hematology EOC

A 36-year-old woman is undergoing chemotherapy for her breast cancer. She is very concerned about the risk of neutropenic fever and wonders if the recent appearance of myalgias and a low-grade temperature (38°C) are signs of this. How many days after her chemotherapy session should she experience a nadir in her absolute neutrophil count?

7 days nadir in the absolute neutrophil count (ANC) will occur 5 to 14 days after completion of a chemotherapy session. Neutropenic Fever One oral temperature of ≥ 38.3°C or ≥ 38°C for ≥ 1 hour + neutropenia Leading cause of cancer death: infection Obtain cultures Rx: empiric antibiotics, admission as needed

Basophilic stippling lead poisoning. These hyperdense lines occur at the metaphyses and are most commonly seen in young children on knee and wrist radiographs normocytic, hypochromic anemia however there can also be cases or microcytic anemia in cases of higher lead concentrations. What is the oral chelation medication for lead poisoning? Answer: Succimer. Lead Poisoning Sx: headache, joint pain, and constipation X-ray: hyperdense lines at metaphyses (lead lines) Labs: normocytic, hypochromic anemia and basophilic stippling on peripheral smear Tx: oral succimer or IV EDTA (edetate calcium disodium, given after dimercaprol) Screening thresholds in children > 3.5-19 mcg/dL: exposure history, environmental investigation, assess for iron deficiency anemia 20-44 mcg/dL: as above plus detailed exam assessing for evidence of lead toxicity, consider abdominal X-ray, contact Poison Control Center > 45 mcg/dL: as above plus consider admission for chelation therapy 45 - 69 mcg/dL in patients without vomiting or CNS symptoms can be managed in the outpatient setting with oral succimer (2,3-dimercaptosuccinic acid [DMSA] Insufficient evidence to recommend routine screening in pregnant women

A 3-year-old girl with a past medical history of constipation is brought to the ED for evaluation of a limp and left knee pain. Her physical exam is unremarkable. You obtain the knee radiograph seen above. What do you expect to see on her peripheral blood smear? ABasophilic stippling BHeinz bodies CHypersegmented neutrophils DSchistocytes

Protamine sulfate Blood pressure management is necessary to prevent further expansion of the hematoma. For patients who have been anticoagulated with heparin, protamine sulfate is the reversal agent of choice. This agent is derived from fish sperm and binds to heparin, inactivating the drug. Protamine is administered through slow intravenous infusion to avoid hypotension. Patients should be monitored for signs of allergic reaction with administration. Which agent is preferred over protamine for reversal of anticoagulation when intracranial hemorrhage is associated with low-molecular-weight heparin? Answer: Andexanet alfa. Fresh frozen plasma (FFP) (B) is a plasma product used in the reversal of anticoagulation in warfarin-associated intracranial hemorrhage. Idarucizumab (C) is an anti-dabigatran monoclonal antibody fragment used in the reversal of anticoagulation with dabigatran. This agent may be associated with thrombosis. Vitamin K (E) is given by slow intravenous infusion for emergent reversal of warfarin anticoagulation in patients with intracranial hemorrhage. Infusion is preferred over oral or subcutaneous administration because of the rapid onset of action associated with this administration route.

A 60-year-old woman with a history of metastatic pancreatic cancer presents to the emergency department with chest pain and dyspnea. A CT scan of the chest reveals a large left pulmonary embolism. The patient is stabilized, admitted to the hospital, and started on heparin therapy for anticoagulation. On day 2 of admission, the patient falls while trying to get to the bathroom, striking her head on the sink. A CT scan of her head is completed and is shown above. After stopping heparin therapy, which of the following should be administered for anticoagulation reversal?

Which of the following lab tests will be abnormal in patients with severe hemophilia B? AActivated partial thromboplastin time BBleeding time CD-dimer DProthrombin time What side effect can patients with hemophilia B and inhibitors experience if given factor replacement?

Activated partial thromboplastin time What side effect can patients with hemophilia B and inhibitors experience if given factor replacement? Answer: Anaphylaxis. Hemophilia B (Christmas Disease) X-linked recessive factor IX deficiency Sx: minor trauma causes large amounts of bleeding or hemarthrosis, spontaneous bleeding involving skin, mucous membranes, and joints Labs: prolonged activated partial thromboplastin time (aPTT) and normal prothrombin time (PT) Tx: factor IX concentrate

Acute Myeloid Leukemia (AML) Patient will be an adult Fever, fatigue, anemia, easy bruising or bleeding, petechiae, bone and joint pain, and persistent orfrequent infections PE will show hepatosplenomegaly Labs will show Auer rods, normocytic, normochromic anemia, blast cells

Analysis of a peripheral blood smear reveals a normocytic, normochromic anemia with blast cells and Auer rods. This is most consistent with what diagnosis?

What is the most common location for spontaneous bleeding in children with hemophilia A? AAnkle BElbow CGastrointestinal tract DKnee

Ankle Hemophilia A Factor VIII deficiency resulting in spontaneous bleeding X-linked recessive, males affected disproportionately Sx: easy bruising, hemarthroses Labs: increased PTT corrected with a mixing study Tx: Factor VIII replacement (recombinant or purified concentrate) 80% of hemorrhage occurs in the joints. In children, the most common joint is the ankle. In adults, the most common joint is the knee, followed by the elbow and ankle. What factor deficiency is the cause of hemophilia B? Answer: Factor IX, also known as Christmas disease.

A 4-year-old boy presents to a family health clinic, with a history of fever and upper respiratory infection 4 weeks ago. His cough has disappeared, but his fever has persisted on and off, and his parents report he has lost his appetite. His vitals are BP of 98/56 mm Hg, HR of 110 bpm, RR of 22/min, oxygen saturation of 95%, and T of 101.4°F. He has lost 2 pounds since his physical 5 months ago and shows pallor. Oropharynx shows no erythema or edema. Cervical, axillary, and inguinal lymph nodes are enlarged and matted, and the spleen is palpable and enlarged. Bilateral femurs are tender to palpation. Petechiae are seen on shins. Hemoglobin is 11.5 g/dL, hematocrit 30 L/L, leukocytes 6 K/μL, and platelets 70 K/μL. Chest X-ray is shown above. Which diagnostic test will confirm the most likely diagnosis? ABone marrow evaluation BEpstein-Barr virus serology CImmunoglobulin electrophoresis DKaryotype analysis ELymph node biopsy

Bone marrow evaluation dx: acute lymphoblastic leukemia (ALL) Signs and symptoms include fatigue, pallor, fever, night sweats, widespread lymphadenopathy with matted lymph nodes, hepato- and splenomegaly, and petechiae. Acute Lymphocytic Leukemia (ALL) Patient will be a child Limping, bone pain, fever, and lymphadenopathy PE will show hepatosplenomegaly Labs will show anemia, thrombocytopenia, and lymphoblasts on peripheral smear Most common acute leukemia in children Children with Down syndrome are at a higher risk What is the most common kind of cancer for children aged 5-9? Answer: Brain and CNS cancer.

