase (P5N) Deficiency and a splenectomy due to splenomegaly and increased hemolysis. The following results are obtained from a complete blood cell count. Blood Cell Count Results White blood cell count 8.0 x 103 /uL Red blood cell count 2.09 x 106 /uL Hemoglobin 7.5 g/dL Hematocrit 22.5% Differential Segmented neutrophils 45% Band neutrophils 0% Lymphocytes 35% Monocytes 18% Eosinophils 2% The Wright's stained peripheral blood smear is significant for the presence of nucleated red blood cells, giant platelets, and the following (refer to the image). What does the cell in the image contain?

Basophilic Stippling

67-year-old woman presents for a routine physical exam. Her only concern is that, in the last year, she finds herself more easily fatigued. Physical exam is unremarkable. Results from a blood count reveal mild anemia and thrombocytopenia, with some rouleaux present on the peripheral blood smear. Serum chemistries are remarkable for elevated BUN and creatinine. Serum protein electrophoresis demonstrates the presence of a large monoclonal protein spike, which immunofixation identifies as IgG antibody. A bone marrow biopsy reveals 31% plasma cells. The best indicator of prognosis would be a blood test for levels of what substance?

Beta-2 microglobulin (B2M)

67-year-old man presents with a 2-day history of fatigue and shortness of breath. His history is significant for stage III colorectal carcinoma; it was diagnosed 15 months ago, and was initially treated by hemicolectomy and adjuvant 5FU/leucovorin with good result. A CT scan performed 3 weeks ago showed the presence of several small lesions in the liver. Fine needle biopsy revealed adenocarcinoma consistent with his primary cancer, and he was restarted on chemotherapy 2 weeks ago. On examination, he is pale and in obvious distress. Blood pressure is 96/62 mm Hg, and heart rate is 112. He has a well-healed midline abdominal scar and scattered petechiae over the right abdomen. Stool is positive for occult blood. Significant lab values are as follows: WBC of 1.8 x 103/uL, Hgb of 7.1 g/dL, and platelet count of 26,000/µL. What is the most likely primary cause of his anemia?

Bleeding due to thrombocytopenia (secondary to chemotherapy)

29-year-old woman presents with lumps in the neck, fever, weight loss, night sweats, itching, and lethargy. She is ultimately diagnosed with stage IV Hodgkin's lymphoma and treated with chemotherapy followed by radiation. Four months later she develops shortness of breath, and chest CT scan reveals pulmonary fibrosis. What chemotherapeutic agent is associated with this adverse reaction?

Bleomycin

73-year-old man presents with a nosebleed that will not stop. The bleeding has been present for over 2 hours. The patient's nose began dripping blood at breakfast; there was no known trauma. He denies pain. He has tried applying nasal pressure and lying down to rest. Until the bleeding began, the patient had not been experiencing any nasal symptoms, such as congestion, impaired nasal patency, or rhinitis. Because he felt too dizzy to drive, his wife drove him to the emergency department. The patient has no diagnosed medical conditions and takes no medications. He denies prior episodes of severe nosebleeds, easy bruising, and any known bleeding disorder. The patient's vitals are shown in the table. Weight 148 lb Height 69" Pulse 120 Respiratory rate 18 Blood pressure 90/66 mm Hg Temperature 97.4°F/36.3°C On physical exam, the man is holding a large bloodsoaked towel to his nose, with continued brisk bleeding. He is otherwise in no apparent distress but seems somewhat confused. On rhinoscopy, bleeding is observed from bilateral nares. No foreign body, mass, lesions or abrasions are visualized. The bleeding site cannot be identified. What test should be ordered next for this patient?

Blood type and Rh

45-year-old man with progressive weakness and fatigue presents with fever, shaking chills, shortness of breath, sharp chest pain, and productive cough with rusty sputum, mucosal and skin bleeding, and skin rash. He spent several weeks in a nuclear power facility after an earthquake disaster. Physical examination reveals an ill-appearing patient with signs of pallor, purpura, petechiae, stomatitis, diffuse rales, and dull percussion sound over middle parts of both lungs. His temperature is 38 C; blood pressure is 100/60; and respirations are 22. You will immediately stabilize the patient and order the necessary tests. What is the most specific diagnostic test for the primary disorder in this patient?

Bone marrow aspirate and biopsy

A 79-year-old man, with a history of type II diabetes mellitus, was seen 2 weeks ago for a routine follow-up exam. At that time, he had been watching his diet carefully and denied any fluctuations in blood glucose levels. He reported his wife had recently been admitted to a nursing home and his activity has been limited to visiting her on a near daily basis. He denied any other changes to his past medical history, family history, or social history. Labwork drawn prior to his appointment revealed Hgb A1C of 7.1%; the complete blood count and comprehensive metabolic profile results are as follows: WBC 4.0 x 103/mm3 4.5 - 11 RBC 3.73 x 106/μL 4.6 - 6.2 (men) Hemoglobin 11.7 gm/dL 13.5 - 18 (men) Hematocrit 34.3 % 40 - 54 (men) MCV 91.9 μ3 80 - 100 MCH 31.3 pg 26 - 36 MCHC 34 % 32 - 36 Red blood cell distribution width 12.9 % 11.6 - 14.6 Platelets 227,000 mm3 150,000 - 450,000 Neutrophil 51.9 % 53 - 70 Lymphocyte 32.4 % 13 - 46 Monocyte 9.8 % 3 - 9 Eosinophil 4.1 % 0 - 4 Basophil 1.8 % 0 - 1 Test Name Result Units Reference Comp Metabolic Panel Sodium 137 mmol/L 135 - 145 Potassium 4.5 mmol/L 3.5 - 5.2 Chloride 101 mmol/L 97 - 109 Carbon dioxide 30 mmol/L 20 - 29 Calcium 9.9 mg/dL 8.5 - 10.4 Alkaline phosphatase 63 U/L 41 - 142 AST 25 U/L 1 - 37 ALT 15 U/L 2 - 60 Bilirubin, total 0.50 mg/dL 0.20 - 1.20 Glucose, random 167 mg/dL Urea Nitrogen 30 mg/dL 7 - 21 Creatinine 1.8 mg/dL 0.9 - 1.4 BUN/Cr ratio 16.7 7.0 - 20.0 Protein, total 8.7 gm/dL 6.2 - 8.0 Albumin 4.5 gm/dL 3.6 - 5.0 Globulin, calculated 4.2 gm/dL 2.1 - 3.5 A/G Ratio 1.1 1.1 - 2.2 Upon review of these results, he subsequently undergoes testing for serum and urine immunofixation electrophoresis and a radiographic skeletal survey. He presents today with the following findings: Immunofixation - serum Interpretation An abnormal protein/monoclonal protein is identified Interpretation Immunofixation demonstrates abnormal homogeneous bands in the IgG and Kappa regions Interpretation Consistent with monoclonal gammopathy, IgG/Kappa type Specimen type: IgA Immunoglobulin 122 mg/dL 87 - 474 IgG Immunoglobulin 3622 mg/dL 681-1648 IgM Immunoglobulin 55 mg/dL 48 - 312 Immunofixation - urine Interpretation A trace of albumin is present Interpretation No serum protein is detected Interpretation No monoclonal protein is identified Specimen type Concentrated urine Skeletal survey reveals punched-out lesions in the cranium, ribs, and vertebrae. What is the next step in confirming the diagnosis?

