PART XXI Diseases of the Blood

QUESTIONS
USAMA ALJUMAILY
1. The hematologic features of congenital hypoplastic anemia (Diamond-Blackfan
Anemia) include all the following EXCEPT
A. normocytic anemia
B. reticulocytopenia
C. deficiency or absence of red blood cell bone marrow precursors
D. elevated fetal hemoglobin (Hb F)
E. elevated serum iron levels

2. The MOST specific test that is helpful to differentiate Diamond-Blackfan anemia from
transient erythroblastopenia of childhood is
A. hemoglobin electrophoresis
B. reticulocytes count
C. erythrocyte adenosine deaminase (ADA) enzyme level assay
D. bone marrow examination
E. mean corpuscular volume (MCV)

3. Anemia and reticulocytopenia that occur in the 2nd half of infancy period is LEAST
likely due to
A. congenital hypoplastic anemia (Diamond-Blackfan anemia)
B. transient erythroblastopenia of childhood
C. a protracted, prolonged course of the anemia of hemolytic disease of the
newborn
D. aplastic crises complicating various types of chronic hemolytic anemias
E. Fanconi anemia

4. The main stay of treatment for congenital hypoplastic anemia is

A. corticosteroids
B. androgen
C. antithymocyte globulin (ATG)
D. fully matched-related stem cell transplantation
E. fully matched-unrelated stem cell transplantation

5. All the following are true regarding Transient Erythroblastopenia of Childhood (TEC)
EXCEPT
A. it is more common than congenital hypoplastic (Diamond-Blackfan) anemia
B. most of the affected children are older than 12 mo at onset

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 C. Parvovirus B19 infections is a common causative agent
D. virtually all children recover within 1-2 months
E. corticosteroid therapy is of no value

6. The valuable diagnostic feature that differentiate anemia of chronic disorder from
iron deficiency anemia is
A. low serum iron
B. low or normal serum transferrin
C. normal bone marrow cellularity
D. normochromic normocytic RBC
E. leukocytosis

7. All the following are true regarding physiologic anemia of infancy EXCEPT
A. it is exaggerated in premature infants
B. the hemoglobin concentration range is between 9-11 g/dL
C. the hemoglobin concentration rarely falls below 10 g/dL in healthy infants
D. vitamin E deficiency does not play a role
E. iron deficiency is a contributing factor

8. Folic acid deficiency may develop in the following children EXCEPT

A. a term infant fed on goat`s milk
B. an infant fed on unfortified powdered milk
C. an infant fed on pasteurized cow's milk
D. a child with chronic use of phenytoin anticonvulsant
E. a child with trimethoprim prophylaxis for recurrent urinary tract infection

9. A 1-year-old child has folic acid deficiency since the age of 4 months, the best
indicator of this deficiency is
A. significant increment of mean corpuscular volume MCV
B. significant fall of reticulocytes count
C. high level of lactate dehydrogenase LDH
D. decrease level of RBC folate
E. increase number of hypersegmented neutrophils

10. The first laboratory marker in progressive iron deficiency anemia is

A. depletion of bone marrow hemosiderin
B. falling of serum ferritin
C. decrease of serum iron and increase of the iron-binding capacity
D. decrease hemoglobin synthesis
E. hypochromic microcytic anemia

11. An extremely high RBC distribution width (RDW) is MOST likely consistent with

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 A. iron deficiency anemia
B. megaloblastic anemia
C. sideroblastic anemia
D. thalassemia
E. sickle cell anemia

12. The laboratory finding that is MOST likely consistent with the diagnosis of
spherocytosis is
A. high reticulocytes count of more than 10%
B. normal mean corpuscular volume MCV
C. low mean corpuscular hemoglobin MCH
D. high mean corpuscular hemoglobin concentration MCHC
E. indirect hyperbilirubinemia

13. In hereditary spherocytosis, all the following will resolve postsplenectomy EXCEPT
A. osmotic fragility
B. anemia
C. reticulocytosis
D. hyperbilirubinemia
E. hypoplastic/aplastic crises

