Question 1

All of the following are included in the chronic myeloproliferative disorders except:&#10;A) Acute myeloid leukemia&#10;B) Chronic myelogenous leukemia&#10;C) Polycythemia vera&#10;D) Essential thrombocythemia

Accepted Answer

Acute myeloid leukemia is not included in the chronic myeloproliferative disorders as it is classified as a separate type of leukemia. The chronic myeloproliferative disorders include chronic myelogenous leukemia, polycythemia vera, essential thrombocythemia, and primary myelofibrosis.

Question 2

Which of the following would be an unexpected finding in chronic myelogenous leukemia? A) Increased eosinophils (EOs) and basophils in the peripheral blood B) 20% Blasts in the peripheral blood C) Hypercellular bone marrow with granulopoiesis D) White blood count of 100 *10⁹/L

Accepted Answer

In chronic myelogenous leukemia (CML), finding 20% blasts in the peripheral blood would be unexpected as it suggests a progression to a more acute phase of the disease, such as blast crisis, rather than the chronic phase which typically features less than 10% blasts.

Question 3

A patient has an increased red count, hemoglobin, and hematocrit. Which of the following features points to secondary polycythemia over polycythemia vera?&#10;A) Increased white count&#10;B) Increased red cell mass&#10;C) Bone marrow erythroid hyperplasia&#10;D) Decreased arterial oxygen saturation

Accepted Answer

One of the main causes of secondary polycythemia is chronic hypoxia, which stimulates the production of erythropoietin (EPO) and leads to an increase in red blood cell production. As a result, patients with secondary polycythemia often have decreased arterial oxygen saturation. In contrast, polycythemia vera (a primary myeloproliferative disorder) typically presents with an increased red cell mass and bone marrow erythroid hyperplasia, but not necessarily with decreased arterial oxygen saturation. Increased white count may or may not be present in either condition.

Question 4

A high red count, hemoglobin, and hematocrit are found in all of the following except:&#10;A) Polycythemia vera&#10;B) Bone marrow aplasia&#10;C) Dehydration&#10;D) Erythrocytosis secondary to hypoxia

Accepted Answer

Bone marrow aplasia (also known as aplastic anemia) is characterized by a decrease in all blood cell types due to a failure of the bone marrow to produce enough new cells. This would result in a low red count, hemoglobin, and hematocrit. The other options listed all involve an increase in red blood cells and would exhibit high red count, hemoglobin, and hematocrit readings.

Question 5

Which of the following is true for the mutated erythroid progenitors in polycythemia vera?&#10;A) They require extremely high levels of erythropoietin for growth in vitro.&#10;B) They are more resistant to apoptosis than normal erythroid progenitors.&#10;C) They mature abnormally, giving rise to a marked increase in poikilocytosis in peripheral blood.&#10;D) They are Philadelphia chromosome positive.

Accepted Answer

Mutated erythroid progenitors in polycythemia vera are more resistant to apoptosis than normal erythroid progenitors, which leads to the excessive production of red blood cells. A is incorrect because the mutated progenitors actually require less erythropoietin for growth. C is incorrect because poikilocytosis (abnormal shape of red blood cells) is not a characteristic feature of polycythemia vera. D is incorrect because the Philadelphia chromosome, a translocation between chromosomes 9 and 22, is associated with chronic myeloid leukemia, not polycythemia vera.

Question 6

Which of the following is true for chronic myelogenous leukemia?&#10;A) It is a clonal process arising from the pluripotent stem cell.&#10;B) It is most commonly found in children 5 to 10 years old.&#10;C) Common symptoms include an elevated red cell mass.&#10;D) Lymphadenopathy is common.

Accepted Answer

Chronic myelogenous leukemia is a clonal process arising from the pluripotent stem cell.

Question 7

Bone marrow or stem cell transplantation is the therapy of choice for patients younger than 55 years old with:&#10;A) Essential thrombocytosis&#10;B) Polycythemia vera&#10;C) Chronic myelogenous leukemia&#10;D) All of the above

Accepted Answer

Bone marrow or stem cell transplantation is the therapy of choice for younger patients with chronic myelogenous leukemia. It is not typically used for essential thrombocytosis or polycythemia vera unless the patient has progressed to acute leukemia or myelofibrosis.

Question 8

Chronic myelogenous leukemia often progresses to:&#10;A) Chronic neutrophilic leukemia&#10;B) Chronic lymphocytic leukemia&#10;C) Acute leukemia, either myeloid or lymphoblastic&#10;D) Chronic idiopathic myelofibrosis

Accepted Answer

Chronic myelogenous leukemia can transform into an acute leukemia, either myeloid or lymphoblastic, in a process called blast crisis. This is often the final and most aggressive phase of the disease, with a poor prognosis. The other options are not common progression paths for chronic myelogenous leukemia.