Bone marrow aspirate and biopsy

79-year-old man with a history of type II diabetes mellitus was seen 2 weeks ago for routine follow up in the office. At that time, he had been watching his diet carefully and denied any fluctuations in blood glucose levels. He reported his wife had recently been admitted to a nursing home and his activity had been limited to visiting her on a near daily basis. He denied any other changes to his past medical history, family history, or social history. Lab work drawn prior to his appointment revealed Hgb A1c of 7.1%. The following complete blood count and comprehensive metabolic profile results are: WBC 4.0 x 103/mm3 4.5 - 11 RBC 3.73 x 106/μl 4.6 - 6.2 (male) Hemoglobin 11.7 gm/dL 13.5 - 18 (male) Hematocrit 34.3 % 40 - 54 (male) MCV 91.9 μ3 80 - 100 MCH 31.3 pg 26 - 36 MCHC 34 % 32 - 36 Red blood cell distribution width 12.9 % 11.6 - 14.6 Platelets 227,000 mm3 150,000 - 450,000 Neutrophil 51.9 % 53 - 70 Lymphocyte 32.4 % 13 - 46 Monocyte 9.8 % 3 - 9 Eosinophil 4.1 % 0 - 4 Basophil 1.8 % 0 - 1 Test Name Result Units Reference Comp Metabolic Panel Sodium 137 mmol/L 135 - 145 Potassium 4.5 mmol/L 3.5 - 5.2 Chloride 101 mmol/L 97 - 109 Carbon Dioxide 30 mmol/L 20 - 29 Calcium 9.9 mg/dL 8.5 - 10.4 Alkaline Phosphatase 63 U/L 41 - 142 AST 25 U/L 1 - 37 ALT 15 U/L 2 - 60 Bilirubin, Total 0.50 mg/dL 0.20 - 1.20 Glucose, random 167 mg/dL Urea Nitrogen 30 mg/dL 7 - 21 Creatinine 1.8 mg/dL 0.9 - 1.4 BUN/Cr Ratio 16.7 7.0 - 20.0 Protein, Total 8.7 gm/dL 6.2 - 8.0 Albumin 4.5 gm/dL 3.6 - 5.0 Globulin, calculated 4.2 gm/dL 2.1 - 3.5 A/G Ratio 1.1 1.1 - 2.2 Upon review of these results, he subsequently underwent testing for serum and urine immunofixation electrophoresis and a radiographic skeletal survey. He presents today with the following findings: Immunofixation - serum Interpretation An abnormal protein/monoclonal protein is identified. Interpretation Immunofixation demonstrates abnormal homogeneous bands in the IgG and Kappa regions. Interpretation Consistent with monoclonal gammopathy, IgG/Kappa type. Specimen type: IgA Immunoglobulin 122 mg/dl 87 - 474 IgG Immunoglobulin 3622 mg/dl 681-1648 IgM Immunoglobulin 55 mg/dl 48 - 312 Immunofixation - urine Interpretation A trace of albumin is present. Interpretation No serum protein is detected. Interpretation No monoclonal protein is identified. Specimen type Concentrated urine Skeletal survey reveals punched-out lesions in the cranium, ribs, and vertebrae. Based upon these results, what would you most likely expect to find on history and physical examination?

Bone pain on review of systems

38-year-old woman presents with a 2-week history of sore throat and fever; symptoms have been unresponsive to over-the-counter medications. On exam, the patient appears ill. Her temperature is 39 C; Her pharynx is red and injected; you notice painful aphthous ulcers in her mouth. Complete blood count reveals a WBC of 1.60 X 109/L; differential shows 0.5% neutrophils. Hemoglobin is 9.5 g/dL. Urine dipstick shows proteinuria. Blood cultures and other analyses are pending. You immediately admit the patient and order aggressive volume replacement, filgrastim, and what additional treatment?

Carbapenem

28-year-old woman in her 35th week of gestation presents to the emergency room after noting the sudden onset of lower abdominal pain and profuse vaginal bleeding. On examination, she is tachycardic with a blood pressure of 72/46. Oozing is noted from an IV site. A fetal heart tone is barely audible. History is significant for a previous pregnancy, which was uncomplicated. There is no personal or family history of a bleeding disorder. Significant laboratory values include markedly elevated PT and aPTT, a platelet count of 26,000/uL, fibrinogen of 80 mg/dL, and fibrin degradation products (FDP's) > 40 ug/mL. Which of the following therapies is most important in correcting this condition?

Cesarean section

55-year-old man presents with a 1-week history of fatigue, night sweats, and abdominal fullness. On physical examination, you note a palpable spleen. You order a CBC; the results indicate a white blood count of 105,000 cells/mcL with a left shift of the myeloid series. The red blood cell count and morphology show anemia, and he has an elevated platelet count. To help confirm your suspicions, you order genetic studies, and the results come back with the BCR/ABL gene detected. What is the most likely diagnosis?

Chronic myelogenous leukemia

Your patient is a 75-year-old man complaining of fatigue, weak appetite, palpitations, and weakness, as well as sore tongue, frequent stomach upsets, and indigestion problems. He also has tingling in both legs, dizziness, and started forgetting things. During the last year, he fell several times. Your examination reveals low blood pressure, swollen beefy tongue, sinus tachycardia, pallor, and loss of vibratory sensation in lower extremities. What will be your next step in diagnosis?

Complete blood count

55-year-old caucasian male comes to the physician because he noticed, after hearing remarks by his friends and family, that his face had become "redder than usual". He denies any other symptoms, including pruritus, fatigue, tiredness, or any other recent-onset disease. The patient is sedentary and overweight, but has no relevant past medical history. His mother died from heart disease and his father from complications of emphysema. He smoked a pack of cigarettes a day for 20 years until 8 years ago, when he managed to quit. Vital signs are temperature 36.6° C (97.9° F), BP 130/80 mmHg, HR 86 bpm, RR 17 bpm. On physical examination, his face is plethoric. There is no cyanosis, scratch marks on his skin, or signs of labored breathing. Heart rate and rhythm are normal, and peripheral pulses are isochronous and symmetric. The abdomen is soft and nontender to palpation, and there are no organomegalies. The left hypochondriac region is tympanic to percussion. A blood count shows the following: Hematocrit 55%, Hemoglobin 14 g/dL, Platelets 410,000 /mm3 Mean Corpuscular Hemoglobin Concentration (MCHC) 28 fl. Leukocytes 12,500 /mm3 with a normal differential. Examination of a blood film shows no abnormalities. Which of the following is the most probable pathophysiologic mechanism of his blood abnormalities?

Decreased plasma volume

70-year-old woman presents to the ED with nausea, anorexia, vomiting, fever, chills, and flank pain. Symptoms appeared abruptly after a 2-day history of mild dysuria. Her family reports that about 2 months ago, she started having urinary urgency and frequency and noted excessive urinary production of around 3-4 liters daily. She has a history of hypertension, poorly regulated type II diabetes, and mild cognitive impairment. Her long-term medications are aspirin and metformin, and 2 months ago, her family practitioner included furosemide, statin, and proposed insulin, but she was not willing to take any parenteral medication. Her fear of needles was the reason she gave for not agreeing to get recommended immunizations. On admission, she appears lethargic; her blood pressure is 80/40; pulse rate is 120; respiratory is 18; and temperature is 103.4. What is the primary risk factor responsible for her current condition?

Diabetes mellitus

A 45-year-old dialysis patient has a 4-month history of low hemoglobin levels, despite increasing her erythropoietin dose to 300 unit/kg/week. She has been on dialysis for 1 year due to hypertensive nephrosclerosis. She does not take ACE inhibitors; she has had no infections, surgeries, or transplants; age-appropriate cancer screening has been negative. Diagnostic studies include: Hemoglobin 10.5 g/dl Transferrin saturation 30% Ferritin 400 ng/ml B12, Folate, TSH Normal, normal, normal Anti Red cell antibodies normal HIV testing negative LDH normal Aluminum, serum normal What is the next step in the management of this patient?

