Hematology - PACKRAT Questions

Scientific Concepts/Hematology A defect in which of the following physiologic processes can lead to the development of a microcytic, hypochromic anemia? A. Hemoglobin production B. IgG antibody formation C. Red blood cell nuclear maturation D. Red blood cell membrane defects

Explanations (c) A. A DEFICIENCY IN SUBSTANCES REQUIRED FOR HEMOGLOBIN SYNTHESIS RESULT IN RBCS WITH A DEFICIENT MEAN CORPUSCULAR HEMOGLOBIN CONCENTRATION AND USUALLY MICROCYTOSIS. (u) b. IgG antibody formation would led to the development of a hemolytic anemia. (u) C. Alterations in red blood cell nuclear maturation led to the development of macrocytic red blood cells. (u) D. Red blood cell membrane defects led to the development of hemolytic anemia.

History & Physical/Hematology What examination finding would be expected in a patient with von Willebrand disease? A. Gingival bleeding B. Splenomegaly C. Muscle weakness D. Hemarthrosis

Explanations (c) A. A PATIENT WITH VON WILLEBRAND DISEASE MOST COMMONLY PRESENTS WITH MUCOSAL BLEEDING SEEN IN EPISTAXIS, GINGIVAL BLEEDING, AND MENORRHAGIA. (u) B. Splenomegaly is seen with hemolytic anemia, not with von Willebrand disease. (u) C. Muscle weakness is not seen with von Willebrand disease. (u) D. Hemarthrosis is found in hemophilia, not in von Willebrand disease.

Clinical Therapeutics/Hematology A 21 year-old male with a diagnosis of type 1 von Willebrand disease undergoes dental extraction of his wisdom teeth. The patient comes to the clinic with continued oozing of the dental sockets despite packing. Treatment should begin with which of the following? A. DDAVP B. Factor VIII C. vWF concentrate D. FFP

Explanations (c) A. DDAVP CAUSES THE RELEASE OF VWF AND FACTOR VIII FROM STORAGE SITES SIGNIFICANTLY WHICH IS NEEDED TO COMPLETE HEMOSTASIS. FACTOR VIII IS INDICATED FOR PATIENTS WITH HEMOPHILIA A. FRESH FROZEN PLASMA IS INDICATED IN COUMADIN OVERDOSAGE AND VWF CONCENTRATE IS INDICATED IN TYPE 2 AND 3 PATIENTS WITH VON WILLEBRAND DISEASE. (u) B. See A for explanation. (u) C. See A for explanation. (u) D. See A for explanation.

Scientific Concepts/Hematology Normal hemoglobin A is made of what combination of heme and globin chains? A. 2-alphas and 2-betas B. 4-gammas C. 2-alphas and 2-gammas D. 4-betas

Explanations (c) A. HEMOGLOBIN A1 IS COMPOSED OF TWO ALPHA AND TWO BETA CHAINS. (u) B. Barts Hemoglobin-Hydrops Fetalis has 4-gamma chains and is incompatible with life. (u) C. Fetal hemoglobin consists of 2-alpha and 2-gamma chains. (u) D. Hemoglobin H consists of 4-beta chains and is nonfunctional.

Clinical Therapeutics/Hematology Low molecular weight heparin (LMWH) dosage is based on which of the following? A. Weight B. International normalized ratio (INR) C. Prothrombin time (PT) D. Partial thromboplastin time (PTT)

Explanations (c) A. LMWH IS BASED ON A PATIENT'S WEIGHT IN KILOGRAMS. (u) B. INR is used to monitor anticoagulant medications. (u) C. PT is used to monitor warfarin efficacy. (u) D. PTT is used to monitor heparin use but not to determine the dosage.

History & Physical/Hematology A female patient presents with weakness and fatigue for the past three months. A CBC was ordered and revealed the following values: RBC 3.8 X 10(6)mcL (4.7-6.1 x 10(6)mcL) HCT 27% (35-45%) Hgb 9.2 gms/dL (12-15.5 gms/dL) MCV 120 fL (80-100 fL) MCH 35 pg (26-34 pg) On physical examination which of the following would be a consistent finding for this type of anemia? A. Paresthesia of the hands and feet B. Hepatosplenomegaly C. Tachycardia D. Jaundice

Explanations (c) A. NEUROLOGICAL MANIFESTATION IS THE EARLIEST TYPE MOST COMMONLY SEEN WITH MEGALOBLASTIC ANEMIA MOST COMMONLY FROM VITAMIN B12 DEFICIENCY. (u) B. Hepatosplenomegaly and jaundice are commonly seen in hemolytic anemias. (u) C. This compensatory mechanism is common in any type of anemia due to the hypoxemic effects of the anemia. (u) D. See B for explanation.

