Question 1

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF), the bone marrow displays panhyperplasia with a predominance of __________.

Accepted Answer

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF), the bone marrow shows panhyperplasia, which means there is an increased production of all types of blood cells. However, there is a predominance of megakaryocytes and granulocytes, making both A and B correct.

Question 2

Which myeloproliferative disorder is characterized by fibrosis of the marrow, extramedullary hematopoiesis of the spleen or liver, leukoerythroblastosis, and teardrop poikilocytosis?

Accepted Answer

The described features are consistent with the diagnosis of idiopathic myelofibrosis (also known as primary myelofibrosis). This disorder is characterized by fibrosis of the bone marrow, resulting in progressive loss of hematopoietic tissue and replacement by collagenous fibrous tissue. This leads to extramedullary hematopoiesis, often resulting in hepatosplenomegaly. The leukoerythroblastosis and teardrop poikilocytosis are also typical features of this disorder.

Question 3

Platelet dysfunction in myelofibrosis may eventually lead to disseminated intravascular coagulation (DIC) suggested by which coagulation abnormalities?

Accepted Answer

Prolonged thrombin time and elevated fibrin degradation products are both indicative of DIC, which can be caused by platelet dysfunction in myelofibrosis. Shortened prothrombin time is not a typical finding in DIC.

Question 4

According to the 2008 WHO criteria for diagnosis of Polycythemia Vera (PV), the hemoglobin value must be greater than what value for men?

Accepted Answer

According to the 2008 WHO criteria, the hemoglobin value for men must be greater than 18.5 g/dL for the diagnosis of Polycythemia Vera (PV).

Question 5

Metabolic consequences of myelofibrosis include all of the following except:

Accepted Answer

Hypotension is not typically included in the metabolic consequences of myelofibrosis. Night sweats, hyperuricemia, weight loss, and anorexia are all commonly associated with the condition.

Question 6

What is/are the strongest differentiating feature(s) that distinguish chronic myelocytic leukemia (CML) from IMF?

Accepted Answer

Chronic myelocytic leukemia (CML) is characterized by decreased leukocyte alkaline phosphatase (LAP) and the presence of the Philadelphia chromosome, which are key differentiating features from idiopathic myelofibrosis (IMF). Splenomegaly can be present in both conditions and is not a distinguishing feature.

Question 7

What is meant by myeloid metaplasia in idiopathic myelofibrosis?

Accepted Answer

Myeloid metaplasia in idiopathic myelofibrosis refers to the process where hematopoiesis (blood cell formation) occurs outside of the bone marrow, typically in the spleen and liver, due to the fibrosis (scarring) of the bone marrow. This is known as extramedullary hematopoiesis.

Question 8

Bone marrow aspiration in myelofibrosis frequently results in a __________.

Accepted Answer

Myelofibrosis is a disorder characterized by the replacement of bone marrow with fibrous tissue. This makes it difficult to obtain a sample of bone marrow through aspiration. Hence, bone marrow aspiration in myelofibrosis frequently results in a dry tap, meaning no hematopoietic cells are obtained in the aspirate.

Question 9

In the fibrotic (f-CIMF) phase of CIMF, which of the following is characteristic?

Accepted Answer

In the fibrotic (f-CIMF) phase of CIMF, all of the above characteristics are typically present. Fibrosis of the marrow can lead to ineffective hematopoiesis and bone pain. Extramedullary hematopoiesis can occur as the bone marrow is replaced by fibrous tissue. Leukoerythroblastosis of the peripheral blood refers to the presence of immature white blood cells and red blood cells in the bloodstream, which can be a sign of bone marrow stress. Splenomegaly is a common finding in CIMF, particularly in the fibrotic phase.

Question 10

In the absolute erythrocytosis, the red cell mass is __________.

Accepted Answer

In absolute erythrocytosis, the red cell mass is increased, which is the defining characteristic of this condition. Decreased or normal red cell mass would not be considered erythrocytosis.

Question 11

Which of the following characterizes the BM in the prefibrotic stage (p-CIMF) of CIMF according to the World Health Organization (WHO)?