Evaluate PTH levels

An 18-year-old woman is involved in a motor vehicle accident and is transfused 4 units of packed red blood cells to correct the blood loss. 24 hours after transfusion, the nurse notices that the patient is having chills and rigors and measures her temperature. The reading comes back as 100.4°F. Blood is drawn, and the following are the results of the lab investigations: Hemoglobin: 8.9 g/dL WBC: 8000 per cubic millimeter Total bilirubin: 1.2 g/dL Serum haptoglobin: normal Urine analysis: negative for hemoglobin at Hilo seven LDH: normal CRP: normal What is the most likely cause of the patient's condition?

Febrile non-hemolytic transfusion injury

48-year-old African American man presents with easy fatigability, poor concentration, and "stronger than usual" effort-related palpitations. He denies constipation or weight loss. His past medical history includes type 2 diabetes diagnosed 10 years ago; it is well-controlled with diet and metformin, and there are no complications. His parents are in good health; his sister had a malignant breast nodule removed 2 years ago, but she showed no signs of metastatic disease. His physical examination shows mild obesity (BMI 32 kg/m2), otherwise normal. A laboratory workup shows glucose 91 mg/dL, Na+ 138 mEq/L, K+ 4.9 mEq/L, Cl- 100 mEq/L, hemoglobin 11 g/dL, hematocrit 32%, mean corpuscular volume (MCV) 75 fl, mean corpuscular hemoglobin concentration (MCHC) 24 pg/mL, leukocytes 7,400/mm3 with normal differential. Platelets 310,000/mm3, ferritin 29 ng/L (25-300 ng/mL) and an elevated total iron-binding capacity. What is the most appropriate next step in diagnosis?

Fecal occult blood testing (FOBT)

30-year-old woman presents with diarrhea, general malaise, weight loss, and skin changes. Her symptoms started gradually 6 - 7 months prior to presentation; they began with progressive flatulence as well as bulky, greasy, and foul-smelling stools. She lost 8 pounds. Painful erosions appeared recently on the angles of her mouth; she also noted recurrent aphthous ulcers in the mouth. She also noticed pruritic vesicular changes as well as skin erosions grouping mostly over her elbows and shoulders that bleed easily. She started having problems driving at night. She remembers that she had similar gastrointestinal problems in childhood; they were attributed to stress and disappeared spontaneously. The rest of her personal and family history is not contributing. Physical examination reveals a slim woman with angular cheilitis and skin lesions consistent with dermatitis herpetiformis (symmetric vesiculations, crusts, and erosions over the extensor areas of elbows, knees, shoulders, and scalp; they tend to group). Palpation of the abdomen shows slight diffuse tenderness, and the rest of the examination is normal. Peripheral smear shows dimorphic anemia with reticulocyte count of < 2%; Vitamins B12, folate, iron, and electrolytes (magnesium and calcium) are decreased, prothrombin time is prolonged, stool examination for ova and parasites and smear for fecal leucocytes are negative, and Sudan stain for fat in stool is positive. What is the best treatment for her condition?

Gluten-free diet

22-year-old African American man presents with excruciating pain in his back for the past 2 days. He denies any history of trauma. He gives a history of similar pains in the past. His older sister has a similar illness. On examination, his HR is 110 beats/minute and he has a tinge of jaundice. What laboratory result would you expect after analyzing his blood?

Hematocrit of 21%

12-year-old Jewish girl is brought in by her father. The father reports that the child started to bleed profusely following tooth extraction 2 hours ago. Further questioning reveals that there is a history of bleeding disorder in the child's maternal uncle and aunt. Examination reveals tachycardia and bleeding from the site of tooth extraction. Coagulation profile reveals prolonged activated partial thromboplastin time (aPTT), normal prothrombin time (PT) and thrombin time (TT), and deficiency of factor XI. The child was treated with fresh frozen plasma (FFP) and fibrin glue. What is the likely diagnosis?

Hemophilia C

An asymptomatic 62-year-old African American woman is discovered to have marked leukocytosis and thrombocytopenia. Results from a bone marrow biopsy demonstrate the presence of the Philadelphia chromosome, and the diagnosis of chronic myeloid leukemia (CML) is made. She relates a medical history that is remarkable only for breast cancer, for which she underwent a mastectomy 15 years ago. She has a 40-pack/year history of smoking. She had a brother who died of leukemia at a young age. Based on this history, what factor places her at greatest risk for the development of CML?

Her age

66-year-old woman presents with shortness of breath, headaches, and dizziness over the past several weeks. She was diagnosed with multiple myeloma 8 months ago but has not undergone treatment because she has been asymptomatic. Her medical history is otherwise unremarkable. Blood count reveals a Hgb of 9 g/dL, and serum protein electrophoresis shows increased levels of IgG paraprotein. Serum chemistries are significant for hypoalbuminemia and hypercalcemia. After treating the most immediate concern (her hypercalcemia), what should the next step be?

High-dose chemotherapy followed by autologous stem cell transplantation

8-year-old boy presents with a 15-day history of a low-grade continuous fever. He has decreased appetite and significant lethargy. On clinical exam, he is found to have gross pallor, significant bilateral cervical lymphadenopathy, right axillary apical group adenopathy, and two ecchymotic lesions over the abdomen and thigh. The liver is palpable 5.5 cm below the right costal margin, and the spleen is palpable 6 cm below the left costal margin. On auscultation, there is an ejection systolic murmur, grade 3/6 in the pulmonary area. There is no neurological deficit or any bony tenderness. A peripheral smear revealed a few blasts, and a bone marrow aspiration is done on which a diagnosis of ALL-L2 is made. He is started on chemotherapy. During the first week, the child was afebrile and tolerated the chemotherapy well. During the second week, he received his usual dose of vincristine and was monitored thereafter. 8 hours later, the child began speaking irrelevantly and had a generalized tonic clonic seizure lasting 1 minute. A 0.3 mg/kg injection of diazepam was given and the child was moved to the ICU. His blood pressure was normal, and examination of the fundus was normal. In anticipation of an electrolyte abnormality, serum electrolytes were sent. Urine osmolality was noted to be 900 mOsm/kg. What pair of electrolyte disturbances is most likely?

Hyponatremia and hypokalemia

A 62-year-old man presents with a 6-month history of a painless mass on the left side of the neck. He denies any preceding or concurrent illness and is currently afebrile. In fact, recent history is significant only for weight loss from 180 to 160 pounds over the last several months. Past history is significant for severe seasonal allergies. On physical examination, he has left-sided cervical and supraclavicular lymphadenopathy consisting of matted nodes that is painless to palpation. Excisional biopsy of a group of nodes reveals a malignant lymphoma, which is classified as follicular, small-cleaved cell. Chest X-ray demonstrates left-sided hilar adenopathy. A CT scan of the abdomen and pelvis is negative; so is a bone marrow biopsy. According to the Ann Arbor system, what stage would this lymphoma be classified as?

IIB

38-year-old woman presents with increased menstrual bleeding over the course of the past several months; she has also had 2 episodes of epistaxis within the last week. Other than mild fatigue, she relates no other symptoms. She is afebrile and normotensive. Physical exam is significant only for scattered petechiae on the lower extremities. Except for an Hgb of 11.4 g/dL and a platelet count of 40,000/uL, complete blood count is within normal limits. Peripheral blood smear is normal. What is the most likely diagnosis?

