Myeloproliferative Disorders (MPD)

Question 1

Myeloproliferative disorders are a neoplastic proliferation of what?

Answer 1

Mature cells of myeloid lineage

Question 2

What age group more commonly gets myeloproliferative disorders?

Answer 2

50-60 yos

Question 3

What are some general complications of myeloproliferative disorders

Answer 3

Increased risk for hyperuricemia and gout (high turnover of cells)
Progression to marrow fibrosis
Transformation to acute leukemia

Question 4

Name the myeloproliferaive disorders

Answer 4

Chronic myeloid leukemia
Polycythemia vera
Essential thrombocythemia
Myelofibrosis

Question 5

what cells proliferate in chronic myeloid leukemia?

Answer 5

mature myeloid cells, especially granulocytes and their precursors. commonly basophils.

Question 6

Chromosome abnormality commonly seen in chronic myeloid leukemia

Answer 6

Philadelphia chromosome 9;22 which generates BCR-Abl- tyrosine kinase

Question 7

First line treatment of chronic myeloid leukemia

Answer 7

Imatinib - blocks tyrosine kinase activity

Question 8

Complications of chronic myeloid leukemia

Answer 8

Transformation to AML (2/3) or ALL (1/3)

Question 9

How to distinguish chronic myeloid leukemia from leukemoid reaction?

Answer 9

CML will stain negative for leukocyte alkaline phosphatase
Increased basophils in CML
t(9:22) will be absent in leukemoid reaction

Question 10

What is polycythemia vera a proliferation of?

Answer 10

neoplastic proliferation of mature myeloid cells, especially RBCs

Question 11

What mutation is polycthemia vera associated with?

Answer 11

JAK2 kinase

Question 12

Clinical symptoms of polycythemia vera?

Answer 12

blurry vision and headache
Increased risk of venous thrombosis (hepatic vein, portal vein, dural sinus)
Flushed face due to congestion
Itching after bathing (histamine release)

Question 13

Treatment of polycythemia vera

Answer 13

phlebotomy

second line: hydroxyurea

Question 14

How to distinguish polycythemia vera from reactive polycythemia?

Answer 14

polycythemia vera has decreased EPO levels and normal SaO2
Reactive polycythemia has low SaO2 and increased EPO
If reactive polycythemia is due to ectopic EPO production (from renal cell carcinoma) EPO is high and SaO2 is normal

Question 15

What is essential thrombocythemia a proliferation of?

Answer 15

neoplastic proliferation of mature myeloid cells especially platelets

Question 16

What mutation is essential thrombocythemia associated with?

Answer 16

JAK2 kinase mutation

Question 17

Symptoms of essential thrombocythemia ?

Answer 17

Increased risk of bleeding (if platelets are non-functional)

Increased risk of thrombosis (if platelets are functional)

Question 18

What is myelofibrosis a proliferation of?

Answer 18

mature myeloid cells especially megakaryocytes

Question 19

What mutation is myelofibrosis associated with?

Answer 19

JAK2 kinase (50% of cases)

Question 20

clinical features of myelofibrosis?

Answer 20

Marrow fibrosis from megakaryocyte production of excess PDGF
Splenomegaly from intramedullary hematopoiesis
leukoerythroblastic smear (tear drop cells, nucleated RBCs and immature granulocytes)
Increased risk of infection, thrombosis, and bleeding

Question 21

What will be seen on a bloor smear of myelofibrosis?

Answer 21

tear drop cells