Health Maintenance/Hematology A 13 year-old male with sickle cell trait is interested in playing football for his school. Which of the following considerations should be reviewed with the patient and his parents? A. avoid dehydration B. avoid all contact sports C. begin daily use of aspirin D. begin hydroxyurea supplements

Explanations (c) A. NO RESTRICTION OF SPORTS ACTIVITY IS REQUIRED OF PATIENTS WITH SICKLE CELL TRAIT. HYDRATION IS REQUIRED IN ALL SPORTS ACTIVITIES. (u) B. See A for explanation. (u) C. There is no increased risk of thrombosis so daily use of aspirin is not needed. (u) D. Hydroxyurea is used in the treatment of sickle cell disease; sickle cell trait requires no treatment.

Scientific Concepts/Hematology Absorption of vitamin B12 requires intrinsic factor that is secreted by which of the following? A. parietal cells B. chief cells C. mucous cells D. enteroendocrine cells

Explanations (c) A. PARIETAL CELLS ARE RESPONSIBLE FOR ACID SECRETION AND INTRINSIC FACTOR. (u) B. Chief cells are responsible for the secretion of the proteolytic proenzymes pepsinogen I and II. (u) C. Mucous cells secrete mucous and pepsinogen II. (u) D. Enteroendocrine cell secrete gastrin.

Clinical Intervention/Hematology It is essential to advise a patient with infectious mononucleosis to Answers A. avoid contact sports. B. continue to rest as much as possible. C. gargle with salt water for relief of throat pain. D. rest voice to prevent hoarseness.

Explanations (c) A. PATIENTS WITH ACUTE INFECTIOUS MONONUCLEOSIS OFTEN HAVE ENLARGEMENT OF THE SPLEEN. CONTACT SPORTS INCREASE THE RISK FOR SPLENIC RUPTURE AFTER CONTACT. THE OTHER ANSWERS ARE SUGGESTIONS THAT MAY IMPROVE SYMPTOMS BUT DO NOT TRULY IMPACT THE CARE OF THE PATIENT. (u) B. See A for explanation. (u) C. See A for explanation. (u) D. See A for explanation.

Health Maintenance/Hematology A patient with multiple myeloma should be immunized against which of the following organisms? Answers A. Streptococcus pneumoniae B. Hepatitis C virus C. Listeria monocytogenes D. Epstein Barr virus

Explanations (c) A. PATIENTS WITH MULTIPLE MYELOMA ARE PRONE TO INFECTIONS WITH ENCAPSULATED ORGANISMS SUCH AS STREPTOCOCCUS PNEUMONIAE AND HAEMOPHILUS INFLUENZAE. (u) B. See A for explanation. (u) C. See A for explanation. (u) D. See A for explanation.

History & Physical/Hematology A 55 year-old non-smoking male presents with a hemoglobin of 18.5 g/dl and a hematocrit of 56%. Which of the following physical examination findings is the most likely to be noted with this patient? A. splenomegaly B. cheilosis C. purpura D. decreased vibratory sense

Explanations (c) A. PATIENTS WITH POLYCYTHEMIA VERA PRESENT WITH ELEVATED HEMOGLOBIN AND HEMATOCRIT. ON PHYSICAL EXAMINATION PLETHORA, ENGORGED RETINAL VEINS, AND SPLENOMEGALY ARE COMMON. (u) B. Cheilosis is noted in iron deficiency anemia. (u) C. Purpura is typically noted in bleeding disorders. (u) D. Decreased vibratory sense is noted in vitamin B12 deficiency.

History & Physical/Hematology A 55-year-old non-smoking male presents with a hemoglobin of 18.5 g/dl and a hematocrit of 56%. Which of the following physical examination findings https://quizlet.com/165341731/edit#is the most likely to be noted with this patient? Answers A. Splenomegaly B. Cheilosis C. Purpura D. Decreased vibratory sense

Explanations (c) A. PATIENTS WITH POLYCYTHEMIA VERA PRESENT WITH ELEVATED HEMOGLOBIN AND HEMATOCRIT. ON PHYSICAL EXAMINATION PLETHORA, ENGORGED RETINAL VEINS, AND SPLENOMEGALY ARE COMMON. (u) B. Cheilosis is noted in iron deficiency anemia. (u) C. Purpura is typically noted in bleeding disorders. (u) D. Decreased vibratory sense is noted in vitamin B12 deficiency.