Idiopathic (Immune) thrombocytopenic purpura

45-year-old man is evaluated for bleeding gums. He notices easy bleeding with minor trauma, especially after brushing his teeth. He also bruises easily and has multiple ecchymoses. He is a non-smoker and drinks no alcohol. Physical examination shows BP 110/70; BMI 18; Pulse 70/min; Temp normal. Cardiovascular and respiratory exam appear normal. Abdominal exam shows an enlarged liver and spleen, with the spleen measuring more than 10 cm in diameter. Lab investigations are shown below: Hb: 7.2 WBC count: 76,000 Neutrophils: 55% Lymphocytes: 20% Eosinophils: 12% Basophils: 10% Monocytes: 3% Platelet count: 42,000 LAP: low Peripheral smear: numerous granulocytes, with immature myeloid cells (myelocytes and metamyelocytes), and occasional blasts. Bone marrow appears hypercellular, with increase of the myeloid cell line (neutrophils, eosinophils, basophils) and progenitor cells. Megakaryocytes are prominent and increased. Cytogenetic studies show reciprocal translocation between chromosomes 9 and 22. What is considered to be the treatment of choice?

Imatinib

74-year-old man presents with lower back pain and fatigue; symptoms have gotten worse over the past several months. Physical exam is remarkable only for tenderness in the area of the lower spine. An X-ray demonstrates several lytic lesions in the vertebrae and ribs. Results from a blood count reveal mild anemia and thrombocytopenia; there are rouleaux present on the peripheral blood smear. Serum chemistries are remarkable for elevated creatinine and calcium levels, along with hypoalbuminemia. Immunoglobulin levels are decreased. Serum protein electrophoresis demonstrates the presence of a large monoclonal protein spike, which immunofixation identifies as mainly kappa light chains. A bone marrow biopsy reveals 31% plasma cells. What is the most common cause of death among people with this condition?

Infection

15-year-old boy presents with severe pharyngitis; he has been experiencing malaise and fatigue for approximately 1 week. He has been sent home twice from school for reportedly falling asleep in class. Physical examination is remarkable for bilateral anterior/posterior cervical lymphadenopathy and splenomegaly. Body temperature is 39.6° C. A CBC is performed and is remarkable for a WBC of 6,800/µL with a differential of 30% neutrophils, 38% lymphocytes, 14% atypical lymphocytes (see image), and 18% monocytes. Liver function tests are ordered, and the following results are obtained: AST level of 200 U/liter, ALT level of 250 U/liter, and an alkaline phosphatase level of 188 U/liter. A chest radiograph is negative. A throat screen for group A streptococcus is negative. Based on the clinical history and laboratory test results, an additional laboratory test is ordered; this test is positive. What is the most likely diagnosis?

Infectious mononucleosis

29-year-old man presented 3 days ago with sore throat and fever; he was treated with amoxicillin and is now complaining of generalized fatigue, abdominal pain, and maculopapular rash. The patient is sexually active with a single girlfriend who was sick 1 month ago but is now better. The most likely diagnosis is

Infectious mononucleosis

24-year-old woman is evaluated in the endocrinology clinic. She is on 100 mcg of thyroxine per day for treatment of Hashimoto's thyroiditis and receives oral steroids for autoimmune adrenal insufficiency. In addition, she complains of fatigue, numbness, and tingling in her feet. When routine labs were drawn, the following values were noted: Hb: 9.9 g/dL MCV: 102 Iron indices: normal Folate: normal B12: low Parietal cell antibodies: high What would prevent the development of megaloblastic anemia in this patient?

Intramuscular injection of B12

34-year-old woman presents with fatigue, headache, shortness of breath, and dizziness upon rising from a chair. The patient denies pregnancy or recent illness; she is taking no medications. She has no pain. Upon physical exam, her skin is pale; her nails are brittle, and she has a blood pressure of 98/76 mm Hg. CBC reveals a microcytic anemia. What is the most likely diagnosis?

Iron deficiency anemia

42-year-old African-American woman presents with increased fatigue and unusual cravings for chalk and raw potatoes over the past few months. What is the most likely diagnosis?

Iron deficiency anemia

61-year-old man is hospitalized with fatigue and severe anemia. The patient states that about 2 weeks ago he began to feel tired and have shortness of breath after minimal exertion. He also notes that he has had 2 colds in the past 7 or 8 weeks. He denies any significant medical history, but states that he has been exposed to solvent fumes at his job for several years. He works at a dry cleaning facility. Laboratory analysis of his blood shows a decrease in mature red blood cells, along with a decrease in platelets and white blood cells. What statement concerning this patient's condition is true?

It can occur in pregnancy

16-year-old male high school football player was tackled and landed on the football on his left side. He is brought to the emergency department and is found to have nausea, diaphoresis, tachycardia, hypotension, left upper quadrant tenderness, and pain in the tip of the shoulder while lying down with his legs elevated. A CT is done, and he is found to have a ruptured spleen. What is the name of the diagnostic finding most often associated with this patient's illness?

Kehr's sign

A 2-year-old boy presents with his mother due to her concerns about his paleness; she states that he used to be playful, but now looks tired and doesn't even cry as he did previously. She first noticed this occuring 3 months after moving to his grandmother's old house. CBC is ordered and shows the following results: RBC 4 million/microliter, WBC 11,000/mm3, Hb 9 mg/dL, platelets 180,000/mm3, and hematocrit 31%. The image shown is of his blood film. What is the most likely diagnosis?

Lead poisoning

62-year-old man presents with a 6-month history of a painless mass on the left side of the neck. He denies any preceding or concurrent illness, and is currently afebrile. In fact, recent history is significant only for weight loss from 180 to 160 pounds over the last several months. History is significant for severe seasonal allergies. On physical examination, he has left-sided cervical and supraclavicular lymphadenopathy consisting of matted nodes that is painless to palpation. Excisional biopsy of a group of nodes reveals a malignant lymphoma, which is classified as follicular, small-cleaved cell. Chest X-ray demonstrates left-sided hilar adenopathy. A CT of the abdomen and pelvis is negative; so is a bone marrow biopsy. How would you characterize the patient's prognosis?

Likely to be in an advanced disease stage at the time of diagnosis

60-year-old male presents to your office with fatigue and generalized lymphadenopathy. Physical examination reveals massive splenomegaly in addition to lymphadenopathy. A lymph node biopsy was subsequently performed. The image represents a histologic section of the lymph node (refer to the image). The most likely diagnosis is

Mantle cell lymphoma

22-year-old woman presents with an 8-hour history of headache and vomiting. She resides in a college dormitory; she has no remarkable travel history, and reports eating food from a street vendor 24 hours before becoming ill. The patient is lethargic and disoriented. On examination, she is found to have a temperature of 38.5° C and a petechial rash on her legs. Gram stain of exudate from a skin lesion shows Gram-negative diplococci. A lumbar puncture is performed; the cerebral spinal fluid (CSF) reveals a leukocyte count of 5/μL consisting of primarily neutrophils. A Gram stain of CSF is negative for bacteria. What is the most likely diagnosis?

Meningococcemia

15-year-old boy with no significant past medical history is seen in the Emergency Department after he had collapsed after a dental extraction in which prilocaine hydrochloride was used as topical anesthesia. He states that during the procedure he did not feel well. Shortly after the procedure, he developed a headache, difficulty breathing, and a bluish skin color. Physical examination reveals an ill-appearing young man in moderate distress. He is cyanotic and tachypnic. His breath sounds are clear equally. Although in distress, he is alert and oriented. A sample of his arterial blood is drawn and noted to have a deep brown color that does not turn red when exposed to air. The patient is treated with IV methylene blue and begins to improve within 1 hour of treatment. What is the most likely cause of this patient's symptoms?

