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

Almost all patients with this CMPD are positive for the JAK2(V617F) mutation:

The myeloproliferative disorder typically associated with the highest platelet count [>1 million/L (1000  10⁹/L)] is:

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

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

What is the hallmark physical finding in CMPDs?

How is essential thrombocythemia differentiated from chronic myelocytic leukemia?

Which clinical manifestations in polycythemia vera are consistent with those of other myeloproliferative syndromes?

Metabolic consequences of myelofibrosis include all of the following except:

When essential thrombocythemia leads to iron deficiency anemia from associated bleeding, a __________ anemia will result.

In polycythemia vera, therapeutic phlebotomies reduce the total red cell mass by removing 350-500 mL over 2-3 days until the hematocrit is maintained at __________ percent.

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

Which is the expected platelet count in essential thrombocythemia?

Hemorrhage and thrombosis are physiologic complications of __________ due to excessive proliferation of the megakaryocyte lineage.

Tissue hypoxia and increased erythropoietic activity is an example of ___________ erythrocytosis.

What is the usefulness of myelosuppression as a treatment in polycythemia vera?

Which of the following are causes of reactive thrombocytosis?

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

In secondary erythrocytosis, the underlying mechanism is release of __________ as part of a compensatory effect to minimize impending tissue hypoxia.

In both absolute erythrocytosis and relative erythrocytosis the hematocrit is __________.

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