Pathoma: Myeloproliferative Disorders

Question 1

What are some complications of MPNs?

Answer 1

Gout and hyperuricemia, because the high turnover of cells leads to much DNA sent into the plasma; transformation to acute leukemia; fibrotic bone marrow

Question 2

In CML, _________ cells are increased, especially ________.

Answer 2

granulocytic; basophils

Question 3

CML patients often have _________; progressive enlargement indicates _______.

Answer 3

enlarged spleens; transition to acute phase

Question 4

CML can transition to _________.

Answer 4

ALL or AML

Question 5

Leukocyte Alkaline Phosphatase (LAP) will be __________ in leukemoid reactions and ________ in malignancies.

Answer 5

positive; negative

Question 6

Basophilia is highly associated with _________.

Answer 6

CML

Question 7

JAK2 kinase mutations drive _______.

Answer 7

polycythemia vera, myelofibrosis, and essential thrombocythemia

Question 8

Budd-Chiari syndrome, in which ________, is often found in association with ________.

Answer 8

the hepatic vein is occluded by a thrombosis; polycythemia vera

Question 9

How can SaO2 and EPO levels help distinguish PV from reactive polycythemia?

Answer 9

High EPO and low SaO2 = hypoxia-induced polycythemia; high EPO and normal SaO2 = ectopic EPO production; normal EPO and normal SaO2 = PV

Question 10

Essential thrombocythemia does not have an increased risk of _________.

Answer 10

leukemia or gout

Question 11

In myelofibrosis, people get splenomegaly due to __________.

Answer 11

extramedullary hematopoiesis