14. Splenectomy is recommended in all the following conditions EXCEPT

A. a 6-year-old child with hereditary spherocytosis and significant hemolysis
B. a 7-year-old child with hereditary elliptocytosis and a hemoglobin level of 7
g/dL and corrected reticulocytes count of more than 15%
C. an 8-year-old child with hereditary stomatocytosis with hemolysis
D. a 6-year-old child with thalassemia major with splenomegaly and frequent
blood transfusion requirement
E. a 12-year-old child with chronic immune thrombocytopenia not responding to
all modalities of medical treatment

15. The best diagnostic test for paroxysmal nocturnal hemoglobinuria (PNH) is
A. acidified serum hemolysis (HAM) test
B. sucrose lysis test
C. complement assay
D. flow cytometry
E. bone marrow study

16. All the following may occur as a renal manifestation in a child with sickle cell
anemia EXCEPT
A. polyuria
B. hyposthenuria

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 C. nephrotic syndrome
D. hematuria
E. acute renal injury

17. The least common infection that may occur in a 6-year-old child with sickle cell
anemia is
A. Pneumococcus sepsis
B. Haemophilus influenzae meningitis
C. Salmonella osteomyelitis
D. E.coli urinary tract infection
E. Parvovirus B19 aplastic episode

18. Blood transfusion therapy in a child with sickle cell anemia is indicated in all the
following conditions EXCEPT
A. acute chest syndrome
B. stroke
C. chronic intolerable pain syndrome
D. avascular necrosis of the hip joint
E. splenic sequestration

19. The best assessment of iron overload for patients with thalassemia major is
achieved by
A. liver MRI
B. bone marrow biopsy
C. serum iron
D. serum ferritin
E. total iron binding capacity

20. The criteria for successful bone marrow transplantation in thalassemic patients
include all the following EXCEPT
A. age younger than 15-year-old
B. no hepatomegaly
C. no bone deformities
D. no iron overload
E. full HLA-matched sibling

21. The best treatment for spinal cord compression at the vertebral canal with
neurological symptoms caused by extramedullary hematopoiesis in thalassemic
patients is
A. blood transfusion
B. splenectomy
C. iron chelation therapy

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 D. local radiotherapy
E. vitamin D supplementation

22. You are evaluating a 4-year-old child with ß-thalassemia major; he is on chronic
transfusion therapy since the age of 1 year; you suspect transfusion-induced
hemosiderosis.
Of the following, the organ that is LEAST likely to be affected by iron deposition at this
time is
A. liver
B. pancreas
C. heart
D. pituitary gland
E. thyroid gland

23. A healthy 5-mo-old boy appears pale. Examination is unremarkable. Lab findings
include: Hb 8.1 g/dl; WBC 4,800/mm3; platelets 144,000/mm3; MCV, 111 fl; blood film
showed hypersegmented neutrophils; serum B12, 65 pg/mL (low). The infant is
vigorously breast fed.
Of the following, the NEXT step of the management is
A. performing Schilling test
B. check serum B12 of the mother
C. parenteral administration of vitamin B12
D. administration of both folic acid and vitamin B12
E. transfuse packed RBCs

24. A 9-month-old child with a hemoglobin concentration of 10 gm/dL and marked

microcytosis; serum iron and total iron binding capacity are within normal limits; serum
ferritin and hemoglobin electrophoresis are also normal.
Of the following, the MOST likely diagnosis is
A. iron deficiency anemia
B. sideroblastic anemia
C. ß-thalassemia minor
D. α-thalassemia trait
E. anemia due to chronic disease

25. Hereditary intrinsic factor deficiency (HIFD), formerly called congenital pernicious
anemia, differs from the typical adult pernicious anemia by all the following EXCEPT
A. it is prominent at around 3 mo of age
B. stomach secretes acid normally
C. stomach is histologically normal
D. there are no antibodies to parietal cells
E. there are no associated endocrine disorders

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26. Increased incubated osmotic fragility test that is not corrected by the addition of
glucose is MOST likely suggestive of
A. hereditary spherocytosis
B. hereditary elliptocytosis
C. hereditary stomatocytosis
D. glucose-6-phosphate dehydrogenase deficiency
E. pyruvate kinase deficiency

27. All the following may cause autoimmune hemolytic anemia EXCEPT
A. systemic lupus erythematosus (SLE)
B. immunodeficiency
C. mycoplasma pneumoniae infection
D. lymphoproliferative disorder
E. penicillin drug administration