An 18-year-old man with sickle cell disease presents with chest pain, cough, and fever. His vital signs are HR 132 bpm, RR 28/min, BP 110/65 mm Hg, and T 101.1°F. His chest X-ray shows a right lower lobe infiltrate. His hemoglobin is 8.0 g/dL. Which of the following is the first-line management that is most likely indicated? ABroad-spectrum antibiotics and ICU admission BExchange transfusion COral antibiotics and follow-up with hematology DSplenectomy and broad-spectrum antibiotics

Broad-spectrum antibiotics and ICU admission dx: acute chest syndrome requiring broad-spectrum antibioticsand intensive care unit (ICU) admission. SCD is a genetically determined disease due to an abnormal allele for hemoglobin beta chains. Acute chest syndrome is the leading cause of death in SCD and is defined as fever, chest pain, and the presence of new pulmonary infiltrates. Management consists of supportive care, supplemental oxygen if needed, broad-spectrum antibiotics, and admission to the ICU. complications of sickle cell disease[ in image ] What abnormalities will be seen with hemoglobin and reticulocyte count in aplastic crisis? Answer: Both hemoglobin and reticulocyte count will be depressed.

Which of the following types of leukemia is most associated with the Philadelphia chromosome? How is the Philadelphia chromosome identified? A 57-year-old man presents for several weeks of fatigue, night sweats, and episodic chills. His exam is remarkable for splenomegaly. A complete blood count shows leukocytosis at 175,000 and mild thrombocytosis. The peripheral blood smear shows a left-shifted myeloid series; blasts are less than 5%. Which of the following additional findings is most consistent with your suspected diagnosis?

Chronic myelogenous leukemia How is the Philadelphia chromosome identified? Answer: Cytogenetics or PCR for the BCR-ABL gene product. chromosomes 9 and 22. Chronic Myelogenous Leukemia (CML) Most commonly diagnosed at 30-60 years old Sx: many patients asymptomatic, fatigue, weight loss, abdominal pain or early satiety PE: hepatosplenomegaly Labs: ↑WBC, and low leukocyte alkaline phosphatase (LAP), platelets ↑ or normal Clinical course: chronic phase, accelerated phase, blast crisis. Phase determined by various criteria, include blast percentage, thrombocytopenia, etc. Dx: bone marrow biopsy, Philadelphia chromosome t(9;22), BCR-ABL gene Tx: allogeneic HSCT (curative), tyrosine kinase inhibitors (imatinib) initial treatment of choice for chronic-phase CML is imatinib mesylate (Gleevec®) In addition to an absent Philadelphia chromosome, how can CML be differentiated from the reactive leukocytosis seen with an infectious process? Answer: Reactive leukocytosis will have notably lower white blood cell counts (less than 50,000) and absent splenomegaly.

Which of the following lab findings would you expect to find in a patient with beta-thalassemia? What are three other abnormalities seen on peripheral blood smear in thalassemia?

Codocytes, or target cells What are three other abnormalities seen on peripheral blood smear in thalassemia? Answer: Microcytosis, hypochromia, and basophilic stippling. Beta Thalassemia Major (Cooley anemia) History of failure to thriveMay be picked up on the newborn screen More common in people of Mediterranean or African origin PE will show hepatosplenomegaly Severe disease: thalassemic facies (maxilla hyperplasia, flat nasal bridge, frontal bossing), pathologic bone fractures Labs will show severe anemia (Hgb about 6 g/dL), a decreased MCV, and an increased level of HbF Management includes transfusions, splenectomy, iron chelation

A 56-year-old man presents with intermittent headaches, dizziness, and pruritus, especially after showering, over the past 2 weeks. On examination, his heart rate is 87 bpm, blood pressure is 152/82 mm Hg, and oxygen saturation is 97% on room air. He has splenomegaly without associated abdominal tenderness. His neurologic exam is normal. Which of the following studies is required to diagnose his most likely condition? ABone marrow biopsy BComplete blood count CCoombs test DPeripheral smear

Complete blood count dx: Polycythemia Vera Patient presents with headache, dizziness, pruritus after showering PE will show hypertension, splenomegaly Labs will show increased RBC mass, overproduction of all cell lines, increased Hgb Most commonly caused by mutation of the Janus kinase 2 gene (JAK2) Treatment is phlebotomy, hydroxyurea, aspirin What is the most common cause of secondary polycythemia? Answer: Hypoxia.

A 3-year-old girl is brought in after her mother noticed a rash and bruising over her trunk and extremities. She also has intermittent epistaxis over the past few days. She had an upper respiratory illness two weeks ago but otherwise is well. Examination reveals a well-appearing child with scattered petechiae and mild mucosal bleeding. Her hemoglobin is 12 g/dL, WBC 8,000, INR 1.0, and platelets 8,000. Which of the following is the most appropriate initial treatment?

Corticosteroids Primary Immune Thrombocytopenia - Pediatric child 2-6 years old H/o recent viral infection Red spots on skin or easy bleeding PE: petechiae, purpura, and gingival bleeding platelets < 100,000/µL MCC: antiplatelet antibodies Tx: observation, glucocorticoids and IVIG if severe

A 20-year-old man presents to the clinic with fatigue for the past 6 weeks. He describes constantly feeling tired and being unable to focus on work. His weight at his last visit 6 months ago was 180 pounds, and his weight today is 150pounds. Vital signs include a HR of 98 bpm, BP of 120/80 mm Hg, RR of 20/min, oxygen saturation of 98% on room air, and T of 100.6°F. Physical examination is significant for nontender anterior cervical lymphadenopathy and excoriations on his trunk. Which of the following is the most appropriate test to confirm the suspected diagnosis?

Excisional lymph node biopsy dx: Hodgkin lymphoma Hodgkin Lymphoma Patient presents with lymphadenopathy (often cervical and painless), pruritus, fever, night sweats, unintentional weight loss, and frequent infections Diagnosis is made by lymph node biopsy showing Reed-Sternberg cells

A 27-year-old man with a history of von Willebrand disease presents for epistaxis. His bleeding is difficult to control. Which of the following is first line treatment to help decrease bleeding?

Desmopressin Von Willebrand Disease Most common inherited bleeding disorder due to reduced, dysfunctional, or absent von Willebrand factor Sx: increased mucocutaneous bleeding, heavy menses, excessive postpartum bleeding Labs: aPTT may be prolonged, VWF antigen, platelet-dependent VWF activity (ristocetin cofactor assay), factor VIII activity Tx options: desmopressin (DDAVP), von Willebrand factor concentrate Most cases autosomal dominant, consider genetic counseling and testing of 1st degree relatives

A 43-year-old woman with a history of von Willebrand disease presents with epistaxis. Vital signs are normal, and examination reveals oozing from the right naris despite applying pressure. Which treatment is indicated in this patient's management?