Health Maintenance/Hematology Which of the following therapies is recommended for a 13 month-old child with sickle cell disease? A. Folic acid and penicillin V B. Ferrous sulfate and penicillin V C. Folic acid and ferrous sulfate D. Folic acid, ferrous sulfate and penicillin V

Explanations (c) A. PATIENTS WITH SICKLE CELL DISEASE SHOULD RECEIVE PROPHYLACTIC PENICILLIN V STARTING AT 2 MONTHS OF AGE AND FOLIC ACID STARTING AT 1 YEAR OF AGE. FERROUS SULFATE IS NOT GLOBALLY RECOMMENDED FOR PATIENTS WITH SICKLE CELL DISEASE. (u) B. See A for explanation. (u) C. See A for explanation. (u) D. See A for explanation.

Clinical Therapeutics/Hematology A 56 year-old male is noted to have a recent diagnosis of polycythemia vera. His current hemoglobin is 21 gms/dl. What treatment should be instituted for this patient at this time? A. Phlebotomy B. Iron chelation therapy C. Bone marrow radiation therapy D. Normal saline IV hydration

Explanations (c) A. THIS PATIENT HAS A DIAGNOSIS OF POLYCYTHEMIA VERA TREATMENT BEGINS WITH PHLEBOTOMY INSTITUTED ON A WEEKLY BASIS UNTIL THE HEMATOCRIT IS LESS THAN 45%. MAINTAINANCE OF THE HEMATOCRIT AT 45% IS ACHIEVED WITH REPEATED PHLEBOTOMY AS NECESSARY. (u) B. See A for explanation. (h) C. Radiation therapy has no place in this treatment. (h) D. IV hydration with normal saline will place the patient into volume overload.

Clinical Intervention/Hematology A 29 year-old patient with idiopathic thrombocytopenia purpura (ITP) is treated with prednisone therapy. Despite therapy, platelet counts remain consistently below 20,000/microliter over the course of 6 weeks. Which of the following is the most appropriate intervention for this patient? A. Aspirin B. Intravenous immunoglobulin C. Danazol (Danocrine) D. Splenectomy

Explanations (h) A. Aspirin inhibits platelet function and could lead to significant bleeding and death of this patient. (u) B. Intravenous immunoglobulin can be utilized for short-term treatment, but the platelet count is likely to return to baseline within a month. (u) C. Danazol is typically reserved for ITP that fails to respond to splenectomy. (c) D. PERSISTENTLY LOW PLATELET COUNTS (< 20,000) REQUIRE EFFECTIVE LONG-TERM TREATMENT, AND SPLENECTOMY IS THE TREATMENT OF CHOICE.

Clinical Intervention/Hematology A patient with a history of multiple trauma has received a combination of 12 units of packed red blood cells and whole blood while in the emergency department. Later that night it is noted that the patient is beginning to ooze blood from sites of injury and needle punctures. Which of the following is the most appropriate intervention? A. Continue transfusions of packed red blood cells B. Fresh frozen plasma C. Albumin D. Factor VIII concentrate

Explanations (h) A. Continued administration of PRBC without replacement of FFP will result in possible volume overload and continued bleeding. (c) B. WHEN A PATIENT RECEIVES MORE THAN ONE TOTAL BLOOD VOLUME (10 UNITS) WITHOUT REPLACEMENT OF CLOTTING FACTORS, THE PATIENT WILL CONTINUE TO BLEED WITHOUT ADMINISTRATION OF FRESH FROZEN PLASMA. (u) C. Albumin is used for protein and intravascular volume replacement. (h) D. Factor VIII concentrate is used in the treatment of hemophilia A and would not effect the bleeding in this patient.

Diagnosis/Hematology A 26 year-old female required 12 units packed red blood cells during a trauma resuscitation and surgical repair of liver and splenic lacerations. The patient is now 6 hours postoperative and has blood oozing from the suture line and IV sites. There is bloody urine in the Foley bag. Laboratory evaluation demonstrates a platelet count of 10,000/microliter, prolonged prothrombin level, and the presence of fibrin split products. Which of the following is the most likely diagnosis? A. acute ABO incompatibility reaction B. disseminated intravascular coagulation C. exacerbation of idiopathic thrombocytopenia D. inadequate repair of the liver lacerations

Explanations (u) A. ABO incompatibility results in immediate hemolysis and shock. (c) B. DISSEMINATED INTRAVASCULAR COAGULATION IS CHARACTERIZED BY BLEEDING FROM MANY SITES AS ALL COAGULATION FACTORS ARE CONSUMED AND THEN BROKEN DOWN, LEADING TO DECREASED FIBRINOGEN LEVEL AND PLATELET COUNT, PROLONGED PT AND PTT, AND PRESENCE OF FIBRIN SPLIT PRODUCTS. (u) C. Idiopathic thrombocytopenia is characterized by decreased platelet count, but coagulation factors are normal. (u) D. Bleeding would be localized only and would result in shock if lacerations of the liver were not repaired properly.