28. All the following are correct regarding treatment of cold agglutinin disease EXCEPT
A. patient should avoid exposure to cold
B. patient should be treated for underlying disease
C. plasmapheresis is a modality of treatment
D. glucocorticoids treatment is a modality of treatment
E. patient can be treated by splenectomy when there is no response to other
modalities of treatment

29. Fragmentation hemolysis by mechanical injury may be seen in all the following
EXCEPT
A. extensive burns
B. Kasabach-Merritt syndrome
C. after cardiac surgery for prosthetic heart valve replacement
D. thrombotic thrombocytopenic purpura (TTP)
E. hemolytic uremic syndrome (HUS)

30. All the following conditions may be associated with iron deficiency EXCEPT
A. hookworm infestation
B. prolonged intravascular hemolysis
C. celiac disease
D. congenital heart disease with right to left shunt
E. prolonged use of isoniazid (INH)

31. Parvo virus B19 infection may cause transient pancytopenia in

A. thalassemia major
B. sickle cell anemia
C. hereditary spherocytosis

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 D. hereditary elliptocytosis
E. pyruvate kinase deficiency

32. Fresh frozen plasma (FFP) transfusion is the best treatment for which of the
following conditions
A. hemophilia a
B. hemophilia b
C. factor vii deficiency
D. immunoglobulin replacement therapy
E. bleeding due to warfarin therapy

33. Transfusion of fresh frozen plasma (FFP) is efficacious for the treatment of
deficiency of all the following coagulation factors EXCEPT
A. factor V
B. factor X
C. factor XI
D. factor XIII
E. protein C

34. Fresh frozen plasma (FFP) transfusion/replacement is indicated in all the following
conditions EXCEPT
A. a 9-year-old child with chronic liver disease with prolonged clotting times who
needs a liver biopsy
B. a 6-year-old child with protein S deficiency and significant deep venous
thrombosis
C. an 11-year-old child with thrombotic thrombocytopenic purpura (TTP) post
allogenic bone marrow transplantation
D. a 2-day-old preterm neonate with clotting deficiencies to prevent
intraventricular hemorrhage
E. a 1-week-old term neonate with hemorrhage secondary to vitamin K deficiency

35. The main reason behind gamma irradiation of the blood products is to prevent
A. transfusion-related bacterial infections
B. transfusion-related viral infections
C. graft-versus-host disease (GVHD)
D. allergic reactions
E. transfusion-related acute lung injury (TRALI)

36. CMV-transmitted infection is least likely occurring in transfusion of which of the

following blood products?
A. whole blood
B. packed RBCs

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 C. granulocytes
D. platelets
E. fresh frozen plasma

37. A prolonged thrombin time with normal reptilase time is consistent with
A. dysfibrinogenemia
B. afibrinogenemia
C. factor XIII deficiency
D. disseminated intravascular coagulopathy (DIC) with high level of fibrin split
products
E. heparin overdose

38. In hemophilia A, factor VIII level activity should be increased to 100% in

A. epistaxis
B. iliopsoas bleeding
C. tooth extraction
D. hematuria
E. gum bleeding

39. A 9-year-old male child is about to do elective tonsillectomy; preoperative

investigations are all normal except for a significant prolonged partial thromboplastin
time PTT; there was no previous bleeding after circumcision and emergency
appendectomy.
Of the following, the MOST likely diagnosis is
A. hemophilia A
B. hemophilia B
C. hemophilia C
D. prekallikrein deficiency
E. factor XIII deficiency

40. Spontaneous intracranial hemorrhage is more likely to occur in

A. factor VII deficiency
B. factor VIII deficiency
C. factor IX deficiency
D. factor XI deficiency
E. factor XIII deficiency

41. Systemic amyloidosis may be associated with deficiency of

A. factor VII
B. factor VIII
C. factor IX
D. factor X

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 E. factor XI

42. In which of the following conditions you should use a plasma that is frozen within
24 hours after collection?
A. factor V deficiency
B. factor VII deficiency
C. factor IX deficiency
D. factor X deficiency
E. factor XI deficiency