Desmopressin dx: Von Willebrand Disease Most common inherited bleeding disorder due to reduced, dysfunctional, or absent von Willebrand factor Sx: increased mucocutaneous bleeding, heavy menses, excessive postpartum bleeding Labs: aPTT may be prolonged, VWF antigen, platelet-dependent VWF activity (ristocetin cofactor assay), factor VIII activity Tx options: desmopressin (DDAVP), von Willebrand factor concentrate Most cases autosomal dominant, consider genetic counseling and testing of 1st degree relatives Is the patient's platelet count depressed, normal, or increased in von Willebrand disease? Answer: Normal.

A 54-year-old woman presents to discuss options for managing her chronic urticaria. She has tried a broad range of elimination approaches, including avoidance of hot showers, cold, pressure, and multiple suspected allergens. She has also tried a number of antihistamine combinations with minimal results. Which of the following medications would be most promising for this patient?

Doxepin, a tricyclic antidepressant, is approved and can be very effective for managing chronic urticaria that is refractive to treatment with antihistamines. What side effects are typically caused by medications with anticholinergic properties? Answer: Blurry vision, dry eyes, dry mouth, urinary retention, and constipation. Urticaria PE: transient blanching, edematous papules, and plaques Etiology: IgE-mediated reaction to an allergen Treatment: supportive care, antihistamines, glucocorticoids (if associated with angioedema) for many patients, the workup for chronic urticaria will include a long process of elimination to rule out any environmental or dietary triggers, as well as ruling out chronic viral infectious triggers, such as viral hepatitis. Antihistamine H1-antagonists are considered first-line therapy. Dosages may be increased and combinations of antihistamines may be used if initially ineffective. H2-blockers more commonly used for gastroesophageal reflux may be combined with an H1-antagonist to offer additional benefit. Doxepin also has antihistamine properties and is usually very effective, though it poses the drawback of having anticholinergic side effects and would require daily use. UVB phototherapy may also be helpful.

A 14-year-old boy is brought by his grandparents to the Emergency Department due to difficulty breathing. His grandmother notes that the boy was eating his snack of salted peanuts and an apple when he started to complain of generalized itchiness especially around his mouth, face, and extremities, and swelling around his eyes. Physical examination reveals an irritable child in respiratory distress with periorbital edema, tonsillopharyngeal congestion, poor air entry on lung auscultation, and multiple erythematous and raised wheals on the trunk and extremities. Which of the following is the most important medication in treating this child?

Epinephrine dx: anaphylaxis What is the most common cause of anaphylaxis occurring outside the hospital? Answer: Food allergy. Anaphylaxis Preexisting IgE antibodies → mast cell degranulation → shock, airway compromise Leading cause of fatal anaphylaxis: penicillin Epinephrine Adults: 0.3 to 0.5 mL 1:1,000 (1 mg/mL) solution IM q5-15 minutes Children: 0.01 mg/kg 1:1,000 (1 mg/mL) solution IM q5-15 minutes ​Refractory hypotension in a patient on beta-blockers: glucagon Adjunctive medicines include: histamine H1 and H2 antagonists, corticosteroids, beta2 agonists, and glucagon

A patient suffers from recurrent deep vein thromboses. Which of the following is the most likely diagnosis?

Factor V Leiden is the most common inherited hypercoagulability disorder, Factor V Leiden Most commonly caused by an autosomal dominant genetic disorder Most common inherited hypercoagulability disorder

A 34-year-old woman with a history of seizures presents to the office reporting symptoms of progressive fatigue for the past 6 months. She is taking phenytoin and has not had any seizures in over a year. Her temperature is 98.7°F, blood pressure is 118/82 mm Hg, heart rate is 92 bpm, oxygen saturation is 98% on room air, and respiratory rate is 20/min. She has some skin pallor and mild gingival hyperplasia on physical exam. Laboratory studies reveal a hemoglobin of 10.0 g/dL and a mean corpuscular volume of 118 fL. Supplementation with which of the following could have prevented this condition? ACobalamin BFolic acid CPyridoxine DRiboflavin EThiamine

Folic acid

A 43-year-old man with a history of alcohol use disorder presents with generalized weakness. Physical examination reveals pale conjunctiva. The patient's hematocrit is 25%, and his mean corpuscular volume is 110 fL. Which of the following treatments is most likely indicated?

Folic acid supplementation

An 82-year-old man with a history of hypertension, atrial fibrillation, and deep vein thrombosis presents to the emergency department for weakness, fatigue, and dark, tarry stools for the past several days. His current medications include amlodipine, metoprolol, and warfarin. Vital signs reveal a temperature of 36.9°C, blood pressure of 91/59 mm Hg, heart rate of 115 bpm, respiratory rate of 17/min, and SpO2 of 98% on room air. Physical exam reveals an acutely ill-appearing man with an abdomen that is soft, nontender, and nondistended to palpation in all four quadrants. Stool guaiac exam is positive for occult blood. The remainder of his physical exam is normal. Labs reveal WBC 5,000/µL, hemoglobin 8 g/dL, platelets 200,000/µL, INR 8, and fibrinogen 200 mg/dL. Prothrombin complex concentrate is not available for administration. Which of the following is the best next immediate treatment for this patient?

Fresh frozen plasma Deficiency of which factors causes a prolonged activated partial thromboplastin time (aPTT)? Answer: Factors 8, 9, 11, and 12.

Which of the following contributes to immune deficiency in patients with sickle cell disease? ABone marrow hyperplasia BDelayed puberty CFunctional asplenia DVaso-occlusive crises

Functional asplenia True or false: prophylactic penicillin should be given to all children with sickle cell disease until age 5. Answer: True. Sickle Cell Disease Sickling → vaso-occlusive ischemia Hydroxyurea increases fetal hemoglobin (HbF) production, reduces chance of sickling ↓ O2, dehydration, acidosis → sickling Crizanlizumab -> to reduce frequency of vaso-occlusive crises Newborns: initially asymptomatic (due to ↑ HbF) Most common presentation in infants: dactylitis Aplastic crisis: ↓ Hgb + reticulocytopenia, parvovirus B19 Acute chest syndrome Most common cause of death in adults Fever, CP CXR: pulmonary infiltrate Splenic sequestration crisis: rapid splenic sequestration of RBCs → splenomegaly + severe anemia Stroke Aseptic necrosis of the femoral head Dysfunctional spleen → ↑ infection risk Salmonella osteomyelitis S. pneumoniae sepsis: most common cause of death in children

A 9-year-old boy presents to the emergency department with his parent, who reports the patient has had yellowing of the skin, dark urine, and dizziness that developed quickly over the past 24 hours. While you are obtaining the history, the parent mentions the boy recently recovered from an upper respiratory infection but is otherwise healthy. Current vital signs are temperature of 98.7°F, BP of 95/70 mm Hg, HR of 114 bpm, RR of 20/minute, and oxygen saturation of 99% on room air. Physical exam shows jaundice and is otherwise unremarkable. Laboratory studies reveal a hemoglobin of 10.2 g/dL. Bite cells and Heinz bodies are seen on peripheral smear. Which of the following is the most likely diagnosis?

