Deck 8: Aplastic Anemia Including Pure Red Cell Aplasia and Congenital Dyserythropoietic Anemia and Paroxysmal Nocturnal Hemoglobinuria

Question 1

What is the main complication of bone marrow transplants in a patient diagnosed with aplastic anemia?

A) Graft rejection
B) Transfusion reactions
C) Acute graft versus host disease
D) A and B
E) A and C

Answer 1

A and C

Question 2

A patient presents with the following: bone marrow examination reveals multinuclear erythroblasts, megaloblastic synchrony, and thin internuclear chromatin bridges between two erythroblasts. Which condition is most likely present?

A) CDA type 1
B) CDA type 2
C) CDA type 3
D) CDA type 4
E) None of the above

Answer 2

CDA type 1

Question 3

What would the RBC indices reveal in a patient with aplastic anemia?

A) Normocytic/hypochromic
B) Macrocytic/normochromic
C) Normocytic/normochromic
D) Microcytic/hypochromic
E) None of the above

Answer 3

Normocytic/normochromic

Question 4

Which qualitative term describes the bone marrow in aplastic anemia?

A) Hyperplasia
B) Hypoplasia
C) Hypercellular
D) Synchrony
E) Asynchrony

Question 5

What is a characteristic finding in CDA type 3?

A) Positive HEMPAS test
B) Megaloblastic synchrony
C) Giant multinucleated erythroblasts
D) All of the above
E) None of the above

Question 6

Which is a congenital disease associated with aplastic anemia?

A) Fanconi's anemia
B) Richter's syndrome
C) Hodgkin's disease
D) Kala-azar
E) None of the above

Question 7

A patient is diagnosed with an associated aplastic anemia syndrome. His bone marrow examination reveals normal cellularity with absence of erythroid precursors. What is the most likely condition?

A) Congenital dyserythropoietic anemia
B) Pure red cell aplasia
C) Fanconi's anemia
D) Erythroleukemia
E) None of the above

Question 8

Bone marrow aspiration of a patient with aplastic anemia often results in:

A) Direct smear
B) Particle smear
C) Dry tap
D) Clotted specimen
E) None of the above

Question 9

What is the treatment of choice for a patient younger than 50 years of age who has aplastic anemia?

A) Bone marrow transplant
B) Steroids
C) Androgens
D) Cord blood transplant
E) None of the above

Question 10

How is CDA type 2 characterized?

A) Mononuclear erythroblasts
B) Negative HEMPAS (hereditary erythroblast multinuclearity with positive acidified serum) test
C) Positive HEMPAS test
D) Megaloblastic synchrony
E) None of the above

Question 11

Symptoms of aplastic anemia include all except:

A) Fatigue
B) Dyspnea
C) Palpitation
D) Dehydration
E) None of the above

Question 12

Which population of cells is thought to be damaged in the bone marrow, which results in pancytopenia in the peripheral blood in aplastic anemia?

A) Megakaryocytes
B) Colony-stimulating factors
C) Progenitor cells
D) Pluripotent stem cells
E) None of the above

Question 13

All may be present in the peripheral blood of a patient with aplastic anemia except:

A) Myelocyte
B) Metamyelocyte
C) Relative lymphocytosis
D) Reticulocytosis
E) None of the above

Question 14

Which qualitative term is characteristically used to describe the peripheral blood smear in aplastic anemia?

A) Thrombocytopenia
B) Pancytopenia
C) Leukocytosis
D) Erythrocytosis
E) None of the above

Question 15

What is characteristic of congenital dyserythropoietic anemia (CDA)?

A) Ineffective erythropoiesis
B) Multinuclear erythroblasts
C) Direct hyperbilirubinemia
D) A and B
E) B and C

Question 16

What is the etiology of aplastic anemia?

A) Radiation
B) Drugs
C) Chemicals
D) All of the above
E) None of the above

Question 17

A patient who is diagnosed with aplastic anemia and has no history of exposure to drugs, radiation, or chemicals can be said to have:

A) Acquired aplastic anemia
B) Congenital aplastic anemia
C) Idiopathic aplastic anemia
D) Genetic defect
E) None of the above

Question 18

What does a bone biopsy reveal in a patient with aplastic anemia?

A) Hypercellularity
B) Hypocellularity
C) Increase in all cell lines
D) Two of the above
E) None of the above

Question 19

Which drug(s) is/are the primary cause(s) of aplastic anemia?

A) Chloramphenicol
B) Phenylbutazone
C) Penicillin
D) A and B
E) B and C

Question 20

What is the most common cause of aplastic anemia?

A) X-ray exposure
B) Benzene exposure
C) Gamma-ray exposure
D) Arsenic exposure
E) Idiopathic

Question 21

The abnormal red cell membrane in PNH is highly sensitive to:

A) Alloantibodies
B) Complement
C) Drug-induced antibodies
D) IL-1
E) None of the above

Question 22

One of the major complications of PNH is:

A) Iron overload
B) Venous thrombosis
C) Splenomegaly
D) Hepatomegaly
E) None of the above

Question 23

What would be considered a typical platelet count in a patient with PNH?

A) >500  10⁹/L
B) <50  10⁹/L
C) 50-100  10⁹/L
D) 100-400  10⁹/L
E) None of the above

Question 24

The defect in PNH affects which cell type?

