1.7 Lymphoproliferative and Myeloproliferative Disorders Flashcards
Repeated phlebotomy in patients with PV may lead to the development of:
A. Folic acid deficiency
B. Sideroblastic anemia
C. IDA
D. Hemolytic anemia
C. IDA
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. Increased in number and functionally abnormal
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
D. Reed-Sternberg (RS) cells
In myelofibrosis, the characteristic abnormal RBC morphology is that of:
A. Target cells
B. Schistocytes
C. Teardrop-shaped cells
D. Ovalocytes
C. Teardrop-shaped cells
PV is characterized by:
A. Increased plasma volume
B. Pancytopenia
C. Decreased O2 saturation
D. Absolute increase in total RBC mass
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.
Features of secondary polycythemia include all of the following except:
A. Splenomegaly
B. Decreased O2 saturation
C. Increased RBC mass
D. Increased erythropoietin
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.
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
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
In PV, what is characteristically seen in peripheral blood?
A. Panmyelosis
B. Pancytosis
C. Pancytopenia
D. Panhyperplasia
B. Pancytosis
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
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.
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. CML: low LAP; leukemoid; high LAP
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
D. All of these options
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
B. The prognosis is better if Ph1 is present
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
D. An increase in basophils
In which of the following conditions does LAP show the least activity?
A. Leukemoid reactions
B. IMF
C. PV
D. CML
D. CML
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
C. Presence of granulocytes at different stages of development
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
D. Splenomegaly
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
D. Osteolytic lesions
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
C. Rouleaux
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
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.
Multiple myeloma is most difficult to distinguish from:
A. CLL
B. Acute myelogenous leukemia
C. Benign monoclonal gammopathy
D. Benign adenoma
C. Benign monoclonal gammopathy
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
D. All of the options
Waldenstrom macroglobulinemia is a malignancy of the:
A. Lymphoplasmacytoid cells
B. Adrenal cortex
C. Myeloblastic cell lines
D. Erythroid cell precursors
A. Lymphoplasmacytoid cells
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
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.
The JAK2(V617F) mutation may be positive in all of the following chronic myeloproliferative disorders except:
A. ET
B. IMF
C. PV
D. CML
D. CML
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
D. Evidence of clonality