Diagnosis/Hematology A 69 year-old female presents to the clinic complaining of fatigue. Physical examination reveals lymphadenopathy, splenomegaly and pale conjunctiva. The remainder of the examination is unremarkable. CBC reveals a normochromic normocytic anemia. White blood cell count is 45,000/mm3, with a differential of 77% mature lymphocytes, 3% eosinophils, 18% segmented neutrophils and 1% basophils and monocytes. Platelets appear adequate in number. What is the most likely diagnosis? A. Acute myelocytic leukemia B. Acute lymphocytic leukemia C. Chronic lymphocytic leukemia D. Chronic myelocytic leukemia

Explanations (u) A. Acute leukemias present with circulating blast cells with pancytopenia. (u) B. See A for explanation. (c) C. CHRONIC LYMPHOCYTIC LEUKEMIA TYPICALLY OCCURS AFTER AGE 50 PRESENTING WITH LYMPHOCYTOSIS WITH LYMPHOCYTES THAT APPEAR SMALL AND MATURE. (u) D. See C for explanation.

Diagnosis/Hematology A 48 year-old male complains of weakness and general malaise for about 2 months. Patient denies any recent illness and does not take any medications. Physical examination reveals a pale looking male in no acute distress. His heart rate is 110 beats/minute without a murmur and his abdominal examination reveals hepatosplenomegaly. A CBC reveals the WBC to be 62,000/microliter, Hgb is 8.3 gms/dl, Hct is 24.6%. A differential reveals a predominance of monoblasts and promyelocytes with Auer rods present. What is the most likely diagnosis? A. Acute lymphocytic leukemia B. Acute myelogenous leukemia C. Chronic lymphocytic leukemia D. Chronic myelogenous leukemia

Explanations (u) A. Acute lymphocytic leukemia (ALL) is a leukemia most often seen in children with lymphoblasts predominating. Adults who develop ALL usually have a prolymphocytic cell presentation. (c) B. ACUTE MYELOGENOUS LEUKEMIA (AML) IS A LEUKEMIA THAT PRESENTS IN ADULTS WITH A CELL LINEAGE OF THE IMMATURE GRANULOCYTIC CELLS SEEN IN THE PERIPHERAL BLOOD E.G. MYELOBLASTS, PROMYELOCYTES. AUER RODS ARE COMMONLY SEEN IN THIS CONDITION. (u) C. Chronic lymphocytic leukemia (CLL) is a leukemia that presents in older adults with WBC counts up to 100,000/mm3. On peripheral blood smears the cell that predominates is a mature lymphocyte. (u) D. Chronic myelogenous leukemia (CML) is a leukemia that presents in adults with a cell lineage of mature cell lines with a marked increase in basophils and eosinophils. These cells are also known for having the Philadelphia chromosome. Ref: (28)

Diagnosis/Hematology A 60-year-old presents with fatigue and splenomegaly. CBC reveals the following: WBC- 24,000/microliter, Hgb- 13.5 g/dl, Hct- 40%, MCV- 87 fL, MCHC- 34 g/dl, MCH- 28 pg, and platelets- 380,000/mL. The differential reveals neutrophils- 11%, lymphocytes- 80%, monocytes- 8%, and basophils- 1%. What is the most likely diagnosis? Answers A. Acute lymphocytic leukemia B. Acute myelogenous leukemia C. Chronic lymphocytic leukemia D. Chronic myelogenous leukemia

Explanations (u) A. Acute lymphocytic leukemia is more common in children and presents with blasts in the peripheral blood. (u) B. Acute myelogenous leukemia presents with pancytopenia and presence of blasts in the peripheral blood. (c) C. CHRONIC LYMPHOCYTIC LEUKEMIA PRESENTS WITH A WBC COUNT GREATER THAN 20,000/MICROLITER AND ABSOLUTE LYMPHOCYTE COUNT OF GREATER THAN 5000/MICROLITER. (u) D. Chronic myelogenous leukemia presents with elevated WBC count, marked left shift in the myeloid series of cells, and positive for Philadelphia chromosome.

Scientific Concepts/Hematology Pernicious anemia is due to deficiency in which of the following? Answers A. Cobalamin-binding proteins B. Pancreatic enzymes C. Spectrin D. Intrinsic factor

Explanations (u) A. Cobalamin-binding proteins and pancreatic enzyme deficiencies are causes of vitamin B12 deficiency, but not pernicious anemia. (u) B. See A for explanation. (u) C. Abnormalities in spectrin and actin are noted in hereditary spherocytosis. (c) D. PERNICIOUS ANEMIA IS CAUSED BY LACK OF INTRINSIC FACTOR.