Methemoglobinemia

84-year-old widow presents with a history of osteoarthritis, gastroesophageal reflux, anemia, and very mild dementia. The patient lives alone; her daughter, who has brought her in, visits every 1 - 2 weeks to help with shopping and housework. Until now, the patient has been able to manage fairly well; lately the daughter feels her mother is becoming increasingly confused. Although there is adequate food in the house, she suspects her mother is not eating properly and may not be taking her medications. The patient states she is 'fine' except that her feet "always feel tingly, like they are asleep," She states that she eats enough for herself, but is vague when asked about her medications; they include a multivitamin, pantoprazole (Protonix) and donepezil (Aricept) daily, and alendronate (Fosamax) weekly. Her daughter notes that her mother is not known to drink alcohol. except for an occasional glass of wine with dinner. The patient's physical exam is generally unremarkable; the exception is her neurologic exam which is significant for mild ataxia and decreased vibratory and proprioception in her hands and feet. There is no nystagmus. Mini-mental state examination score is 23. Her laboratory studies are as follows: WBC 8.6 x 1,000 cells/cmm; RBC 3.41 x 100,000 cells/mcL; HCT 35.1 L%; MCV 100.6 fL; MCHC 34.4%; PLT 179,000/mcL; B12 208 pg/m (211 - 911 pg/mL); and folate 4 ng/m (5.4 - 24 ng/m). What single lab result, if elevated, would best correlate with the patient's symptoms?

Methylmalonic acid

29-year-old man presented 3 days ago with a sore throat and fever; he was treated with amoxicillin and now presents with generalized fatigue, abdominal pain, and a maculopapular rash. The patient is sexually active with one partner who was sick 1 month ago; she is now better. What is the most important single test to be done now?

Monospot heterophile antibody test

52-year-old man has a backache and is easily fatigued. The problem used to settle with aspirin and rest, but it has progressed and worsened over the last month. His family doctor orders some lab tests and X-rays. Remarkable findings at this time are Hb 7.8, serum creatinine 2.9 mg percentage, and compression fractures of vertebral bodies of T12, L1, and L4. What is the most likely diagnosis?

Multiple myeloma

56-year-old man presents to the outpatient clinic with increased fatigability, purpura, weight loss, bone pain, and pallor. CBC was done and revealed marked anemia, thrombocytopenia, and increased white blood cell count, and differential showed marked increase in plasma cells. CT head was done and showed multiple hypodense areas in the skull bone. What is the most likely diagnosis?

Multiple myeloma

62-year-old man presents with increasing left rib pain over several months; he has also experienced tingling and numbness of his lips and extremities over the past several weeks. Serum chemistries reveal marked hypercalcemia, and X-rays demonstrate several rib fractures and diffuse osteoporosis. Serum protein electrophoresis shows the presence of a monoclonal protein spike in the gamma region. What is the most likely diagnosis?

Multiple myeloma

67-year-old woman presents with lower back pain that has progressively worsened over the last 6 months. She has also recently noticed that she is more easily fatigued. Aside from tenderness in the lumbar spine, her physical exam is unremarkable. X-rays of the spine reveal a compression fracture of the 3rd lumbar vertebra. Results from a blood count reveal a moderate, normocytic anemia; serum chemistries, platelet, and WBC values are within normal limits. The laboratory reports the presence of rouleaux in the peripheral blood smear. Serum protein electrophoresis demonstrates the presence of a large monoclonal protein spike, which immunofixation identifies as IgG antibody. What is the most likely cause of the patient's anemia?

Multiple myeloma

A 60-year-old man presents with sharp pain in his ribs and sternum; he has a history of allergies and has been sneezing frequently. Except for a heart rate of 120/min, the physical examination is normal. With the exception of a tonsillectomy as a child, his medical and surgical history is unremarkable. He has chronic seasonal allergies. Family history reveals nothing significant. Radiographs on the ribs and sternum reveal lytic lesions and a significant fracture of the sternum. Neurological examination is unremarkable. The laboratory results are shown in the table. Peripheral blood smear reveals rouleaux formation of RBCs. Bone marrow aspiration reveals large numbers of plasma cells. Tests Results Differential BUN 24 mg/dL Metas 0.30% Protein 12.0 g/dL Bands 3.20% Albumin 3.8 g/dL Segs 55.30% Uric acid 11.9 mg/dL Eos 4.00% Calcium 8.4 mEq/L Basos 0% Sodium 137 mmol/L Lymphs 34.00% Urinalysis Protein 4+ Monos 3.20% RBC 4.3 x 1012/L Platelets 380 x 109/L Hgb 12.4 g/dL Hct 0.35 MCV 82 fL MCHC 34% MCH 28 pg WBC 8.0 x 109/L What is the most likely diagnosis?

Multiple myeloma

A 62-year-old woman presents with severe pain of her ribs. She claims to have developed the pain immediately after having sneezed forcefully the previous night. She also describes a generalized joint pain throughout her body. She denies any cough and fever. Her past medical history is not significant for any recent travel or illness. Radiographs of her ribs are significant for fractures and what appeared as "punched-out lytic lesions" on the sternum. A CBC is significant for a normocytic-normochromic anemia, rouleaux formation of red blood cells, and the occasional presence of the following cell (refer to the image) on the differential. Serum globulin is elevated. The patient is positive for Bence Jones proteinuria. What is the most likely diagnosis?

Multiple myeloma

55-year-old male firefighter suddenly develops fever, palpitations, and shortness of breath 7 days after skin transplant for severe facial burns. He also has fever resistant to intravenous antibiotics therapy introduced at the onset of fever. Because of oropharyngeal lesions, he is on parenteral nutrition. On examination, transplant shows neither signs of infections nor signs of rejection. Patient appears lethargic, with a blood pressure of 80/40, pulse rate of 120, respiratory rate of 18, and temperature of 103.4°F. Stat CBC shows neutropenia and eosinophilia. Why would you order a blood culture for systemic fungal infection?

Neutropenia

62-year-old man was diagnosed with multiple myeloma 1 year ago; he is currently hospitalized with intractable nausea and vomiting. Since he was asymptomatic at the time of diagnosis, he has not been treated for his cancer. His wife reports he has not eaten in several days, but he is constantly drinking water. He appears confused and lethargic. When adjusted for the albumin level, serum chemistries reveal an elevated level of calcium (12.0 mg/dL). What should the initial treatment of his hypercalcemia include?

Normal saline

28-year-old woman, who did not receive prenatal care, gives birth to her 3rd child. The newborn develops severe anemia, hyperbilirubinemia, and hypoalbuminemia within 24 hours of birth. Results from the blood bank reveal that the mother's blood type is O negative. Her indirect antiglobulin test is positive; however, the antibody is yet to be identified by the lab. What is the infant's blood type?

O positive

67-year-old woman presents for a routine physical exam. Her only complaint is that in the last year she finds herself more easily fatigued. Physical exam is unremarkable. Results from a blood count reveal mild anemia (Hgb 12 g/dL) and mild thrombocytopenia; there are some rouleaux present on the peripheral blood smear. Serum chemistries are unremarkable. Serum protein electrophoresis demonstrates the presence of a large monoclonal protein spike, which immunofixation identifies as IgG antibody. A bone marrow biopsy reveals 21% plasma cells. What is the most appropriate treatment for her condition?