Glucose-6-phosphate dehydrogenase deficiency G6PD Deficiency History of taking antimalarials, sulfonylureas, quinolones, nitrofurantoin, fava beansInfection is also a cause for the hemolysis Labs will show Heinz bodies, presence of bite cells on the smear Consider testing prior to starting potential agents in patients who may be at risk X-linked recessive Why should patients with glucose-6-phosphate dehydrogenase avoid fava beans? Answer: Fava beans are high in glucosides (divicine and convicine), which are damaging to RBCs. . Medications that may trigger an episode of hemolytic anemia in patients with G6PD deficiency include sulfa drugs, sulfonylureas, nitrofurans, dapsone, phenazopyridine, methylene blue, isoniazid, antimalarials, fluoroquinolones, and chloramphenicol. Symptoms range from mild to severe and typically include jaundice and dark urine due to increased indirect bilirubin in the blood secondary to the breakdown of RBCs. Dizziness, nausea, headache, and acute kidney injury may also be seen.

Which of the following types of hemoglobin does hydroxyurea increase?

HbF What are factors that promote sickling in sickle cell disease? Answer: Cold, dehydration, hypoxia, infection, stress, menses, alcohol consumption.

A 12-year-old girl presents for her annual well child check. She had menarche eight months prior, and has had menses every month. Each cycle lasts two weeks. The patient uses at least six pads on most days of the cycle. On exam, she is pale but otherwise well appearing. Her heart rate is 80 beats per minute, blood pressure 110/65, and respiratory rate 14. The remainder of her exam is within normal limits. Which of the following are the most likely laboratory findings? AHemoglobin 12 g/dL, mean corpuscular volume 80, RDW 10 BHemoglobin 6 g/dL, mean corpuscular volume 60, RDW 12 CHemoglobin 6 g/dL, mean corpuscular volume 60, RDW 18 DHemoglobin 8 g/dL, mean corpuscular volume 100, RDW 10

Hemoglobin 6 g/dL, mean corpuscular volume 60, RDW 18 dx: iron deficiency anemia secondary to dysfunctional uterine bleeding. Iron deficiency anemia is characterized by a microcytic anemia (low hemoglobin and low MCV). Iron Deficiency Anemia Sx: weakness, dizziness, and fatigue PE: pallor, tachycardia, atrophic glossitis, or koilonychia (spoon nails) Labs: microcytic, hypochromic red blood cells, decreased serum iron level, an increase in the total iron binding capacity (TIBC), and decreased serum ferritin levels Most common cause of microcytic anemia

A 14-month-old boy presents to the emergency department with his adoptive parent, who reports that he has had increased irritability with episodes of inconsolable crying for the past 3 hours with no history of injury. He has had a recent upper respiratory tract infection with a runny nose and cough for the past week. His parent notices that he cries more when he is walking and that he is easily fatigued. His parent adopted him about 6 months ago and does not have a record of his medical history. His temperature is 98.7°F, blood pressure is 105/64 mm Hg, heart rate is 106 bpm, oxygen saturation is 98% on room air, and respiratory rate is 25/min. On a physical exam, the patient is ill-appearing, irritable, and crying inconsolably. He has evidence of scleral icterus and has swelling to both hands and both feet with tenderness to palpation to the dorsal surfaces. His hemoglobin is 8.2 g/dL. Which of the following tests will establish the patient's diagnosis?

Hemoglobin electrophoresis Sickle Cell Disease Symptom onset typically occurs during the first year of life. The disease can affect almost every organ and can cause delayed puberty. Chronic hemolytic anemia causes jaundice, pigment gallstones, splenomegaly, and poorly healing ulcers, particularly over the lower tibia. Severe anemia may occur during hemolytic or aplastic crises. Sickle cell disease is also characterized by recurrent episodes of pain due to acute vaso-occlusion from clustered sickle red cells. These spontaneous episodes are self-limiting, may last several hours to days, and are triggered by infection, dehydration, or hypoxia. Patients will report pain in multiple sites, including the spine and long bones (e.g., tibia, humerus, femur) due to bone infarction. Pain crises are also occasionally accompanied by a low-grade fever. Hand-foot syndrome is characterized by painful swelling of the dorsa of the hands and feet and is seen in infancy and early childhood, ------------------------------------------------------------- Osmotic fragility testing (D) is used to test the ability of red blood cells to swell and rupture in hypotonic solutions and is used to diagnose conditions such as hereditary spherocytosis. Schilling test (E) is a test that was used to diagnose vitamin B12 deficiency but is no longer used since a serum vitamin B12 level is now the test of choice.

A 5-year-old boy presents to the clinic with concerns for fatigue and pallor. His family history is positive for his father having a "blood disorder." Physical exam reveals a palpable spleen. Laboratory studies reveal a normocytic anemia. Reticulocyte count and mean corpuscular hemoglobin concentration are increased. Which of the following is the most likely diagnosis?

Hereditary spherocytosis Hereditary Spherocytosis Patient presents with symptoms of anemia: fatigue or pallor Positive family history Labs will show microcytic or normocytic anemia and is the only disorder that will cause an increase in mean corpuscular hemoglobin concentration (MCHC) Peripheral smear will show spherocytes and Howell-Jolly body Most commonly caused by an autosomal dominant genetic disease Treatment includes daily folic acid What result would you expect if you ordered bilirubin testing in hereditary spherocytosis? Answer: Increased serum indirect bilirubin.

Which of the following characteristics of transfusion-related acute lung injury (TRALI) differentiates it from transfusion-associated circulatory overload (TACO)?

High fever Fever is not common in transfusion-associated circulatory overload (TACO). Transfusion Complications Massive transfusion: coagulopathy, hypothermia, hypocalcemia Febrile reaction: most common complication, fever or chills Hemolytic reaction: ABO incompatibility, immediate fever or chills, HA Rx: stop transfusion, IVF, diuretics Allergic reaction: urticaria or hives TRALI: like ARDS Rx: stop transfusion Delayed reaction: 3-4 weeks after transfusion, decreased Hgb GVHD: immunocompromise, rash, pancytopenia, increased LFTs Prevention: irradiated blood products in immunocompromised

A 17-year-old boy presents to the ED with a painless mass on the left side of his neck, which he first noticed three months ago, that is now increasing in size. The patient also reports a 10-pound weight loss and night sweats. On exam, you note a 5 cm rubbery, firm, and mobile mass located in the anterior cervical triangle. The overlying skin is intact, with no redness, streaking, or drainage. What is the most likely diagnosis?

Hodgkin lymphoma most common malignancy of the head and neck in children. Lymphoma Non-Hodgkin lymphomaMultiple, peripheral nodes; noncontiguous spreadExtranodal involvementHIV and autoimmune associationMost common hematopoietic neoplasm Associated with EBV Hodgkin lymphoma Localized, single group of nodes; contiguous spread Bimodal age distribution: 15-35 and > 55 B symptoms (fevers, night sweats, weight loss) Reed-Sternberg cells Associated with EBV What virus is most commonly associated with Burkitt lymphoma? Answer: Epstein-Barr virus.