43. All the following are true regarding factor XIII EXCEPT
A. the half-life is 5-7 days
B. PT, PTT, and thrombin time (TT) are prolonged when the factor is deficient
C. clot solubility test with 5m urea is a screening test for factor XIII deficiency
D. factor assay is a specific test to detect its level
E. the hemostatic level is only 2-3%

44. In severe liver disease with prolongation of the bleeding time, the best effective
treatment before doing liver biopsy is
A. intravenous vitamin K
B. intramuscular vitamin K
C. oral vitamin K
D. fresh frozen plasma
E. intravenous DDAVP

45. Vitamin K-dependent clotting factors include all the following EXCEPT
A. factor I
B. factor II
C. factor VII
D. factor IX
E. protein S

46. A 16-year-old female is found to have multiple bruising and hypochromic microcytic
anemia; during her first pregnancy she noticed disappearance of the bruises; she has a
past history of uncomplicated emergency appendectomy at the age of 10 year.
Of the following, the drug MOST likely beneficial for alleviation of her symptoms is
A. ferrous sulphate
B. desmopressin acetate (DDAVP)
C. recombinant factor VIII
D. recombinant factor IX
E. aminocaproic acid

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47. Low platelets count may be encountered in all the following EXCEPT
A. Von Willebrand disease (VWD) type 2 B
B. VWD type 2 N
C. Platelet type pseudo VWD
D. Bernard Soulier syndrome
E. Wiskott Aldrich syndrome

48. Which of the following is FALSE about factor V Leiden mutation?

A. it is the most common inherited risk factor for thrombosis
B. heterozygous individuals have less risk for thrombosis than homozygotes
C. heterozygotes have an increased risk of arterial thrombosis
D. there is an increased frequency of thrombosis while receiving oral
contraceptive agents
E. there is an increased risk of recurrent abortions

49. Arterial thrombosis is the least likely cause of

A. stroke
B. a cold and pulseless lower extremity
C. renal infarction
D. myocardial infarction
E. pulmonary embolism

50. Lupus anticoagulants usually affect the following laboratory test

A. bleeding time (BT)
B. partial thromboplastin time (PTT)
C. prothrombin time (PT)
D. thrombin time (TT)
E. clot solubility test

51. Which of the following is FALSE regarding standard unfractionated heparin

therapy?
A. it acts by enhancing the antithrombin III activity
B. it can be used safely during pregnancy
C. it should not be given for a child with bacterial endocarditis
D. it can be given intramuscularly
E. it needs monitoring by PTT test

52. All the following are true regarding low molecular weight heparin (LMWH) in
comparison with unfractionated heparin (UFH) EXCEPT
A. it is more stable
B. it is ease to titrate
C. it is monitored by activated PTT

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 D. it can be used subcutaneously
E. it can be used as an outpatient

53. A healthy 5-day-old male neonate develops bruising and melena. The pregnancy,
and delivery course were unremarkable. The mother received heparin therapy for
recurrent abortions secondary to lupus anticoagulants. The infant is vigorously
breastfeeding. Examination reveals only multiple bruises. Lab tests reveals:
hemoglobin, 8.4 g/dL; WBC, 8600/mm3; platelets count, 200000/mm3; PTT, 74
seconds; PT, 28 seconds; serum fibrinogen is normal.
Of the following, the MOST likely cause of this bleeding is
A. disseminated intravascular coagulopathy (DIC)
B. hemophilia
C. vitamin K deficiency
D. heparin therapy
E. lupus anticoagulants

54. A previously healthy 10-month-old boy develops pallor over 2 weeks. Examination
is unremarkable except for pallor. Lab tests includes: hemoglobin, 4.9 g/dL; WBC,
6700/mm3; platelets count, 180000/mm3; MCV, 79 fL; reticulocyte count 0.8%;
hemoglobin electrophoresis, normal.
Of the following, the MOST likely cause for these findings is
A. pure red cell aplasia
B. transient erythroblastopenia of childhood
C. thalassemia minor
D. aplastic anemia
E. parvovirus infection

55. You are evaluating a 5-year-old male child with multiple ecchymoses distributed all
over the extremities and trunk; examination reveal a palpable spleen 3 cm below the
left costal margin. Lab findings include: hemoglobin, 11.3 g/dL; white blood cell count,
8700/mm3; platelets count, 21000/mm3.
Of the following, the most proper NEXT step is
A. bleeding time
B. coagulation profile
C. platelets aggregation test
D. abdominal ultrasonography
E. bone marrow study

