Heme 1.7 Lymphoproliferative and Myeloproliferative Disorders Flashcards

1
Q
  1. Repeated phlebotomy in patients with PV may lead to the development of:

A. Folic acid deficiency
B. Sideroblastic anemia
C. IDA
D. Hemolytic anemia

A

C. IDA

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2
Q
  1. In ET, the PLTs are:

A. Increased in number and functionally abnormal
B. Normal in number and functionally abnormal
C. Decreased in number and functional
D. Decreased in number and functionally abnormal

A

A. Increased in number and functionally abnormal

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3
Q
  1. Which of the following cells is considered pathogenomonic for Hodgkin disease?

A. Niemann–Pick cells
B. Reactive lymphocytes
C. Flame cells
D. Reed–Sternberg (RS) cells

A

D. Reed–Sternberg (RS) cells

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4
Q
  1. In myelofibrosis, the characteristic abnormal RBC morphology is that of:

A. Target cells
B. Schistocytes
C. Teardrop-shaped cells
D. Ovalocytes

A

C. Teardrop-shaped cells

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5
Q
  1. PV is characterized by:

A. Increased plasma volume
B. Pancytopenia
C. Decreased O2 saturation
D. Absolute increase in total RBC mass

A

D. Absolute increase in total RBC mass

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6
Q
  1. In PV, what is characteristically seen in peripheral blood?

A. Panmyelosis
B. Pancytosis
C. Pancytopenia
D. Panhyperplasia

A

B. Pancytosis

PV is a myeloproliferative disorder characterized by uncontrolled proliferation of erythroid precursors. However, production of all cell lines is usually increased. Panhyperplasia is a term used to describe the cellularity of bone marrow in PV.

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7
Q
  1. Features of secondary polycythemia include all of the following except:

A. Splenomegaly
B. Decreased O2 saturation
C. Increased RBC mass
D. Increased erythropoietin

A

A. Splenomegaly

Splenomegaly is a feature of PV but not characteristic of secondary polycythemia. The
RBC mass is increased in both primary polycythemia (PV) and secondary polycythemia. Erythropoietin is increased, and O2 saturation is decreased in secondary polycythemia.

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8
Q
  1. Erythrocytosis in relative polycythemia occurs because of:

A. Decreased arterial O2 saturation
B. Decreased plasma volume of circulating blood
C. Increased erythropoietin levels
D. Increased erythropoiesis in bone marrow

A

B. Decreased plasma volume of circulating blood

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9
Q
  1. Leukocyte alkaline phosphatase (LAP) staining performed on a patient gives the following results:
    10(0)
    48(1+)
    38(2+)
    3(3+)
    1(4+)
    Calculate the LAP score.

A. 100
B. 117
C. 137
D. 252

A

C. 137

One hundred mature neutrophils are counted and scored. The LAP score is calculated as follows:
(the number of 1+ cells × 1) + (2+ cells × 2) + (3+ cells × 3) + (4+ cells × 4).
That is, 48 + 76 + 9 + 4 = 137. The reference range is approximately 20 to 130.

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10
Q
  1. CML is distinguished from leukemoid reaction by which of the following?

A. CML: low LAP; leukemoid: high LAP
B. CML: high LAP; leukemoid: low LAP
C. CML: high WBC; leukemoid: normal WBC
D. CML: high WBC; leukemoid: higher WBC

A

A. CML: low LAP; leukemoid: high LAP

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11
Q
  1. Which of the following occurs in idiopathic myelofibrosis (IMF)?

A. Myeloid metaplasia
B. Leukoerythroblastosis
C. Fibrosis of bone marrow
D. All of these options

A

D. All of these options

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12
Q
  1. What influence does the Philadelphia (Ph1) chromosome have on the prognosis of patients with CML?

A. It is not predictive
B. The prognosis is better if Ph1 is present
C. The prognosis is worse if Ph1 is present
D. The disease usually transforms into AML when Ph1 is present

A

B. The prognosis is better if Ph1 is present

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13
Q
  1. Which of the following is (are) commonly found in CML?