Clinical Therapeutics/Hematology A 6 year-old male presents with hemarthrosis of the left knee. Coagulation studies reveal the following results: PT- 12.5 seconds with an INR: 1.0, aPTT:58 seconds, platelet count: 82 430,000/microliter, and bleeding time:4 minutes. Which of the following is the best treatment option for this patient? Answers A. Desmopressin acetate B. Corticosteroids C. Vitamin K D. Cryoprecipitate

Explanations (u) A. Desmopressin acetate is indicated in von Willebrand's disease, which presents with a prolonged bleeding time. (u) B. Corticosteroids are indicated in immune-mediated thrombocytopenia. (u) C. Vitamin K deficiency will prolong the PT greater than the aPTT. Vitamin K supplement is not indicated in this patient. (c) D. HEMOPHILIA A PRESENTS WITH A PROLONGED APTT AND NORMAL PLATELET COUNT AND FUNCTION. HEMOPHILIA A IS TREATED WITH FACTOR VIII CONCENTRATE OR CRYOPRECIPITATE.

Clinical Therapeutics/Hematology A 6 year-old male presents with hemarthrosis of the left knee. Coagulation studies reveal the following results: PT 12.5 seconds (normal range 12-14 seconds), INR 1.0, aPTT 58 seconds (normal range 18-28 seconds), platelet count 430,000/microliter (normal range 150,000-450,000/microliter), and bleeding time 4 minutes (normal range 2-12 minutes). Which of the following is the best treatment option for this patient? A. Desmopressin acetate B. Corticosteroids C. Vitamin K D. Cryoprecipitate

Explanations (u) A. Desmopressin acetate is indicated in von Willebrand's disease, which presents with a prolonged bleeding time. (u) B. Corticosteroids are indicated in immune-mediated thrombocytopenia. (u) C. Vitamin K deficiency will prolong the PT greater than the aPTT. Vitamin K supplement is not indicated in this patient. (c) D.HEMOPHILIA A PRESENTS WITH A PROLONGED APTT AND NORMAL PLATELET COUNT AND FUNCTION. HEMOPHILIA A IS TREATED WITH FACTOR VIII CONCENTRATE OR CRYOPRECIPITATE.

Diagnostic Studies/Hematology A positive osmotic fragility test is seen in which of the following? A. G6PD deficiency B. Sickle cell anemia C. Hereditiary spherocytosis D. Autoimmune hemolytic anemia

Explanations (u) A. G6PD deficiency may result in episodic hemolysis in response to oxidant drugs or infections and is not associated with a positive osmotic fragility test. (u) B. Sickle cell anemia is an abnormal hemoglobinopathy leading to chronic hemolytic anemia and is confirmed with hemoglobin electrophoresis. An osmotic fragility test would be negative. (c) C. HEREDITARY SPHEROCYTOSIS WILL HAVE A POSITIVE OSMOTIC FRAGILITY TEST SECONDARY TO A DEFECTIVE RED BLOOD CELL MEMBRANE. (u) D. Coombs' testing forms the basis for the diagnosis of autoimmune hemolytic disorders.

Scientific Concepts/Hematology Which of the following conditions is caused by a disorder in the red blood cell membrane? A. G6PD deficiency B. Folate deficiency C. Sickle cell anemia D. Hereditary spherocytosis

Explanations (u) A. G6PD deficiency results in an increased destruction of red cells that leads to anemia. (u) B. Folate is essential in DNA synthesis, lack of folate leads to ineffective erythropoiesis. (u) C. Sickle cell anemia is a hemoglobinopathy which leads to increased red cell destruction. (c) D. THE CELL MEMBRANE DEFECT OF SPHEROCYTOSIS LEADS TO HEMOLYSIS DUE TO TRAPPING OF THE CELLS BY THE SPLEEN.

Diagnostic Studies/Hematology Which of the following leukemias is most closely associated with the Philadelphia chromosome? A. hairy cell B. acute lymphocytic C. acute myelocytic D. chronic myelocytic

Explanations (u) A. Hairy cell leukemia is a rare lymphocytic leukemia. See D for explanation. (u) B. See D for explanation. (u) C. See D for explanation. (c) D. PHILADELPHIA CHROMOSOME IS NOTED MOST COMMONLY IN PATIENTS WITH CHRONIC MYELOGENOUS LEUKEMIA.