56. Both quantitative and qualitative platelets defects are seen in

A. generalized vasculitis
B. Bernard-Soulier syndrome
C. Glanzmann's Thrombasthenia

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 D. type I von Willebrand disease
E. uremia

57. Which of the following viruses is more likely to be associated with chronic immune
thrombocytopenia?
A. cytomegalovirus
B. parvovirus
C. influenza virus
D. parainfluenza virus
E. human immune deficiency virus

58. Which of the following is least likely to be associated with prolonged

thrombocytopenia in children?
A. immune thrombocytopenia
B. systemic lupus erythematosus (SLE)
C. HIV infection
D. type 2B von Willebrand disease
E. Wiskott-Aldrich syndrome (WAS)

59. Which of the following drugs is least likely to cause thrombocytopenia in children?
A. valproic acid
B. phenytoin
C. sulfonamides
D. trimethoprim-sulfamethoxazole
E. heparin

60. Microthrombocytes are MOST likely to be seen in

A. immune thrombocytopenia (ITP)
B. Bernard-Soulier syndrome
C. Wiskott-Aldrich syndrome (WAS)
D. Glanzmann's Thrombasthenia
E. type 2B von Willebrand disease

61. Thrombocytopenia that improves with age is MOST likely a feature of

A. congenital amegakaryocytic thrombocytopenia
B. Bernard-Soulier syndrome
C. Wiskott-Aldrich syndrome
D. thrombocytopenia absent radius syndrome (TAR)
E. aplastic anemia

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62. You are going to explain the role of anti-D therapy to the parents of a 4-year-old
boy with acute immune thrombocytopenia (ITP) who has several ecchymotic lesions
located over extremities and trunk. There is no mucosal bleeding.
An important statement that should be included during the discussion is
A. anti-D therapy is more effective than IVIG therapy in acute ITP
B. anti-D therapy is very effective to all children with ITP
C. when anti-D therapy is given, monitoring of hemoglobin concentration is
recommended
D. anti-D therapy is used in acute ITP only
E. anti-D therapy can be used as initial treatment for children with acute ITP

63. A 9-month-old boy appears pale. Examination reveals a palpable liver 3 cm below
the right costal margin and palpable spleen 4 cm below the left costal margin. Lab
findings include: hemoglobin concentration, 6.4 g/dl; mean corpuscular volume (MCV),
67 fL; and WBC, 15,000/mm3.
Of the following, The MOST valuable test to confirm the diagnosis is
A. blood smear
B. serum iron, total iron binding capacity, and serum ferritin
C. hemoglobin electrophoresis
D. bone marrow study
E. abdominal ultrasonography

64. You are explaining the risk of sepsis after splenectomy to medical students. An
important statement that should be included during this discussion is that risk is
especially high in children
A. older than 5 yr at the time of surgery
B. with hereditary spherocytosis
C. with chronic immune thrombocytopenia (ITP)
D. after trauma
E. with Hodgkin lymphoma

65. You are evaluating a 7-year-old male child with cervical lymphadenopathy; the
MOST likely finding that raise your suspicion of malignancy is
A. matted nodes
B. fluctuant nodes
C. fixed nodes
D. presence of systemic symptoms
E. no regression in the size of the nodes to its normal within 2 weeks

66. Of the following, the MOST accurate reflection of active erythropoiesis of the bone
marrow is
A. hemoglobin concentration

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 B. packed cell volume (PCV)
C. reticulocyte count
D. mean corpuscular volume (MCV)
E. reticulocyte index

67. Hematologic disease with upper limb orthopedic congenital abnormalities with
presence of thumbs is MOST likely seen in
A. Fanconi anemia
B. thrombocytopenia with Absent Radii (TAR) Syndrome
C. congenital Amegakaryocytic Thrombocytopenia
D. Glanzmann Thrombasthenia
E. Bernard-Soulier Syndrome

68. Which of the following is not a feature of Wiskott–Aldrich Syndrome (WAS)

A. thrombocytopenia
B. eczema
C. recurrent otitis media and pneumonia
D. propensity to develop autoimmune disorders
E. giant platelets by blood smear