A 54-year-old man presents to the clinic with abnormal laboratory results. He is currently taking warfarin for chronic atrial fibrillation. His INR was found to be 4.9 today. He denies any bleeding or other symptoms at this time. He has not changed his diet, and his only other medication is bisoprolol. Which of the following is the most appropriate management of this patient?

Hold warfarin temporarily For patients with an asymptomatically elevated INR that is less than 10, the current recommendation is to hold warfarin temporarily. For major bleeding, the current recommendation is to administer prothrombin complex concentrate (PCC) Reversal of warfarin in patients with life-threatening bleeding is characteristically done with fresh frozen plasma or prothrombin complex concentrate. Warfarin Oral anticoagulant Blocks synthesis of II, VII, IX, X (clotting factors), protein C and S Monitor for bleeding Monitor for drug interactions Monitor skin for necrosis Monitor PT and INR Reverse with vitamin K, consider four-factor prothrombin concentrate or fresh frozen plasma (FFP) for any life-threatening bleed

An 18-year-old African-American man presents with questions about his diagnosis of sickle cell anemia. He has been managing his condition since childhood by treating his symptoms and having occasional blood transfusions. Now he is experiencing more frequent painful episodes and wants to know if there are other treatment options. Which of the following is the most appropriate pharmacologic treatment? How is sickle cell disease diagnosed?

Hydroxyurea Sickle Cell Disease Sickling → vaso-occlusive ischemia Hydroxyurea increases fetal hemoglobin (HbF) production, reduces chance of sickling ↓ O2, dehydration, acidosis → sickling Crizanlizumab -> to reduce frequency of vaso-occlusive crises Newborns: initially asymptomatic (due to ↑ HbF) Most common presentation in infants: dactylitis Aplastic crisis: ↓ Hgb + reticulocytopenia, parvovirus B19 Acute chest syndrome Most common cause of death in adults Fever, CP CXR: pulmonary infiltrate Splenic sequestration crisis: rapid splenic sequestration of RBCs → splenomegaly + severe anemia Stroke Aseptic necrosis of the femoral head Dysfunctional spleen → ↑ infection riskSalmonella osteomyelitis S. pneumoniae sepsis: most common cause of death in children How is sickle cell disease diagnosed? Answer: Hemoglobin electrophoresis.

A 26-year-old woman presents with worsening fatigue over the last 2 weeks. She became concerned today when she noticed tingling in her feet bilaterally. She changed her diet 5 years ago and became a vegan. Which of the following would you expect to find on her laboratory studies?

Increased mean corpuscular volume dx: megaloblastic anemia Vitamin B12 is only found in foods of animal origin. Symptoms of B12 deficiency include those typical of anemia (e.g. fatigue, pallor, dyspnea with exertion) as well as neurologic symptoms (e.g. paresthesias of the distal extremities, decreased proprioception, and weakness of the lower extremities). Laboratory studies will show a macrocytic anemia characterized by an increased mean corpuscular volume (MCV) as well as a decreased B12 level. Treatment consists of supplementation with parenteral therapy in cases due to malabsorption or oral therapy in cases of dietary deficiency. Vitamin B12 (Cobalamin) Deficiency Risk Factors: vegan diet, metformin use Sx: fatigue, weakness, and peripheral neuropathy PE: pallor and glossitis Labs: MCV > 100 fL, hypersegmented neutrophils, elevated homocysteine, elevated methylmalonic acid Tx: parenteral vitamin B12, oral considered if no neurologic sx or malabsorption Neuropathy is more common with vitamin B12 deficiency (as opposed to folate deficiency) Pernicious anemia: autoimmune destruction of cells that produce intrinsic factor (IF), resulting in vitamin B12 deficiency What tapeworm infection is associated with vitamin B12 deficiency? Answer: Diphyllobothrium latum infection causes vitamin B12 malabsorption. What are other causes of macrocytic anemia not related to megaloblastic changes? Answer: Liver disease (most common) and hypothyroidism.

A 12-month-old girl is in the clinic for a well-child check. She can walk and is eating more table foods. The parents have given her goat's milk for two months. Physical examination is normal. Laboratory screening obtained shows hemoglobin of 10 g/dL, mean corpuscular volume 77 fL and elevated red cell distribution width. Which of the following is the most likely diagnosis?

Iron deficiency anemia Sx: weakness, dizziness, and fatigue PE: pallor, tachycardia, atrophic glossitis, or koilonychia (spoon nails) Labs: microcytic, hypochromic red blood cells, decreased serum iron level, an increase in the total iron binding capacity (TIBC), and decreased serum ferritin levels Most common cause of microcytic anemia What is the most common nutritional deficiency in children? Answer: Iron deficiency.

A patient who is undergoing a transesophageal echocardiogram suddenly develops profound hypoxemia and cyanosis. His respiratory rate is 18 breaths/minute, but his saturation remains at 80% despite adequate application of supplemental oxygen by non-rebreather mask. A quick review of the medication record reveals that he received benzocaine, fentanyl, midazolam, and propofol during the procedure. What substance should you administer to treat his underlying condition?

Methylene blue dx: methemoglobinemia Methemoglobinemia Fe3+ → Hgb can't bind O2 Leftward shift of oxyhemoglobin dissociation curve Pulse ox in high 80s, Normal PaO2, unresponsive to O2, SOB + clear lungs on PE + normal CXR Cyanosis Brown blood Causes: dapsone, nitrates, antimalarials, -aines, naphthalene (moth balls) Treatment: Methylene blue benzocaine, can disrupt this pathway, leading to an accumulation of methemoglobin and reducing the oxygen-carrying capacity of the blood. It may also alter the appearance of the arterial blood and make it appear chocolate colored. To confirm methemoglobinemia, an arterial blood gas with co-oximetryis needed to show the levels of methemoglobin and oxyhemoglobin. The classic finding in methemoglobinemia is a pulse oximetry reading around 80-85% despite administration of supplemental oxygen. What is the normal level of methemoglobin in arterial blood? Answer: Less than 2%.

A 75-year-old man on chemotherapy for metastatic prostate cancer presents with a fever. His white blood cell count is 1,200 cells/microL with 22% neutrophils, 44% lymphocytes, 8% monocytes, and 9% bands. Which of the following mandates antibiotic treatment?

Oral temperature of 101°F measured once dx:neutropenia is a known complication due to suppression of bone marrow production of all cell lines. The Infectious Disease Society of America defines neutropenic fever as a single oral temperature measurement of 101°F (38.3°C). Additionally, two oral temperature measurements measured at least one hour apart of 100.4°F (38°C) is also considered a fever. In neutropenic fever patients with a bacterial infection, what bacteria are the most common source? Answer: Gram positives.