Observation

5-year-old boy presents because of repeated bruising with ecchymoses and prolonged bleeding. 2 days ago, a minor cut in his knee bled for 2 hours and only improved after application of a compressive dressing. He has received all immunizations, reached all developmental milestones at appropriate ages, and has no relevant past medical history. His mother and 8-year-old brother are both in good health. Vital signs are within normal range. On examination, he has diffuse ecchymoses on his hands, knees, and legs. There is no palpable lymph node enlargement. His liver is palpable 1 cm below the right costal border and has a smooth texture and outline. There are no signs of splenomegaly. A laboratory workup shows the following: Ht 39% Hb 13 mg/dL, MCV 89 fl, platelets 31,000/mm3. Liver function tests were as follows: AST 14 U/L, ALT 12 U/L, γGT 22 U/L. Total bilirubin 0.5 mg/dL, direct bilirubin 0.2 mg/dL. A peripheral blood smear shows normal erythrocytes and leukocytes, a reduction in the number of platelets, and a few giant platelets. What is the most appropriate next step?

Observation only.

45-year-old man is evaluated for a 6-month history of palpitations, easy fatigability, and chest pain on exertion. He does not smoke or consume alcohol; he has no significant past medical history. His body weight has remained stable. He has spent the last 2 years traveling the world. His wife says that he eats "unhealthily." He is a strict vegan, consumes a lot of snacks, and has never taken any vitamin supplements. Examination shows a 5'7" male with a BMI of 19. His BP is 130/70 and pulse 90/min; his temperature is 98.4°F. Conjunctival pallor is present. Auscultation shows a grade 2/6 murmur ejection systolic murmur heard all over the precordium. Lab investigations are sent and are given below: Hb: 8.8 g/dLWBC: 6000/mm3MCV: 72Ferritin: lowTIBC: elevatedStool occult blood: negativeESR: 8 mm/hourTSH: normal What is the most appropriate medical therapy?

Oral ferrous sulfate

A 35-year-old man with stage 3 chronic kidney disease has had a decreasing hemoglobin count from 12 g/dl to 10.5 g/dl over the past 18 months. He is asymptomatic. Work up shows: Hemoglobin 10.5 g/dl Iron 49 ug/dl (normal 50-150 ug/dl) Transferrin Saturation 18% Ferritin 75 ng/ml Stool occult blood Negative, 3 samples B12 Normal Folate Normal TSH Normal Reticulocytes Low What treatment is suggested?

Oral iron supplements

A 16-year-old boy presents for a routine physical examination. He has just recently moved into the area. His mother is asked to fill out a routine medical history form. The history form and physical examination are unremarkable. A complete blood cell count and routine urinalysis are performed. The urinalysis results are within normal limits. The complete blood cell count results are as follows: Blood Cell Count Results White blood cell count 9.0 x 103 /ul Red blood cell count 4.50 x 106 /ul Hgb 14.0 g/dl Hct 42.0% Differential Segmented neutrophils 55% Band neutrophils 16% Lymphocytes 23% Monocytes 2% Eosinophils 2% Basophils 2% The Wright's stained peripheral smear is observed and found to have the following cells (refer to the image). This patient's peripheral smear is significant for the presence of what?

Pelger-Huet Anomaly

32-year-old patient is receiving 2 units of packed blood cells because of severe anemia after a miscarriage. During the transfusion, she becomes confused. Examination shows generalized rigidity, high temperature (40 degrees C), tachycardia (120/min), and hypotension (80/55). Urine obtained from the catheter is bloody, and she is oozing blood from the IV access line. She receives Cefoxitin. Complete blood count shows severe anemia (hemoglobin 6 g/dL), leukocytosis (white blood cells 15,000), prolonged bleeding time, low platelets (100,000), elevated prothrombin time (PT) and partial thromboplastin time (PTT) (20 seconds and 75 seconds respectively), low fibrinogen (50 mg/dL) and high fibrin split products, elevated LDH (500 U/L), and elevated both total and unconjugated bilirubin. Urine is positive for hemoglobin, and both BUN and creatinine are elevated (35 mg/dL and 1.8 mg/dL, respectively). You started the therapy immediately. What will be your next step in the diagnosis?

Perform blood typing of patient and donor

64-year-old man is admitted to the hospital for evaluation of profound fatigue and severe back pain. The patient had no history of fever, and vitals were unremarkable upon admission. He has no significant past medical history or travel history. A complete blood cell count, urinalysis, BUN and creatinine, and electrolytes are performed. Urinalysis, BUN/creatinine, and electrolyte results are unremarkable. The complete blood cell results demonstrate anemia (Hct 21%). The Wright's stained peripheral blood smear is significant for the rouleaux formation and the following (refer to the image). Based on these results, a bone marrow biopsy is performed that is similarly remarkable. A serum immunological electrophoresis it ordered; it is significant for the presence of a monoclonal gammopathy of IgG origin. What is most likely present in the Wright's stained peripheral blood smear from this patient?

Plasma cell lymphocytes

68-year-old woman presents with a 2- to 3-month history of dyspnea on exertion, easy fatigability, and lassitude. She states that recently she has been having difficulty swallowing. She denies hemoptysis, GI, or vaginal bleeding. She claims her diet is good, but her appetite has varied. On physical examination, no significant physical findings are noted other than pallor. Occult blood is negative. A hemoglobin and hematocrit are ordered; the results are 7.2 g/dL and 22%, respectively. She is diagnosed with iron deficiency anemia. The results of a barium esophagram reveal esophageal webbing. What is the most likely diagnosis?

Plummer-Vinson syndrome

77-year-old man presents with left-sided chest pain, headaches, night sweats, a burning sensation in his hands and feet, and shortness of breath. He began having headaches 2 days ago, and his symptoms have progressively worsened. On exam, the patient is thin and ill-appearing. His spleen is palpated 8 cm below the costal margin, and the liver 5 cm below the costal margin. Laboratory analysis shows that his red blood cell count is elevated, and he has a decrease in plasma volume. White blood cells and platelets are within normal limits. Appearance of the red blood cells is normal. Arterial blood gas analysis shows an O2 saturation of 94% on room air. What is the most likely cause of this patient's symptoms?

Polycythemia vera

47-year-old woman presents after developing a low-grade fever and mild jaundice over the past several days. Her recent history is significant for a total abdominal hysterectomy performed 12 days ago, at which time she received 2 units of packed red blood cells without incident. Although there is no evidence of bleeding at present, her hemoglobin level is only 10.2 g/dl. You believe this may be a delayed transfusion reaction. What is the most likely cause of the reaction?

Recipient antibodies against donor red cell antigens not detected on cross match

28-year-old woman presents with heavy menstrual bleeding; she soaks through a pad every hour or so. She also has a 2-week history of fatigue and bleeding from her gums. Physical examination reveals a pale woman with an enlarged spleen and diffuse petechiae. A complete blood cell count shows leukocytosis (WBC 10,000), with 29% blasts as well as the presence of Auer rods, anemia (hemoglobin 7.9 g/dL), and thrombocytopenia (platelet count 25,600). A bone marrow biopsy is positive for AML. What will be your next step in the management of the disease?

Remission induction

6-year-old boy is brought in after cutting his right hand 30 minutes ago. Despite continuous compression, the cut continues to ooze blood. His past medical history includes idiopathic thrombocytopenic purpura starting 8 months ago, which responded initially to intravenous immunoglobulin and resolved completely after a laparoscopic splenectomy 6 months ago. His immunizations are up to date, and he has no other relevant past medical history. Family history is also unremarkable. Vital signs are BP 110/ 80 mmHg, HR 98 bpm, RR 18 rpm, temperature 36.3° C (97.2° F). On physical examination, he is alert and oriented, showing no signs of acute distress. There are several bruises and ecchymoses around his ankles, elbows, and conjunctivae. The remainder of the physical examination shows no abnormalities. A complete blood count shows Hemoglobin 14 g/dL, Hematocrit 40%, Mean Corpuscular Volume (MCV) 88 fl, Mean Corpuscular Hemoglobin Concentration (MCHC) 29 pg/mL, leukocytes 8,000 /mm3 w/normal differential, and platelets 18,000 /mm3. A peripheral blood smear shows no abnormalities except for a paucity of platelets. What modality is most likely to yield a diagnosis?