Diagnostic Studies/Hematology An 18 year-old woman presents to the clinic complaining of fatigue. She reports a past history of lifelong frequent nosebleeds and bleeding gums. She also has menorrhagia. Her mother and maternal grandfather have a similar bleeding history. Initial lab results are as follows: WBC 9,500/mm3, Hgb 10.9 g/dL, HCT 33%, MCV 69 fL, MCHC 26 pg and platelets 284,000/mm3. Which of the following tests should be ordered to evaluate this patient's diagnosis? A. Hemoglobin electrophoresis B. Bleeding time and platelet aggregometry C. Bone marrow aspiration D. PT and aPTT

Explanations (u) A. Hemoglobin electrophoresis would be utilized to evaluate microcytic, hypochromic anemias. (c) B. THE PATIENT'S PRESENTATION IS CONSISTENT WITH A CONGENTIAL QUALITATIVE PLATELET DISORDER, MOST LIKELY VON WILLEBRAND'S DISEASE, NECESSITATING A BLEEDING TIME AND EVALUATION OF PLATELET FUNCTION. (u) C. Bone marrow aspiration is not utilized in the evaluation of qualitative platelet disorders. (u) D. A PT and aPTT would be utilized to evaluate for bleeding consistent with abnormalities with the coagulation cascade. Ref: (11)

Diagnosis/Hematology A 60 year-old male presents with a normochromic, normocytic anemia and splenomegaly. His past history reveals several episodes of bacterial pneumonia in the past year. The WBC count is 43,000 mm3 with 25% segmented neutrophils, 3% blasts, 70% mature lymphocytes, 1% basophils, and 1% eosinophils. This most likely represents A. myelodysplastic syndrome. B. acute lymphocytic leukemia. C. chronic lymphocytic leukemia. D. chronic myelogenous leukemia.

Explanations (u) A. Myelodysplastic syndrome may present with this clinical picture, but a CBC would indicate a normal or reduced white cell count. (u) B. A hallmark of acute leukemia is pancytopenia with numerous circulating blasts. (c) C. CHRONIC LYMPHOCYTIC LEUKEMIA USUALLY OCCURS AFTER THE AGE OF 50 PRESENTING WITH LYMPHOCYTOSIS > 20,000 MM3 AND LYMPHOCYTES THAT APPEAR MATURE. (u) D. Chronic myelogenous leukemia presents with markedly elevated WBC count (mean 150,000/mm3) with left shifted myeloid series.

Health Maintenance/Hematology An adult patient who has undergone a splenectomy secondary to idiopathic thrombocytopenic purpura should receive which prophylactic agent? A. Bicillin 1.2 million units IM every month B. Pneumococcal vaccine IM one time only C. Gamma globulin IM every month D. Hepatitis B vaccine IM series

Explanations (u) A. Patients who are being treated for rheumatic fever may receive Bicillin 1.2 million units every month for Group A beta-streptococcal prophylaxis. (c) B. PNEUMOCOCCAL VACCINE SHOULD ROUTINELY BE GIVEN TO ALL PATIENTS WHO UNDERGO SPLENECTOMY FOR PROPHYLAXIS TO STREPTOCOCCAL PNEUMONIAE. (u) C. Gamma globulin is routinely given to patients for prophylaxis to hepatitis exposure. (u) D. Hepatitis B vaccine has no specific indication in patients after splenectomy.

Diagnostic Studies/Hematology An African American male is placed on hydroxychlorquine (Plaquenil) for travel to Africa. Within six weeks he presents complaining of fatigue. CBC shows anemia. Which of the following is the diagnostic study of choice in this patient? A. Peripheral smear B. G6PD assay C. Total iron binding capacity D. Hemoglobin electrophoresis

Explanations (u) A. Peripheral smear may reveal "bite" cells suggestive of G6PD, but is not diagnostic. (c) B. G6PD ASSAY IS THE DIAGNOSTIC STUDY OF CHOICE FOR G6PD DEFICIENCY. (u) C. TIBC would be indicated in the evaluation of iron deficiency anemia, and is not indicated in the evaluation of G6PD deficiency. (u) D. Hemoglobin electrophoresis is indicated in the evaluation of hemolytic anemia and is not indicated in the evaluation of G6PD deficiency.

History & Physical/Hematology A 52 year-old female is diagnosed with chronic myeloid leukemia (CML). Which of the following would you expect to find on physical examination of this patient? A. Hepatomegaly B. Splenomegaly C. Posterior cervical lymphadenopathy D. Inguinal lymphadenopathy

Explanations (u) A. See B for explanation. (c) B. CML PATIENTS PRESENT WITH AN ENLARGED SPLEEN. (u) C. CML patients typically present with enlarged spleen and fatigue, but there are not enlarged lymph nodes. (u) D. Patients with CML do not present with inguinal lymphadenopathy.