Question 9

A patient has a normal white blood count, a moderately high platelet count, and mild anemia. The differential shows immature granulocytes, nucleated red cells, and teardrop-shaped red cells. Which of the following is most likely?

A) Polycythemia vera
B) Chronic myelogenous leukemia
C) Primary myelofibrosis
D) Essential thrombocythemia

Accepted Answer

The combination of immature granulocytes, nucleated red cells, and teardrop-shaped red cells suggests a myeloproliferative disorder with bone marrow fibrosis, which is characteristic of primary myelofibrosis. Polycythemia vera would typically show elevated red cell counts, while chronic myelogenous leukemia would show elevated white cell counts with immature myeloid cells. Essential thrombocythemia would show primarily elevated platelet counts, without the presence of immature granulocytes or nucleated red cells.

Question 10

The Janus kinase (JAK) mutation is seen in more than 90% of cases of what disease?&#10;A) Chronic myelogenous leukemia&#10;B) Primary myelofibrosis&#10;C) Plasma cell myeloma&#10;D) Polycythemia vera

Accepted Answer

The Janus kinase 2 (JAK2) mutation is most commonly associated with polycythemia vera, where it is present in over 90% of cases.

Question 11

Which of the following is an expected finding in polycythemia vera?&#10;A) Thrombocytosis&#10;B) Anemia&#10;C) Low leukocyte alkaline phosphatase&#10;D) Normal white count

Accepted Answer

The answer of Which of the following is an expected...

Question 12

Which of the following would be an unexpected finding in primary myelofibrosis?&#10;A) 80% Blasts in the peripheral blood&#10;B) Micromegakaryocytes in the peripheral blood&#10;C) Autoantibodies&#10;D) Extramedullary hematopoiesis

Accepted Answer

The answer of Which of the following would be an...

Question 13

The bone marrow fibrosis observed in primary myelofibrosis is due to:&#10;A) Malignant proliferation of fibroblasts&#10;B) Increased erythropoietin, which stimulates fibroblast proliferation&#10;C) Increased release of fibroblastic growth factors such as platelet-derived growth factor&#10;D) Decreased apoptosis of normal fibroblasts

Accepted Answer

The answer of The bone marrow fibrosis observed in primary...

Question 14

What do all chronic myeloproliferative disorders share?&#10;A) Philadelphia chromosome&#10;B) Increased red cell mass&#10;C) Increased blood cells; overlapping clinical and laboratory features&#10;D) Serious thromboembolic complications

Accepted Answer

The answer of What do all chronic myeloproliferative disorders share?&#10;A)...

Question 15

Although found in most chronic myeloproliferative disorders, increased fibrosis detected by the silver techniques and trichrome stain is the key feature of:&#10;A) Secondary polycythemia&#10;B) Chronic myelogenous leukemia&#10;C) Essential thrombocythemia&#10;D) Primary myelofibrosis

Accepted Answer

The answer of Although found in most chronic myeloproliferative disorders,...

Question 16

Which of the following is the most specific laboratory test to diagnose chronic myelogenous leukemia?&#10;A) Increased bone marrow fibrosis and reticulin fibers&#10;B) Increased EOs and basophils in peripheral blood&#10;C) 5% Blasts in the bone marrow&#10;D) Leukocyte alkaline phosphatase

Accepted Answer

The answer of Which of the following is the most...

Question 17

The Philadelphia chromosome:&#10;A) Is a balanced reciprocal translocation between chromosomes 7 and 19&#10;B) Results in a chimeric gene (bcr/abl)&#10;C) Is diagnostic for polycythemia vera&#10;D) Manifests as a tyrosine kinase that blocks cell maturation

Accepted Answer

The answer of The Philadelphia chromosome:&#10;A) Is a balanced reciprocal...

Question 18

What clinical feature is more often associated with essential thrombocytosis than with the other chronic myeloproliferative disorders?&#10;A) Splenomegaly&#10;B) Bleeding and thrombosis&#10;C) Fatigue&#10;D) Infections

Accepted Answer

The answer of What clinical feature is more often associated...

Question 19

A patient has a platelet count of 1200 *10⁹/L. Many platelets are giant and have abnormal shapes. A mild anemia is present. The bone marrow has increased megakaryocytes in clusters; iron stores are present. Which of the following is most likely?

A) Essential thrombocythemia
B) Chronic myelogenous leukemia
C) Thrombocytosis secondary to blood loss
D) Polycythemia vera

Accepted Answer

The answer of A patient has a platelet count of...

Question 20

Which chronic myeloproliferative disorder is treated with Imatinib mesylate (Gleevec), a drug that inhibits tyrosine kinase?&#10;A) Polycythemia vera&#10;B) Chronic myelogenous leukemia&#10;C) Essential thrombocythemia&#10;D) Primary myelofibrosis

Accepted Answer

The answer of Which chronic myeloproliferative disorder is treated with...

Deck 34: Myeloproliferative Neoplasms