Scintigraphy with 99mTc-labeled heat-damaged erythrocytes

67-year-old woman presents for examination. She mentions that, over the past year, she has found herself becoming fatigued more easily. Physical exam is unremarkable. Results from a blood count reveal marked lymphocytosis; other values are within normal limits. Looking at her medical records, you note that the lymphocytosis has developed over several years. A bone marrow sample reveals infiltration of 40% of the marrow with mature-appearing lymphocytes, which stain positive for CD20. While you do not feel treatment is necessary at this point, what is the most likely complication of this condition?

Serious infection

64-year-old man presents to his PCP for the first time in 4 years with a recent history of fatigue with pallor and worsening back pain in the lumbar region for the past 3-4 months. He denies any injury to his back or similar pain in the past. He had a colonoscopy 3 months ago, and 2 benign polyps were removed without other abnormalities. At that time, he also had a PSA level of 3.1 ng/mL. On examination, BP: 143/92 mm Hg, P: 75 bpm and regular, Resp: 16/minute, Temp: 97.6°F. Skin exam reveals marked pallor without lesions or bruising. Musculoskeletal exam reveals bony tenderness along lumbar vertebrae without paraspinal muscle tenderness on palpation. Decreased range of motion of the spine is noted due to pain elicited during the examination. The remainder of the examination including digital rectal exam, focal neurological, pulmonary, and cardiac exams are within normal limits. What test will be most helpful in making your diagnosis?

Serum protein electrophoresis

11-month-old African American boy presents with an acute onset of anorexia, irritability, unexplained bruising, and jaundice. On examination, you note pale conjunctivae, icteric sclerae, and splenomegaly. Laboratory studies reveal decreased hemoglobin and hematocrit and a significantly elevated reticulocyte count. Hemoglobin electrophoresis reveals the presence of hemoglobin S. What is the most likely diagnosis?

Sickle cell disease

A 9-year-old African American female presents to the emergency room with a 4-hour history of "strange behavior." Her stepmother states that she appears "dazed," and she refuses to walk. She and her siblings have all had "colds" with clear rhinorrhea, sore throats, and non-productive coughs. She has "no appetite," but she has been drinking fluids "fairly well." She has had low-grade fevers up to 99.0°F orally and no history of true fevers. Otherwise, she has been well. Her stepmother denies a history of significant health problems. She states that her daughter takes a daily multivitamin and daily penicillin VK tablet. Her immunizations are up to date. She has a history of normal growth and development. Family history is significant for several family members who have a history of gallstones and a cousin who receives frequent blood transfusions. On exam, patient is a well appearing with no evidence of fluctuating consciousness or cardiorespiratory distress. Vital signs are stable. Her HEENT exam is significant for clear rhinorrhea. Cardiovascular exam demonstrates a 2/6 systolic ejection murmur. Abdominal exam demonstrates a palpable spleen 4 cm below the left costal margin. Her neurologic exam is significant for unilateral, same-sided weakness of her upper and lower extremity. She is unable to move the impaired arm or leg. She demonstrates no evidence of pain with involuntary movement of any of her extremities. The remainder of her exam is unremarkable. Available laboratory studies demonstrate a WBC of 15,000 cells/mc, Hb 10 gm/dL platelet count 250,000 per μl (microliter) of blood and peripheral blood smear demonstrates many target cells and one crescent shaped cell reported. Other laboratory studies are pending at this time. The most likely underlying cause of this patient's signs and symptoms is:

Sickle cell hemoglobinopathy

16-month-old African American boy presents with a 2-day history of irritability and refusal to bear weight. His mother denies any recent history of fever, vomiting, diarrhea, rash, or trauma. Family history is significant for a maternal uncle who had a stroke and died when he was 35. On exam, vital signs are stable. Patient appears smaller than stated age and is irritable but consolable. He is in no apparent distress, but he refuses to bear weight or play. The only significant findings on exam are swollen hands and feet. CBC reveals WBC 18,000 mm3, with 40% neutrophils, 30% lymphocytes, and 1% monocytes. Hb is 8 g/dL and platelets are 400,000 mm3. What test would be helpful in making a diagnosis?

Sickle cell solubility test

63-year-old woman presents with a 2-month history of fatigue. She notes that she has been looking pale and feeling more tired than usual. She denies recent illness or travel, and she states that she has no major medical conditions. Physical exam, chest radiograph, and urinalysis are within normal limits. Basophilic stippling and target cells are seen in the blood smear. There is an increase in serum iron and ferritin, while the total iron-binding capacity and MCV are decreased. What is the most likely cause of this patient's symptoms?

Sideroblastic anemia

55-year-old woman presents with a 4-month history of malaise, abdominal discomfort, nausea, and vomiting; symptoms have persisted despite a strict gluten-free diet. She has a past medical history of long-standing celiac disease, which is confirmed by the presence of antibodies. She states that she has constipation since she introduced a lot of red meat in the diet; because of that, she thinks that her celiac disease might be completely cured. Physical examination reveals a pale slim woman with a tender and distended abdomen. Blood tests show low hemoglobin and hematocrit, with high red blood cell distribution width (RDW); low mean corpuscular volume (MCV) and peripheral smear reveal the presence of microcytic anemia. What should you suggest as the most accurate next diagnostic step?

Small bowel biopsy

29-year-old man presented 3 days ago with sore throat and fever; he was treated with amoxicillin and is now complaining of generalized fatigue, abdominal pain, and maculopapular rash. The patient is sexually active with a single girlfriend who was sick 1 month ago but is now better. Most serious short term complication of the disease is

Spleen rupture

17-year-old boy presents with abdominal pain following a motor vehicle accident. The patient was the passenger in the vehicle and was wearing restraints. He states that he has pain in the left side of his abdomen, which began at the time of the accident. Physical exam reveals a well-developed young man in moderate distress. He has left upper quadrant tenderness, nausea, and positive Kehr's sign. He also has diaphoresis, tachycardia, and hypotension. What is the most likely cause of this patient's symptoms?

Splenic injury

15-year-old boy presents with a 1-week history of malaise, a low-grade fever, and a sore throat. On exam, you note pharyngeal erythema, scant yellow exudates, and enlarged posterior cervical lymph nodes. There are no current signs of airway compromise. There is no skin eruption noted, and the rapid strep screen is negative. What other physical examination finding is usually present with this condition?

Splenomegaly

22-year-old Asian man is presenting to a hematology clinic for the first time to seek management for his alpha thalassemia (hemoglobin H disease type). He has a strong family history of the disorder and was previously being managed by a different hematologist. Today, he receives a thorough history and physical examination, as well as various blood tests. What physical examination finding is most likely present?

Splenomegaly

2-year-old Caucasian boy presents with a 1-month history of decreased energy and increased somnolence. His mother admits that he is a picky eater and that he "loves to eat mattresses." Review of systems reveals that he drinks milk only with cereal and has occasional constipation. His vital signs are within normal limits. You suspect this patient may have iron deficiency anemia. What finding is most supportive of this diagnosis?

Spoon nails: 6 Hb (g/dL), 68 MCV (fL), and decreased reticulocyte count

40-year-old woman presents with fatigue, headache, vision changes, and shortness of breath. Her past medical history is significant for type 2 diabetes and obesity. She states that her symptoms began about 3 days ago and they have steadily worsened.Physical exam reveals an obese woman in mild distress. She has left upper quadrant tenderness to deep palpation. Both the spleen and liver are palpable. The rest of her exam is normal. Laboratory analysis reveals that the patient has an elevated red blood cell volume. The rest of the analysis is within normal limits. What statement regarding the patient's condition is true?