A) Leukocytes
B) Platelets
C) Erythrocytes
D) All of the above
E) None of the above

Question 25

Most PNH patients present with a normocytic/normochromic anemia and:

A) Periodic acute hemolytic episodes
B) Bone marrow hypoplasia
C) Cytopenia
D) All of the above
E) None of the above

Question 26

Slight macrocytosis or polychromasia may be seen in the peripheral blood of a PNH patient due to increased __________.

A) Leukocytes
B) Reticulocytes
C) Erythrocytes
D) NRBCs
E) Platelets

Question 27

Which statement is true regarding a positive Ham's test result?

A) Hemolysis occurs with control cells but not patient cells.
B) Hemolysis is enhanced with heat-activated serum.
C) Hemolysis occurs with a patient's cells but not control cells.
D) Hemolysis is enhanced by complement-deficient acidified serum.
E) None of the above

Question 28

The bone marrow in a PNH patient reveals:

A) Erythroid hyperplasia
B) Myeloid hyperplasia
C) Erythroid hypoplasia
D) Myeloid hypoplasia
E) None of the above

Question 29

Which factors help to determine the presence of PNH?

A) Increased acetylcholinesterase
B) Decrease in acetylcholinesterase
C) Increase in leukocyte alkaline phosphatase (LAP)
D) Spherocytosis
E) None of the above

Question 30

Which condition will lead to iron-deficiency anemia masking the diagnosis of PNH?

A) Hemoglobinuria
B) Hemosiderinuria
C) Hemoglobinemia
D) Hemochromatosis
E) None of the above

Question 31

Which laboratory findings are associated with chronic hemolysis of PNH?

A) Leukopenia
B) Thrombocytopenia
C) Thrombocytosis
D) A and B
E) A and C

Question 32

When aplastic anemia occurs in conjunction with PNH, what morphological condition(s) is/are present?

A) Pancytopenia
B) Marrow hypoplasia
C) Hemoglobinuria
D) All of the above
E) None of the above

Question 33

The defect in PNH is associated with:

A) Defect in globin chains
B) Abnormal clone of hematopoietic stem cells
C) Defect in porphyrin synthesis
D) All of the above
E) None of the above

Question 34

The presentation of hemoglobinuria in a PNH patient is due to:

A) Intravascular hemolysis
B) Extravascular hemolysis
C) Biphasic hemolysis
D) Immune hemolysis
E) None of the above

Question 35

The definition of aplastic anemia represents what morphological characteristics?

A) Decreased RBCs
B) Decreased white blood cells (WBCs)
C) Decreased platelets
D) Decreased reticulocytes
E) All of the above

Question 36

A person diagnosed with PNH may have which of the following in his or her urine?

A) Intact red cells
B) Red blood cell casts
C) Hemoglobin casts
D) All of the above
E) None of the above

Question 37

The diagnosis of PNH depends on the detection of complement-sensitive __________ in the peripheral blood.

A) Granulocytes
B) Platelets
C) Erythrocytes
D) Monocytes
E) None of the above

Question 38

Which of the following represents an acquired intracorpuscular defect?

A) Paroxysmal nocturnal hemoglobinuria (PNH)
B) RBC membrane defects
C) Paroxysmal cold hemoglobinuria (PCH)
D) Thalassemia
E) Immune hemolytic anemias

Question 39

Schistocytes or fragmented red cells seen on a peripheral blood smear of a PNH patient may indicate:

A) Intravascular thrombosis
B) Extravascular thrombosis
C) Liver damage
D) Vitamin B₁₂ deficiency
E) None of the above

Question 40

Which classes of PNH is deficient in both decay-accelerating factor (DAF) and membrane inhibitor of reactive lysis (MIRL)?

A) PNH I and PNH II
B) PNH II and PNH III
C) PNH I, PNH II, and PNH III
D) PNH I only
E) None of the above

Question 41

What is the function of decay-accelerating factor (DAF) as a complement protein?

A) Accelerates the spontaneous decay of C5-convertase
B) Accelerates the spontaneous decay of C3-convertase
C) Slows the spontaneous decay of C5-convertase
D) Slows the spontaneous decay of C3-convertase
E) None of the above

Question 42

Which immunophenotype is associated with a diagnosis of PNH?

A) CD10, 12, 14 positive
B) CD1, 2, 3, 5 positive
C) CD14, CD16, CD24, CD48 positive
D) All of the above
E) None of the above

Question 43

What membrane deficiency renders PNH erythrocytes more sensitive to lysis by complement?

A) C3 convertase enzymes
B) GPI-anchor proteins
C) Acetylcholinesterase
D) All of the above
E) None of the above

Question 44

The most common cause of death in a PNH patient is:

A) Iron overload
B) Liver disease
C) Thromboembolism
D) Anemia
E) None of the above

Question 45

Which test is used to confirm the diagnosis of PNH?

A) Autohemolysis test
B) Sugar water test
C) Ham's test
D) Coombs' test
E) None of the above

Question 46

In differentiating PNH red cells from normal red cells in the sugar water test, PNH red cells will be _________________.

A) Unaffected
B) Lysed
C) Fragmented
D) Sickled
E) None of the above

Question 47

In PNH, treatment is directed at controlling complications that arise, such as:

A) Infections
B) Anemia
C) Thrombosis
D) All of the above
E) None of the above

Question 48

What percentage of cell lysis in a sugar water test is considered negative for PNH?

A) <5%
B) 10-50%
C) 10-80%
D) 80-100%
E) None of the above