A two-year-old boy develops pallor following a viral upper respiratory tract infection. His mother reports a family history of "some kind of low blood levels". His serum studies reveal a normocytic anemia and the presence of spherocytes on the peripheral blood smear. Which of the following tests can be done to confirm the diagnosis?

Osmotic fragility test dx: Hereditary spherocytosis Patient presents with symptoms of anemia: fatigue or pallor Positive family history Labs will show microcytic or normocytic anemia and is the only disorder that will cause an increase in mean corpuscular hemoglobin concentration (MCHC) Peripheral smear will show spherocytes and Howell-Jolly body Most commonly caused by an autosomal dominant genetic disease Treatment includes daily folic acid Children with hereditary spherocytosis are at risk for aplastic crisis as result of an infection from which virus? Answer: Parvovirus B19.

Which of the following hematological disorders is characterized by intermittent venous and arterial thrombosis, splenomegaly, and abnormal proliferation of all three myeloid cell lines? What disease is suggested by an increased hemoglobin concentration, leukocytosis, and thrombocytosis? A 60-year-old man presents to your office complaining of generalized pruritus following warm showers. He also states that he has been fatigued and had several nosebleeds in the past month. Physical exam reveals splenomegaly and engorged retinal veins. You suspect polycythemia vera. Which of the following findings will confirm that your diagnosis?

Polycythemia vera JAK2 Polycythemia Vera Patient presents with headache, dizziness, pruritus after showering PE will show hypertension, splenomegaly Labs will show increased RBC mass, overproduction of all cell lines, increased Hgb Most commonly caused by mutation of the Janus kinase 2 gene (JAK2) Treatment is phlebotomy, hydroxyurea, aspirin Which gastric hormone is hypersecreted, resulting in peptic ulcer disease and pruritus, a common complication of polycythemia vera? Answer: Histamine. What is the prognosis for polycythemia vera? Answer: Median survival from treatment to death is 9-14 years.

A 49-year-old man presents with mental status changes and fever. Brain CT scan is normal. His urine toxicology screen is negative. Laboratory findings show a serum sodium of 137 mEq/L, serum potassium of 5.1 mEq/L, serum creatinine of 1.9 mg/dL, WBC of 9,000/L, hemoglobin of 9.3 g/dL, and platelets of 19,000/L. Which of the following would you expect to see on skin examination?

Purpura Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are microvascular thrombotic disorders They both present with microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, but TTP may have concurrent neurologic abnormalities and fever. Both diseases are associated with thrombocytopenia that often leads to purpura, petechiae, and bleeding. HUS usually occurs in children 5-10 days after an episode of E. coli-induced bloody diarrhea, in which the Shiga toxin activates platelets and renovascular cells, leading to thrombosis. TTP usually occurs in adults TTP and HUS HUS E. coli O157:H7Autoimmune hemolysis, renal failure, thrombocytopenia (ART)Bloody diarrhea Avoid Abx TTPART + fever + neurologic abnormalities (FARTN)Normal coagulation studiesPlasmapheresis

Which of the following indices reported in a standard complete blood count panel is most helpful to differentiate between iron deficiency anemia and thalassemia?

Red blood cell distribution width measure of the deviation in volume of the RBCs. It is calculated bydividing the standard deviation of the MCV by the mean MCV and multiplying by 100. RDW = (SD MCV / mean MCV) x 100 What red blood cell disorder has a characteristic increase in mean corpuscular hemoglobin concentration? Answer: Spherocytosis.

A 43-year-old woman on chemotherapy for breast cancer presents with a fever to 102°F. She also complains of a cough and generalized fatigue. Physical examination and chest X-ray are unremarkable except for the presence of a mediport. Complete blood count reveals a white blood cell count of 600/µL with 30% neutrophils and no band forms. What management is indicated?

Send blood and urine cultures and start vancomycin and cefepime Neutropenic Fever One oral temperature of ≥ 38.3°C or ≥ 38°C for ≥ 1 hour + neutropenia Leading cause of cancer death: infection Obtain cultures Rx: empiric antibiotics, admission as needed What is the most common gram-negative pathogen identified in neutropenic fever? Answer: Pseudomonas aeruginosa.

A 67-year-old man presents to the family medicine clinic for worsening low back pain for the past 6 weeks. The patient reports no recent trauma or heavy lifting. He currently takes lisinopril 20 mg daily for hypertension and has no other relevant medical history. Current vital signs are temperature of 99.6°F, BP of 110/78 mm Hg, HR of 94 bpm, RR of 18/minute, and oxygen saturation of 97% on room air. A physical exam reveals moderate tenderness along the lumbar spinous processes. The patient appears fatigued and has a generalized pallor. Laboratory studies reveal hemoglobin of 9.8 g/dL, calcium of 12.5 mg/dL, and creatinine of 1.4 mg/dL. Which of the following is the most appropriate next step in evaluation?

Serum protein electrophoresis dx: Multiple myeloma is a malignancy of the hematopoietic stem cells proliferation of plasma cells and producing a monoclonal immunoglobulin. plasma cells into the bone marrow leads to lytic lesions, bone pain, pathologic fracture, and end-organ damage. . Hypercalcemia is often present due to destruction of bone. Bone pain, elevated creatinine, fatigue, weight loss, and increased rate of infections are also commonly seen. Multiple Myeloma Patient will be older Back pain Signs and symptoms: CRAB: hypercalcemia, renal insufficiency, anemia, lytic bone lesions or back pain Labs will show monoclonal antibody spike X-ray will show lytic lesions Peripheral blood smear: rouleaux formations Serum protein electrophoresis: M spike Protein electrophoresis urine analysis: Bence Jones proteins Most commonly caused by single clone plasma cell malignancy

A 3-year-old boy is undergoing a diagnostic work-up for a hereditary cause of a moderate anemia. Lab studies reveal a microcytic anemia and an increased reticulocyte count. Labs also reveal hyperbilirubinemia and an increased mean corpuscular hemoglobin concentration. An osmotic fragility test is abnormal. Which of the following may be eventually considered as a treatment option for this disorder?

Splenectomy dx:Hereditary spherocytosis is an autosomal dominant diseasethat causes an abnormal shape to the red blood cell (RBC). What RBC membrane proteins are defective in hereditary spherocytosis? Answer: Spectrin or actin. Hereditary Spherocytosis Patient presents with symptoms of anemia: fatigue or pallor Positive family history Labs will show microcytic or normocytic anemia and is the only disorder that will cause an increase in mean corpuscular hemoglobin concentration (MCHC) Peripheral smear will show spherocytes and Howell-Jolly body Most commonly caused by an autosomal dominant genetic disease Treatment includes daily folic acid

A 24-year-old woman presents to the office reporting progressive fatigue for the past several months. She is otherwise healthy and not on any medications. Her menstrual cycles are regular, lasting between 3 and 5 days, and she uses about five or six regular tampons per day. Her temperature is 98.6°F, blood pressure is 112/75 mm Hg, heart rate is 85 bpm, oxygen saturation is 98% on room air, and respiratory rate is 22/min. A physical exam is unremarkable. Her CBC reveals a hemoglobin of 10.2 g/dL and a mean corpuscular volume of 72 fL. The patient is prescribed daily oral ferrous sulfate and returns for a follow-up in 6 weeks. A repeat CBC is unchanged. Which of the following is the most likely diagnosis?