Scientific Concepts/Hematology A 27 year-old male nonsmoker is diagnosed with upper extremity venous thrombosis. What is the most likely factor abnormality found in this patient? A. Factor VIII B. Factor V C. Factor I D. Factor X

Explanations (u) A. See B for explanation. (c) B. IN YOUNG ADULTS WITH OTHERWISE UNEXPLAINED THROMBOSIS, FACTOR V LEIDEN IS THE MOST COMMON ASSOCIATED ABNORMALITY. (u) C.See B for explanation. (u) D.See B for explanation.

Clinical Intervention/Hematology A 26 year-old male presents with a four week history of fatigue, night sweats, and a painless mass in his neck. Physical examination confirms the presence of an enlarged right posterior cervical lymph node. What is the next best step in the evaluation of this patient? A. bone marrow biopsy and aspiration B. lymph node biopsy C. chest CT scan D. reexamine in 2-4 weeks

Explanations (u) A. See B for explanation. (c) B. THIS PATIENT PRESENTS WITH POSSIBLE LYMPHOMA. DIAGNOSIS IS MADE BY LYMPH NODE BIOPSY. BONE MARROW BIOPSY AND CT SCAN OF THE CHEST ARE USED FOR STAGING OF THE DISEASE. (u) C. See B for explanation. (u) D. With the presence of B-symptoms (fever, night sweats, and weight loss) the patient needs a biopsy to evaluate for possible lymphoma.

Health Maintenance/Hematology Which of the following Rh genotypes in a mother and father would represent a risk for hemolytic disease of the newborn? A. Mother Rh-positive, father Rh-negative B. Mother Rh-positive, father Rh-positive C. Mother Rh-negative, father Rh-positive D. Mother Rh-negative, father Rh-negative

Explanations (u) A. See C for explanation. (u) B. See C for explanation. (c) C. IF AN RH-NEGATIVE WOMAN CARRIES AN RH-POSITIVE FETUS, SHE MAY DEVELOP ANTIBODIES AGAINST RH WHEN FETAL BLOOD CELLS ENTER HER CIRCULATION. (u) D. See C for explanation.

Clinical Intervention/Hematology A patient receiving heparin therapy for 6 days for deep vein thrombosis develops thrombosis at the IV site. The INR is 1.1. The aPTT is 66 seconds and the platelet count is 47,000 down from 148,000 on admission. Which of the following is the most appropriate treatment? A. Protamine sulfate administration B. Platelet administration C. Discontinue the heparin D. Schedule the patient for plasmapheresis

Explanations (u) A. See C for explanation. (u) B. See C for explanation. (c) C. ONCE THE DIAGNOSIS OF HEPARIN INDUCED THROMBOCYTOPENIA IS SUSPECTED TREATMENT SHOULD INCLUDE IMMEDIATE DISCONTINUATION OF ALL FORMS OF HEPARIN AND TREATMENT WITH A DIRECT THROMBIN INHIBITOR SHOULD BEGIN. PLATELET ADMINISTRATION IS GENERALLY NOT INDICATED. PROTAMINE SULFATE IS USED FOR HEPARIN OVERDOSAGE AND PLASMAPHERESIS WOULD NOT BE INDICATED AS THE PLATELET COUNT WILL RESUME WHEN THE HEPARIN IS DISCONTINUED. (u) D. See C for explanation.

Diagnostic Studies/Hematology A positive direct Coombs' test may be seen in which of the following conditions? A. G6PD deficiency B. Sickle cell anemia C. Hereditary spherocytosis D. Autoimmune hemolytic anemia

Explanations (u) A. See D for explanation. (u) B. See D for explanation. (u) C. See D for explanation. (c) D. A POSITIVE DIRECT COOMBS' TEST INDICATES THAT ANTIBODY HAS ATTACHED TO AN ANTIGEN ON THE RBC WHICH CAUSES AGGLUTINATION. AUTOIMMUNE HEMOLYTIC ANEMIA RESULTS WHEN THE PATIENT HAS ANTIBODIES AGAINST THEIR OWN RBCS. G6PD DEFICIENCY DOES RESULT FROM AN ANTIBODY ANTIGEN REACTION. SICKLE CELL ANEMIA IS CAUSED BY A DEFECTIVE HEMOGLOBIN WHICH DETECTED BY HEMOGLOBIN ELECTROPHORESIS. HEREDITARY SPHEROCYTOSIS IS DETECTED UTILIZING THE OSMOTIC FRAGILITY TEST.

