1.7 Lymphoproliferative and Myeloproliferative Disorders

Question 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

Answer 1

C. IDA

Question 2

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

Answer 2

A. Increased in number and functionally abnormal

Question 3

Which of the following cells in considered pathognomonic for Hodgkin disease?
A. Niemann-Pick cells
B. Reactive lymphocytes
C. Flame cells
D. Reed-Sternberg (RS) cells

Answer 3

D. Reed-Sternberg (RS) cells

Question 4

In myelofibrosis, the characteristic abnormal RBC morphology is that of:
A. Target cells
B. Schistocytes
C. Teardrop-shaped cells
D. Ovalocytes

Answer 4

C. Teardrop-shaped cells

Question 5

PV is characterized by:
A. Increased plasma volume
B. Pancytopenia
C. Decreased O2 saturation
D. Absolute increase in total RBC mass

Answer 5

D. Absolute increase in total RBC mass

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.

Question 6

Features of secondary polycythemia include all of the following except:
A. Splenomegaly
B. Decreased O2 saturation
C. Increased RBC mass
D. Increased erythropoietin

Answer 6

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.

Question 7

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

Answer 7

B. Decreased plasma volume of circulating blood

Relative polycythemia is caused by reduction of plasma, rather than an increase in RBC volume or mass. RBC mass is increased in both PV and secondary polycythemia, but erythropoietin levels are high only in secondary polycythemia

Question 8

In PV, what is characteristically seen in peripheral blood?
A. Panmyelosis
B. Pancytosis
C. Pancytopenia
D. Panhyperplasia

Answer 8

B. Pancytosis

Question 9

Leukocyte alkaline phosphatase (LAP) staining performing 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

Answer 9

C. 137

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

Question 10

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

Answer 10

A. CML: low LAP; leukemoid; high LAP

Question 11

Which of the following occurs in idiopathic myelofibrosis (IMF)?
A. Myeloid meta plasma
B. Leukoerythroblastosis
C. Fibrosis of bone marrow
D. All of these options

Answer 11

D. All of these options

Question 12

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

Answer 12

B. The prognosis is better if Ph1 is present

Question 13

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

Answer 13

D. An increase in basophils

Question 14

In which of the following conditions does LAP show the least activity?
A. Leukemoid reactions
B. IMF
C. PV
D. CML

Answer 14

D. CML

Question 15

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

Answer 15

C. Presence of granulocytes at different stages of development

Question 16

Which of the following is often associated with CML but not with AML?
A. Infections
B. WBCs greater than 20.0 x 10^9/L
C. Hemorrhage
D. Splenomegaly

Answer 16

D. Splenomegaly

Question 17

Multiple myeloma and Waldenstrom 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

Answer 17

D. Osteolytic lesions

Question 18

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

Answer 18

C. Rouleaux

Question 19

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

Answer 19

D. Ph1 chromosome

The Ph1 chromosome is a diagnostic marker for CML. Osteolytic lesions, monoclonal gammopathy, and bone marrow infiltration by plasma cells constitute the triad of diagnostic markers for multiple myeloma.

Question 20

Multiple myeloma is most difficult to distinguish from:
A. CLL
B. Acute myelogenous leukemia
C. Benign monoclonal gammopathy
D. Benign adenoma

Answer 20

C. Benign monoclonal gammopathy

Question 21

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 the options

Answer 21

D. All of the options

Question 22

Waldenstrom macroglobulinemia is a malignancy of the:
A. Lymphoplasmacytoid cells
B. Adrenal cortex
C. Myeloblastic cell lines
D. Erythroid cell precursors

Answer 22

A. Lymphoplasmacytoid cells

Question 23

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

Answer 23

C. Hairy cell leukemia (HCL)

A variable number of malignant cells in HCL will stain positive with tartrate-resistant acid phosphatase (TRAP+). Although this cytochemical reaction is fairly specific for HCL, TRAP activity has occasionally been reported in B-cell leukemia and, rarely, T-cell leukemia.

Acid phosphatase main diagnostic use is in the diagnosis of T-cell ALL and hairy cell leukaemia.

Question 24

The JAK2(V617F) mutation may be positive in all of the following chronic myeloproliferative disorders except:
A. ET
B. IMF
C. PV
D. CML

Answer 24

D. CML

Question 25

All of the following are major criteria for the 2008 WHO diagnostic criteria for ET except:
A. PLT count 450 x 10^9/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

Answer 25

D. Evidence of clonality