18. Chronic Myeloproliferative Disorders: II. Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis Flashcards
- What is the origin of MPD’s
a. Fibroid infiltration of major organs
b.Neoplastic transformation of multipotential stem cells
c.Widespread deterioration of cellular function
d. Splenic sequestration of normal blood cells
b.Neoplastic transformation of multipotential stem cells
2.Which of the following is not a characteristic of a chronic MPD?
a. Extramedullary hematopoiesis
b. Possible termination into acute leukemia
c. Cytogenetic abnormalities
d. Hypoplasia of bone marrow
d. Hypoplasia of bone marrow
3.The Philadelphia chromosome involves:
a.t(9;22)
b. The BCR and c-ABL genes
c. A 210-kD protein possessing increased tyrosine kinase activity
d.All of the above
d.All of the above
- What is the predominant abnormal erythrocyte morphol-ogy associated with idiopathic myelofibrosis?
a.Schistocytes
b.Ovalocytes
c.Teardrop cells
d.Target cells
c.Teardrop cells
5.Which features of chronic myelogenous leukemia are the most important characteristics that distinguish it from myelofibrosis?
a.Presence of increased platelets and fibroblasts
b.Decreased erythrocytes with abnormal morphology
c. Increased leukocytes with hypercellular bone marrow
d. Low LAP score and presence of Ph chromosome
d. Low LAP score and presence of Ph chromosome
- Which of the following factors does not cause fibroblast proliferation ?
a. CSF
b. TGF-B
c. bFGF
d. PDGF
a. CSF
7.What are thelaboratory findings in PV?
a. Decreased hematocrit; increased RBCs and granulo-cytes;decreased platelets
b.Increased hematocrit;increased RBCs,granulocytes,and platelets
c.Normal hematocrit; normal RBCs; increased granulo-cytes and platelets
d. Increased hematocrit; increased RBCs; decreased granulocytes and platelets
b.Increased hematocrit;increased RBCs,granulocytes,and platelets
8.What is the expected erythropoietin value in PV?
a.Normal
b.Increased
c.Deneesed
c.Deneesed
- When is myelosuppression advocated in patients with PV ?
a. If the patient is less than 20 years old
b. When thrombosis-associated risk factors are present
c. If the patient shows signs of glossitis
d. If the patient has failed iron therapy
b. When thrombosis-associated risk factors are present
10.Which features help to distinguish secondary erythrocytosis and relative erythrocytosis from PV ?
a. absence of splenomegaly; normal leukocyte, platelet,and LAP levels
b.Presence of splenomegaly;increased leukocyte,platelet,and LAP levels
c.Presence of hepatomegaly; decreased leukocyte,platelet,and LAP levels
d. Presence of both splenomegaly and hepatomegaly; increased leukocytes and platelets; decreased LAPscore
a. absence of splenomegaly; normal leukocyte, platelet,and LAP levels
11.What is the safest and least expensive treatment for patients with polycythemia vera?
a. High altitude
b.Decrease of iron levels
c.Therapeutic phlebotomy
d.Decrease of erythropoietin levels
c.Therapeutic phlebotomy
- What condition is defined by a platelet count greater than 600 X 109/L, megakaryocytic hyperplasia, absence of Ph chromosome, and hemoglobin of 13 g/dL or more (or normal red cell mass)?
a. Essential thrombocythemia
b.May-Hegglin anomaly
c. Acute myelogenous leukemia
d.Polycythemia vera
a. Essential thrombocythemia
- The thrombosis seen in patients with essential thrombo-cythemia is a result of which of the following?
a.Protein C deficiency
b. Marked fibroblast proliferation
c. Intravascular clumping of sludged hyperaggregable platelets
d.Splenic sequestration of platelets
c. Intravascular clumping of sludged hyperaggregable platelets
- What condition is not characteristically associated with reactive thrombocytosis?
a.Acute hemorrhage
b.Aplastic anemia
c. Chronic inflammatory disorders
d.Iron-deficiency anemia
b.Aplastic anemia
- Which of the followingchronic MPDs is associated with the best prognosis?
a.CML
b.IMF
c.PV
d.ET
a.CML