Symptoms may take months or years to develop.

25-year-old woman is sent to a hematologist after she is found to be anemic. With the exception of an enlarged spleen, her physical exam is normal. Laboratory analysis of her blood shows that the mean corpuscular volume (MCV) is decreased, and her serum iron level is elevated. A smear of her blood shows red blood cells with basophilic stippling. What statement regarding this patient's condition is true?

Target cells can be seen in the blood smear.

22-year-old woman is started on methimazole for the treatment of her hyperthyroidism. She has been diagnosed with Graves' disease and has been symptomatic for the last 6 months. She is otherwise healthy and does not take any other medications. You receive a call 2 weeks later from the patient; she tells you that she has had a mild sore throat for the past 2 days and has been feeling like she is coming down with the flu. She states that the symptoms of her hyperthyroidism are a little bit better. What should you do?

Tell her to present ASAP for total and differential blood count.

62-year-old man presents for a follow-up visit. He was diagnosed with chronic lymphocytic leukemia (CLL) 6 months prior to presentation, and he remains asymptomatic at this time. However, he has several younger brothers and sisters, and he is concerned that other members of his family may develop the disease. He wants to know about genetic testing for CLL. What does the most common genetic abnormality associated with CLL involve?

The bcl-2 oncogene

4-year-old boy is taken to the Emergency Department after ingesting household plant fertilizer that he found in his garage. While in the ED, he was found to be lethargic, light-headed, cyanotic, and tachypnic. A sample of his arterial blood was taken and was found to be chocolate brown in color. Laboratory analysis revealed higher than normal levels of methemoglobin in his blood. What is true regarding this patient's condition?

There are acquired and inherited forms of the disease.

46-year-old woman presents with generalized petechiae, left-sided weakness, headaches, mild confusion, nausea, and vomiting. She is febrile, with an oral temperature of 103° F. On exam, she appears mildly confused and has decreased motor strength in the left extremities. Petechiae are scattered over the inner thighs and upper extremities. Significant lab values are as follows: Hgb = 7.2 g/dL, Hct = 22%, WBC = 10.2 x 103/uL, platelet count = 22,000/uL. Peripheral blood smear is remarkable for the presence of schistocytes. Coagulation studies and serum chemistries are unremarkable, with the exception of elevated bilirubin and LDH levels. What is the most likely diagnosis?

Thrombotic thrombocytopenic purpura

38-year-old HIV-positive man presents with fever, headache, right hemiparesis, and expressive aphasia. Recent CD4 count is 45, but he is not on any prophylactic medications. Computed tomography scan (CT) reveals multiple ring-enhancing lesions in the parietal and frontal lobes. What is the most likely cause?

Toxoplasmosis

Your patient is a 65-year-old man with progressive fatigue. His fatigue started -7 months ago, and it is worsening; sometimes, he feels "too tired even to get out of bed". Over the last month, he has started having bone pain, and he has stopped his usual evening walks because of a decreased exercise tolerance. He has also noticed that he is sweating at night. At the same time, he has started having a feeling of abdominal fullness and early satiety; it is accompanied by a constant sensation of bloating, even after a small sandwich or an apple. He has lost approximately 5 kg over the last 6 months. Physical examination reveals liver palpable about 4 cm below the right costal margin, and the spleen is palpable about 8 cm below the left costal margin; the rest of his physical examination is within normal limits. His laboratory analyses are below: Result Finding Range Units WBC (White Blood Cell Count) 39.0 4.0 - 10.80 1000/mcL RBC (Red Blood Cell Count) 5.0 4.7 - 6.1 1000/mcL Differential Neutrophils 60.0 30.0 - 85.0 % Myeloblasts 1 0 % Hb (Hemoglobin) 13.3 9.6 - 18.0 g/dL HCT (Hematocrit) 39.0 32.7 - 57.7 % Platelet Count 150 150 - 350 1000/mcL MCV 88 80 - 94 fL Glucose (FPG) 89 65 - 99 mg/dL Sodium 140 136 - 145 mEq/L Potassium 4.2 3.5 - 5.1 mEq/L Chloride 101 98 - 107 mEq/L Carbon Dioxide (Bicarb) 27.0 22.0 - 29.0 mEq/L Creatinine 1.0 0.6 - 1.3 mg/dL Bilirubin - Total 0.7 0.0 - 1.0 mg/dL Alkaline Phosphatase 100 50 - 136 IU/L AST (SGOT) 25 15 - 37 IU/L ALT (SGPT) 22 15 - 37 IU/L LDH 500 105 - 333 IU/L Uric acid 15 3.5 - 7.2. mg/dL Peripheral blood shows the presence of immature granulocytes and erythrocytes, teardrop-shaped red blood cells, and mild anisopoikilocytosis. Bone marrow from sternum resulted in the dry tap. Bone marrow aspirate and biopsy from iliac crest shows reticulin fibrosis, hypercellularity, increased granulopoiesis and megakaryopoiesis, pleomorphic atypical megakaryocytes, and lymphoid aggregates. His karyotype is normal; JAK2 mutation is positive, and BCR-ABL1 is negative. What will be your therapeutic approach?

Tyrosine kinase inhibitor (Ruxolitinib)

26-year-old man with alcoholic cirrhosis suffers an injury on a shrimp boat in the Gulf of Mexico; his 3rd left finger is crushed under a metal pallet. The patient is treated in the emergency department for a fracture of the middle phalanx, and he is immunized against tetanus. The next day the patient returns to the emergency room; examination reveals a temperature of 38.5° C and hypotension. The patient's wound had become swollen, erythematous, and necrotic. Laboratory results confirm septicemia with a Gram-negative organism; selective media later identifies the etiologic agent. What organism is the most probable cause of the patient's illness?

Vibrio vulnificus

A 55-year-old man presents due to loss of appetite, fatigue, palpitations, and shortness of breath during the exercise. His past medical history is significant for the presence of active tuberculosis, for which he started taking Rifampicin, Isoniazid, Pyrazinamid, and Ethambutol 5 months ago. Physical examination is not remarkable except for the presence of pallor and seborrhoeic dermatitis-like skin changes. Laboratory reveals slight leukocytosis, normal platelets, and anemia (see below). Peripheral smear shows anisocytosis and poikilocytosis with Pappenheimer bodies. Bone marrow shows erythroid hyperplasia, and Prussian blue stain of red blood cells in marrow aspirate shows ringed sideroblasts and the increase of stainable marrow hemosiderin. Result Normal Units Hct 25 41 - 50 % Hgb 10.2 13.5 - 16.5 g/dL MCV 98 80-00 fL MCHC 33.4 32 - 36 g/L RDW 25 < 14.5 Iron 20.6 11.6-13,6 µmol/L Ferritin 380 13 - 300 ng/ml Transferrin saturation 55 20-50 % TIBC 66 43-85 µmol/L What is the most probable cause of his anemia?

Vitamin B6 deficiency

A 35-year-old African-American woman on dialysis has had a 4-month history of hemoglobin of 9 g/dL, despite erythropoietin and intravenous iron therapies. Diagnostic studies have shown the following: Transferrin saturation 30% Ferritin 400 ng/mL Parathyroid hormone level 700 pg/mL (normal for stage 5 kidney disease 150-300 pg/mL) Serum aluminum Normal Total white blood cell count 5x10 3/mm3 Platelet count 200x10 3/mL What therapy should be used in addition to erythropoietin and iron to treat anemia in this patient?

Vitamin D therapy