Thalassemia A CBC will reveal anemia with red blood cells that are microcytic (low mean corpuscular volume [MCV]) and hypochromic (due to low mean corpuscular hemoglobin [MCH]). Additionally, there may be an elevated reticulocyte count and a normal to elevated red blood cell count due to an increased number of small and pale red blood cells being produced. The mild forms of thalassemia are commonly mistaken for iron deficiency anemia. Iron deficiency anemia can be ruled out with an unsuccessful trial of iron therapy, like this patient, or with further iron studies, which will be normal or reveal an elevated transferrin saturation or ferritin. Mild thalassemia does not require treatment. Those with severe thalassemia usually require regular blood transfusions, folic acid supplementation, and in some cases, splenectomy or allogeneic stem cell transplantation. dx: Thalassemias Mediterranean, African, Asian origin Microcytic, hypochromic Mutations and deletions: severity Beta-thalassemia major: crew-cut skull X-ray, chipmunk facies, Frequent transfusions: Fe toxicity What treatment is required to prevent organ damage from iron overload in patients with regular blood transfusions? Answer: Oral or parenteral iron chelation therapy.

A 40-year-old woman presents to the emergency department for confusion and headache. She has a past medical history of seasonal allergies and hypothyroidism. Her pulse is 78 beats/minute, respiratory rate is 18 breaths/minute, blood pressure is 141/89 mm Hg, and the temperature is 101.1°F. Physical exam is remarkable for scattered petechiae. Laboratory findings include hemoglobin of 10 g/dL, platelet count of 25,000/microL, creatinine of 1.2 mg/dL, and normal fibrinogen. A peripheral blood smear is notable for multiple schistocytes. Which of the following is the most likely diagnosis? ADisseminated intravascular coagulation BHemolytic uremic syndrome CIdiopathic thrombocytopenic purpura DThrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura Thrombotic Thrombocytopenic Purpura (TTP) Risk factors: female sex, age < 50, pregnancy Sx: fever, confusion, difficulty speaking, headache, seizure, nausea, vomiting, diarrhea Labs: elevated LDH, elevated indirect bilirubin, normal coagulation studies, microangiopathic hemolytic anemia, and thrombocytopenia Caused by autoantibody against ADAMTS13 leading to severe deficiency of the enzyme Treatment is plasma exchange with intravenous corticosteroids Which drugs are associated with an increased risk of thrombotic microangiopathy? Answer: Bleomycin, cyclosporine, mitomycin, cisplatin, ticlopidine, and quinine.

A 15-year-old girl is in your clinic with her mother for a sports physical examination. She denies any symptoms and is doing well in school. There is no family history of sudden death or heart disease. She has been a vegan for the past year and takes food supplements. She has regular menstrual periods. Physical examination is normal. You perform a screening complete blood count (CBC) that reveals hemoglobin of 10 g/dL, mean corpuscular volume (MCV) of 68, with ferritin of 11 ng/mL (normal for females 12-150 ng/mL). Which of the following is the next best step in management?

Trial treatment with oral iron supplements dx: Iron Deficiency Anemia Sx: weakness, dizziness, and fatigue PE: pallor, tachycardia, atrophic glossitis, or koilonychia (spoon nails) Labs: microcytic, hypochromic red blood cells, decreased serum iron level, an increase in the total iron binding capacity (TIBC), and decreased serum ferritin levels Most common cause of microcytic anemia What is the test to order as screening for iron deficiency anemia as recommended by the American Academy of Pediatrics? Answer: Hemoglobin. Starting parenteral iron (C) should be reserved for patients with severe, persistent anemia who have proven intolerance to oral supplements, malabsorption, or poor compliance to oral therapy, which is not the case for the girl in the vignette.

A 30-year-old woman presents to the clinic to obtain preoperative clearance for her elective cholecystectomy. She has a history of a bleeding disorder, diagnosed when she was a teenager. Her symptoms include easy bruising, prolonged bleeding after prior dental extractions, and heavy menstrual bleeding. Other family members have the same bleeding disorder. Laboratory evaluation reveals a normal platelet count, decreased platelet function, normal prothrombin time, a normal activated partial thromboplastin time, and a prolonged bleeding time. Which of the following is the most likely diagnosis?

Von Willebrand disease symptoms often experience easy bruising, skin bleeding, and prolonged bleeding from mucosal surfaces History of heavy menstrual bleeding Laboratory testing often reveals a normal platelet count, a normal prothrombin time, and a normal activated partial thromboplastin time, Von Willebrand Disease Most common inherited bleeding disorder due to reduced, dysfunctional, or absent von Willebrand factor Sx: increased mucocutaneous bleeding, heavy menses, excessive postpartum bleeding Labs: aPTT may be prolonged, VWF antigen, platelet-dependent VWF activity (ristocetin cofactor assay), factor VIII activity Tx options: desmopressin (DDAVP), von Willebrand factor concentrate Most cases autosomal dominant, consider genetic counseling and testing of 1st degree relatives What role does von Willebrand factor play in primary hemostasis? Answer: It binds to both platelets and endothelial components, forming an adhesive bridge between platelets and vascular subendothelial structures at sites of endothelial injury.

A 35-year-old woman with a history of schizophrenia presents to your office with a complaint of auditory hallucinations and suicidal ideation. The patient has tried several second-generation antipsychotics without relief from her symptoms. You decide to prescribe clozapine. Which of the following tests should be conducted on a recurrent basis to monitor this patient? What conditions are relative contraindications to the use of clozapine?

White blood cell and absolute neutrophil count white blood cell monitoring for agranulocytosis must occur at specific intervals What conditions are relative contraindications to the use of clozapine? Answer: Seizure disorder, pre-existing neutropenia, and heart disease. Clozapine Agranulocytosis Seizures Myocarditis Increased mortality in older patients with dementia-related psycho

A 16-year-old boy presents with a marked hemarthrosis of his knee after tripping and striking it against the edge of a step. This has happened in the past. His activated partial thromboplastin time is prolonged and his prothrombin time and thrombin time are normal. What is the most likely diagnosis? What medication is recommended before dental procedures in high-risk individuals in order to prevent the breakdown of blood clots?

dx: Hemophilia B (Christmas Disease) X-linked recessive factor IX deficiency Sx: minor trauma causes large amounts of bleeding or hemarthrosis, spontaneous bleeding involving skin, mucous membranes, and joints Labs: prolonged activated partial thromboplastin time (aPTT) and normal prothrombin time (PT) Tx: factor IX concentrate What medication is recommended before dental procedures in high-risk individuals in order to prevent the breakdown of blood clots? Answer: Aminocaproic acid.