A. Many teardrop-shaped cells
B. Intense LAP staining
C. A decrease in granulocytes
D. An increase in basophils

A

D. An increase in basophils

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14
Q
  1. In which of the following conditions does LAP show the least activity?

A. Leukemoid reactions
B. IMF
C. PV
D. CML

A

D. CML

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15
Q
  1. A striking feature of the peripheral blood of a patient with CML is:

A. Profusion of bizarre blast cells
B. Normal number of typical granulocytes
C. Presence of granulocytes at different stages of development
D. Pancytopenia

A

C. Presence of granulocytes at different stages of development

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16
Q
  1. Which of the following is often associated with CML but not with AML?

A. Infections
B. WBCs greater than 20.0 × 109/L
C. Hemorrhage
D. Splenomegaly

A

D. Splenomegaly

17
Q
  1. Multiple myeloma and Waldenström macroglobulinemia have all of the following in common except:

A. Monoclonal gammopathy
B. Hyperviscosity of blood
C. Bence–Jones protein in urine
D. Osteolytic lesions

A

D. Osteolytic lesions

Osteolytic lesions indicating destruction of bone, as evidenced by radiography, are seen in multiple myeloma but not in Waldenström macroglobulinemia. In addition, Waldenström macroglobulinemia gives rise to lymphocytosis, which does not occur in multiple myeloma, and differs in the morphology of the malignant cells.

18
Q
  1. What is the characteristic finding seen in the peripheral blood smear from a patient with multiple myeloma?

A. Microcytic hypochromic cells
B. Intracellular inclusion bodies
C. Rouleaux
D. Hypersegmented neutrophils

A

C. Rouleaux

19
Q
  1. All of the following are associated with the diagnosis of multiple myeloma except:

A. Marrow plasmacytosis
B. Lytic bone lesions
C. Serum and/or urine M component (monoclonal protein)
D. Ph1 chromosome

A

D. Ph1 chromosome

20
Q
  1. Multiple myeloma is most difficult to distinguish from:

A. CLL
B. Acute myelogenous leukemia
C. Benign monoclonal gammopathy
D. Benign adenoma

A

C. Benign monoclonal gammopathy

21
Q
  1. The pathology of multiple myeloma includes which of the following?

A. Expanding plasma cell mass
B. Overproduction of monoclonal immunoglobulins
C. Production of osteoclast activating factor (OAF) and other cytokines
D. All of these options

A

D. All of these options

22
Q
  1. Waldenström macroglobulinemia is a malignancy of the:

A. Lymphoplasmacytoid cells
B. Adrenal cortex
C. Myeloblastic cell lines
D. Erythroid cell precursors

A

A. Lymphoplasmacytoid cells

Waldenström macroglobulinemia is a malignancy of lymphoplasmacytoid cells, which manufacture IgM. Although the cells secrete immunoglobulin, they are not fully differentiated into plasma cells and lack the characteristic perinuclear halo, deep basophilia, and eccentric nucleus characteristic of classic plasma cells.

23
Q
  1. Cells that exhibit positive staining with acid phosphatase and are not inhibited by tartaric acid are characteristically seen in:

A. Infectious mononucleosis
B. Infectious lymphocytosis
C. Hairy cell leukemia (HCL)
D. T-cell acute lymphoblastic leukemia

A

C. Hairy cell leukemia (HCL)

24
Q
  1. The JAK2(V617F) mutation may be positive in all of the following chronic myeloproliferative disorders except:

A. ET
B. IMF
C. PV
D. CML

A

D. CML

25
Q
  1. All of the following are major criteria for the 2008 WHO diagnostic criteria for ET except:

A. PLT count 450 × 109/L or greater
B. Megakaryocyte proliferation with large and mature morphology and no or little granulocyte or erythroid proliferation
C. Demonstration of JAK2(V617F) or other clonal marker
D. Evidence of clonality

A

D. Evidence of clonality