. Health Maintenance/Hematology Adult patients with a compromised immune system should not receive which of the following immunizations? A. Hepatitis A B. Pneumococcal C. Influenza D. Varicella

Explanations (u) A. See D for explanation. (u) B. See D for explanation. (u) C. See D for explanation. (c) D. VARICELLA VACCINE IS A LIVE VACCINE AND IS CONTRAINDICATED IN THOSE INDIVIDUALS WITH A COMPROMISED IMMUNE SYSTEM. HEPATITIS A, PNEUMOCOCCAL AND INFLUENZA ARE RECOMMENDED FOR PATIENTS WITH A COMPROMISED IMMUNE SYSTEM.

Diagnostic Studies/Hematology An 8-year-old presents with splenomegaly. CBC results reveal the following: WBC- 6,300/microliter, Hgb- 10.5 g/dl, Hct- 31%, MCV- 87 fL, MCHC- 39 g/dl, MCH- 28 pg, and platelets- 317,000/mL. Examination of the RBC morphology reveals 80% spherocytes. Which of the following would be most helpful in confirming the diagnosis? 27 Answers A. Direct Coombs test B. Osmotic fragility C. G-6-PD level D. Serum ferritin

Explanations (u) A. The direct Coombs test would be negative and would not be helpful in diagnosing hereditary spherocytosis. (c) B. HEREDITARY SPHEROCYTOSIS PRESENTS WITH A NORMOCYTIC, NORMOCHROMIC ANEMIA AND MANY SPHEROCYTES. DIAGNOSIS IS CONFIRMED WITH A POSITIVE OSMOTIC FRAGILITY TEST. (u) C. G-6-PD deficiency presents with minimal or no RBC morphologic abnormalities and is diagnosed by measuring G-6-PD enzyme activity level. (u) D. Iron deficiency anemia typically presents with microcytic, hypochromic red blood cells and is diagnosed with a serum ferritin.

Clinical Intervention/Hematology A 68 year-old male with a history of atrial fibrillation treated with warfarin (Coumadin) presents to the emergency department after vomiting large amounts of bright red blood. INR is 3. Which of the following is most appropriate to rapidly lower the patient's INR? A. Discontinue warfarin B. Administer fresh frozen plasma C. Administer protamine sulfate D. Administer heparin sulphate

Explanations (u) A. This will not immediately reverse the effects of warfarin. (c) B. FRESH FROZEN PLASMA IS THE MOST RAPID WAY TO LOWER THE PATIENT'S INR. (u) C. Protamine sulfate is used to neutralize heparin sulphate not warfarin. (h) D. Heparin administration would increase the bleeding and be harmful.

Clinical Therapeutics/Hematology A 35 year-old female presents with fatigue. CBC results reveal the following: WBC: 6,300/microliter Hgb: 9.5 g/dl Hct: 28% MCV: 75 fL MCHC: 32 g/dl MCH: 24 pg Platelets: 550,000/mL 46 Which of the following is the best treatment option for this patient? A. folic acid B. vitamin B12 C. prednisone D. ferrous sulfate

Explanations (u) A. Vitamin B12 and folate deficiency present with macrocytic cells and are treated with vitamin B12 and folate respectively. (u) B. See A for explanation. (u) C. Prednisone is used to treat immune-mediated hemolytic anemias which present with normocytic, normochromic red blood cells. (c) D. IRON DEFICIENCY ANEMIA IS A MICROCYTIC, HYPOCHROMIC ANEMIA AND IS TREATED WITH FERROUS SULFATE.

History & Physical/Hematology Which of the following physical findings suggest pernicious anemia? A. Splenomegaly and hepatomegaly B. Petechiae and ecchymosis C. Loss of position and vibratory sensation D. Cheilosis and koilonychia

Explanations (u)A. Splenomegaly and hepatomegaly are typically seen in hemolytic anemias. (u) B. Petechiae and ecchymosis are seen in thrombocytopenia. (c) C. LOSS OF POSITION AND VIBRATORY SENSATION ARE COMMON NEUROLOGIC FINDINGS IN PERNICIOUS ANEMIA. (u) D. Cheilosis and koilonychia are seen in iron deficiency anemia.

Diagnosis/Hematology A mean corpuscular volume (MCV) of less than 80 cubic microns is a manifestation of which of the following diagnoses? A. Iron deficiency B. Vitamin B12 deficiency C. Folate deficiency D. G6PD deficiency

Explanations (c) A. IRON DEFICIENCY IS ASSOCIATED WITH MICROCYTIC ANEMIA. (u) B. Vitamin B12 deficiency is associated with macrocytic anemia. (u) C. Folate deficiency is associated with macrocytic anemia. (u) D. G6PD deficiency is not associated with a low MCV.