A five-year-old girl is brought to the clinic for evaluation of petechiae. The parents deny fever, decreased appetite, bone or joint pain, or weight loss. She did have a cold and runny nose about a month ago. On physical exam, the girl appears well with normal vital signs, petechiae and purpura on the upper and lower extremities. There are no signs of mucosal bleeding with normal lymph nodes, liver, or spleen. Laboratory studies reveal platelet count of 60,000/microL and an otherwise normal complete blood count. Which of the following is the most likely diagnosis?

dx: immune thrombocytopenia (ITP). ITP is one of the most common causes of symptomatic thrombocytopenia in children. At what age does immune thrombocytopenia present? Answer: Any age. child 2-6 years old H/o recent viral infection Red spots on skin or easy bleeding PE: petechiae, purpura, and gingival bleeding platelets < 100,000/µL MCC: antiplatelet antibodies Tx: observation, glucocorticoids and IVIG if severe

A four-year-old boy is in the clinic for evaluation of jaundice. He had a runny nose and a fever three days ago, and last night, the parents noticed he had decreased appetite and yellowing of the eyes. He has a history of requiring phototherapy during his neonatal period. The boy and his mother both have O positive blood type. On physical exam, the boy appears ill with icteric sclerae and splenomegaly. Laboratory testing shows anemia, reticulocytosis, elevatedmean corpuscular hemoglobin concentration (MCHC), elevated indirect bilirubin, negative Coombs test as well as numerous spherocytes found on peripheral blood smear. Which of the following is the most likely diagnosis?

dx:hereditary spherocytosis (HS). It is the most common hemolytic anemia due to a red cell membrane defect. Hereditary Spherocytosis Patient presents with symptoms of anemia: fatigue or pallor Positive family history Labs will show microcytic or normocytic anemia and is the only disorder that will cause an increase in mean corpuscular hemoglobin concentration (MCHC) Peripheral smear will show spherocytes and Howell-Jolly body Most commonly caused by an autosomal dominant genetic disease Treatment includes daily folic acid What is the most common complication of hereditary spherocytosis? Answer: Bilirubin gallstones.

1)What are four other inherited hypercoagulable disorders? Answer: Prothrombin mutation, hyperhomocysteinemia, protein C and S deficiencies. 2)Which leukemia is commonly associated with smudge cells (abnormally fragile lymphocytes when smeared onto a glass slide, giving rise to many broken or smudge appearing cells)? Answer: Chronic lymphocytic leukemia. 3) What jagged, irregularly shaped, red blood cells indicate disseminated intravascular coagulation, a complication of acute myelogenous leukemia? Answer: Schistocytes. 4)Ascorbic acid, which is found in orange juice, doubles the absorption of non-heme iron. 5)Which form of supplemental iron is most easily absorbed? Answer: Ferrous sulfate. 6)What treatment is recommended simultaneously during the first several months of vitamin B12 replacement? Answer: Folic acid replacement. 7) 8) 9) 10)

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1)What red blood cell disorder has a characteristic increase in mean corpuscular hemoglobin concentration? Answer: Spherocytosis. 2)What medication is recommended before dental procedures in high-risk individuals in order to prevent the breakdown of blood clots? Answer: Aminocaproic acid. 3)What are common causes of false-negative heterophile antibody tests in patients with infectious mononucleosis? Answer: Young patient age (infants and children under 4 years of age) and testing in the first week of illness. 4)What role does von Willebrand factor play in primary hemostasis? Answer: It binds to both platelets and endothelial components, forming an adhesive bridge between platelets and vascular subendothelial structures at sites of endothelial injury. 5)Which gastric hormone is hypersecreted, resulting in peptic ulcer disease and pruritus, a common complication of polycythemia vera? Answer: Histamine. 6) What are other conditions in which Howell-Jolly bodies are seen on peripheral smear? Answer: Severe hemolytic anemia, amyloidosis, megaloblastic anemia, hereditary spherocytosis, and myelodysplastic syndrome. 7)Children with hereditary spherocytosis are at risk for aplastic crisis as result of an infection from which virus? Answer: Parvovirus B19. 8)How soon after blood transfusion initiation will anaphylaxis occur? Answer: Within minutes. It is triggered by presence of IgA antibodies in IgA-deficient patients. 9)What are other causes of macrocytic anemia not related to megaloblastic changes? Answer: Liver disease (most common) and hypothyroidism. 10)What treatment is required to prevent organ damage from iron overload in patients with regular blood transfusions? Answer: Oral or parenteral iron chelation therapy. 11) Is the patient's platelet count depressed, normal, or increased in von Willebrand disease? Answer: Normal. 12)Which agent is preferred over protamine for reversal of anticoagulation when intracranial hemorrhage is associated with low-molecular-weight heparin? Answer: Andexanet alfa. 13)What is the 5 year survival rate of acute myeloid leukemia (AML)? Answer: Approximately 25%. 14) What is the mechanism of thrombocytopenia in idiopathic thrombocytopenic purpura? Answer: Autoantibodies directed against platelets lead to platelet destruction. 15) Deficiency of which factors causes a prolonged activated partial thromboplastin time (aPTT)? Answer: Factors 8, 9, 11, and 12. 16)Paired, narrow, white, transverse bands that disappear when pressure is applied to the nail bed are called what? Answer: Muehrcke's lines. 17) In addition to an absent Philadelphia chromosome, how can CML be differentiated from the reactive leukocytosis seen with an infectious process? Answer: Reactive leukocytosis will have notably lower white blood cell counts (less than 50,000) and absent splenomegaly. 18) What tapeworm infection is associated with vitamin B12 deficiency? Answer: Diphyllobothrium latum infection causes vitamin B12 malabsorption. 19) What are the clinical features of Wernicke-Korsakoff syndrome? Answer: Confusion, decreased vision, hypothermia, hypotension, ataxia, amnesia, tremor, and coma. 20) How does aspirin inactivate platelets? Answer: Aspirin inhibits the synthesis of thromboxane A2 (TXA2), a potent stimulator of platelet aggregation.

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You are seeing a patient who presented with microcytosis, microcytic anemia, and abnormal red blood cell morphology with acanthocytes and target cells. You suspect alpha-thalassemia. Which of the following family histories is most commonly associated with this diagnosis? AAshkenazi Jewish descent BBlack descent CItalian descent DSoutheast Asian descent

family history of Southeast Asian or Chinese descent Alpha-Thalassemia 1 gene inactive: silent carrier, no symptoms 2 genes inactive: alpha-thalassemia trait, mild anemia, very low MCV, no symptoms 3 genes inactive: hemoglobin H disease, marked microcytic, hypochromic anemia, splenomegaly, Hgb H 4-10% 4 genes inactive: hydrops fetalis, no fetal or adult Hgb, death in utero or neonatally What test can definitively diagnose alpha-thalassemia trait? Answer: Genetic testing that demonstrates the alpha globin gene deletion.