69. A 5-year-old child has multiple bruises on her extremities and oral mucosal bleeding
of 3 days duration; she had a mild respiratory tract infection 2 weeks before; physical
examination was normal apart from multiple ecchymoses and petechiae.
Of the following, the NEXT diagnostic step is
A. complete blood count
B. prothrombin time
C. bleeding time
D. partial thromboplastin time
E. antinuclear antibody titer

70. Of the following, the LEAST likely feature of Bernard-Soulier syndrome is

A. autosomal recessive inheritance
B. severe thrombocytopenia
C. giant Platelets
D. abnormal ristocetin test
E. platelet transfusion is the only reliable therapy

71. Each of the following may cause thrombocytopenia EXCEPT

A. heparin therapy
B. aspirin ingestion
C. disseminated intravascular coagulation (DIC)
D. systemic lupus erythematosus (SLE)

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 E. hemolytic-uremic syndrome (HUS)

72. Which of the following children with acute immune thrombocytopenia is MOST
likely to have a benefit from splenectomy?
A. an 8-year-old girl with multiple bruises and platelets count of 10,000/ml
B. a 6-year-old boy with mild epistaxis and platelets count of 20,000/ml
C. a 4-year-old boy with subdural hematoma and platelets count of 100,000/ml
after receiving IVIG
D. an 11-year-old boy with severe rectal bleeding and platelets count of 20000/ml
not responding to medical treatment
E. a 13-year-old female adolescent with moderate menstrual bleeding and
platelets count of 50000/ml

73. Of the following, the LEAST likely feature of acute immune thrombocytopenia (ITP)
is
A. a preceding viral infection
B. isolated thrombocytopenia
C. mucocutaneous bleeding
D. isolated splenomegaly
E. increased number of megakaryocytes in the bone marrow

74. A 3-year-old male child develops hematoma and bruising of his right hand next day
after falling on the ground; the mother stated that her child has a poor wound healing
and a history of delayed umbilical separation during the neonatal period.
Of the following, the MOST valuable test for this case is
A. bleeding time
B. partial thromboplastin time
C. prothrombin time
D. thrombin time
E. clot solubility test

75. A 5-year-old child, with hemophilia A of severe type, presents to the emergency
unit with a groin pain after a minor trauma to his back; his blood pressure is 60/30 mm
Hg; his pulse rate is 180/min; he holds his right hip in a flexion position with internal
rotation.
Of the following, the NEXT step in the management of this child is
A. factor VIII replacement therapy
B. intravenous 1-deamino-8-d-arginine vasopressin (DDAVP)
C. factor VIII assay
D. abdominal ultrasonography
E. abdominal computed tomography (CT) scan

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76. In a child with a recent diagnosis of hemophilia A who developed moderate
epistaxis, the aim of the treatment is to increase the level of factor VIII to
A. 5-20%
B. 35-50%
C. 55-60%
D. 65-70%
E. 80-100%

77. Allogeneic bone marrow stem cell transplantation is least likely to be required in
A. Fanconi anemia
B. thrombocytopenia with Absent Radii (TAR) Syndrome
C. congenital Amegakaryocytic Thrombocytopenia (CAT)
D. Wiskott –Aldrich Syndrome (WAS)
E. severe combined immune deficiency

78. Inherited thrombocytopenias include all the following EXCEPT

A. congenital Amegakaryocytic Thrombocytopenia
B. thrombocytopenia with Absent Radii (TAR) Syndrome
C. Glanzmann’s Thrombasthenia
D. Bernard-Soulier Syndrome
E. Wiskott–Aldrich Syndrome (WAS)

79. Which of the following is the best treatment for a 5-year-old girl with acute immune
thrombocytopenia (ITP) and platelets count of 30,000/ml and mild petechial rash all
over her body?
A. intravenous immunoglobulin (IVIG)
B. anti-D gamma globulin
C. corticosteroids
D. platelets transfusion
E. no treatment

80. The standard care for MOST children with severe hemophilia is
A. prevention by F VIII replacement therapy to prevent spontaneous bleeding and
early joint deformities
B. aggressive treatment by F VIII replacement therapy when significant bleeding
occurs
C. avoid trauma
D. avoid aspirin and other NSAID
E. avoid violent contact sports

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