Accepted Answer

The prefibrotic stage of Chronic Idiopathic Myelofibrosis (CIMF), according to the World Health Organization (WHO), is characterized by an increase in granulocytic and megakaryocytic proliferation, the presence of atypical megakaryocytes, and a lack of fibrosis. While there may be a decrease in erythroid precursors, this is not a defining feature of the prefibrotic stage. Therefore, all the options listed (A, B, C, D) collectively characterize the prefibrotic stage of CIMF.

Question 12

Which of the following statements best describes the etiology of the myeloproliferative disorders?

Accepted Answer

Myeloproliferative disorders are a group of clonal hematopoietic stem cell disorders that arise from a neoplastic transformation of a single multipotential stem cell. This leads to the overproduction of one or more blood cell lineages.

Question 13

Anemia in CIMF is a collective result of:

Accepted Answer

Anemia in CIMF is multifactorial and a result of bone marrow failure, ineffective erythropoiesis, and dyserythropoiesis. Bone marrow failure leads to a reduction in the total number of erythrocytes while ineffective and dyserythropoiesis lead to the production of abnormal and nonfunctional erythrocytes, further exacerbating the anemia. Therefore, all the options (A, B, and C) are correct, and the correct answer is D (All of the above).

Question 14

All of the following are included in the classic findings of chronic idiopathic myelofibrosis (CIMF) except:

Accepted Answer

Chronic idiopathic myelofibrosis (CIMF) is characterized by fibrosis of the bone marrow, which leads to extramedullary hematopoiesis, leukoerythroblastosis, and teardrop poikilocytosis in the peripheral blood. Erythroid hyperplasia is not a classic finding in CIMF; instead, the disease typically presents with ineffective erythropoiesis and anemia.

Question 15

A __________ anemia is found in most myelofibrosis patients at presentation:

Accepted Answer

Myelofibrosis often presents with a normocytic/normochromic anemia, which means the red blood cells are of normal size and color, indicating a typical concentration of hemoglobin within the cells. This type of anemia is associated with the bone marrow's inability to produce enough blood cells, rather than a deficiency of iron, vitamin B12, or folate, which would alter the size and color of red blood cells.

Question 16

According to the 2008 WHO criteria for diagnosis of Polycythemia Vera (PV), the hemoglobin value must be greater than what value for women?

Accepted Answer

The 2008 WHO criteria for diagnosis of Polycythemia Vera (PV) states that the hemoglobin value must be greater than 16.5 g/dL for women.

Question 17

A patient diagnosed with myelofibrosis secondary to gastrointestinal disease may develop a __________ anemia due to excessive blood loss.

Accepted Answer

Myelofibrosis is a condition where the bone marrow becomes fibrotic and loses the ability to produce sufficient blood cells. Secondary to gastrointestinal disease, the patient may experience excessive bleeding and develop microcytic/hypochromic anemia due to iron deficiency from the blood loss. Normocytic/normochromic anemia would not occur as the bone marrow is unable to produce enough blood cells. Macrocytic/hyperchromic anemia is commonly associated with vitamin B12 and folate deficiencies, which are not directly related to myelofibrosis secondary to gastrointestinal disease. Therefore, the answer is B.

Question 18

The presence of what gene mutation is one of the major citeria for diagnosis of PV?

Accepted Answer

The answer of The presence of what gene mutation is...

Question 19

A patient with myelofibrosis may develop early satisfaction for food intake due to:

Accepted Answer

Myelofibrosis is a condition where the bone marrow is replaced by fibrous tissue. As a result, the spleen becomes enlarged and may lead to early satiety due to compression of the stomach. Thrombocytopenia, anemia, and infiltration of fibrotic cells in the stomach may also occur in myelofibrosis, but they are not typically associated with early satiety. Hepatomegaly, or enlarged liver, is also possible in myelofibrosis but is not typically associated with early satiety.

Question 20

In relative erythrocytosis, the red cell mass is __________.

Accepted Answer

In relative erythrocytosis, the red cell mass is normal. This condition occurs when the volume of plasma decreases, making the concentration of red cells appear higher, even though their total mass hasn't changed.

Quiz 18: Chronic Myeloproliferative Disorders Ii: Polycythemia Vera, Essential Thrombocythemia, and Idiopathic Myelofibrosis

In the hypercellular (prefibrotic) phase of chronic idiopathic myelofibrosis (CIMF) , the bone marrow displays panhyperplasia with a predominance of __________.