Wk 4 Myeloproliferative Disorders (MPD)

Question 1

What are 4 MPDs?

Answer 1

1. Chronic Myeloid Leukemia (CML)
2. Polycythemia Vera (PV)
3. Essential Thrombocytopenia (ET)
4. Myelofibrosis

Question 2

MPD general characteristics

Answer 2

1. High WBC w/ hypercellular BM
2. avg age 50-60
3. all myeloid lineage cells increased - classified based on cell type
4. risk for hyperuricemia and gout (due to degradation of purines as nuclear material -> uric acid)
5. Can progress to marrow fibrosis
6. can transform to acute leukemia

Question 3

CML

Answer 3

=chronic myeloid leukemia
-neoplastic proliferation of mature myeloid cells, esp granulocytes
-*basophils increased
-t(9;22): BCR-ABL fusion w/ increased tyrosine kinase activity (drive overproduction of cancer cells)

Tx: Imatinib, which blocks tyrosine kinase activity

Question 4

CML phases

Answer 4

1. chronic - enlarged spleen
2. accelerated - enlarging spleen indicative of worsening disease
3. transformation phase - develop acute leukemia (2/3 AML, 1/3 ALL)

Question 5

How to distinguish CML from leukemoid reaction

Answer 5

CML granulocytes have low LAP- (leukocyte alkaline phosphatase - would be positive and high due to infection in leukemoid reaction)
CML assoc w/ increased basophils, not a reaction
CML granulocytes exhibit t(9;22)

Question 6

Polycythemia Vera

Answer 6

=neoplastic proliferation of mature myeloid cells, esp RBCs
-granulocytes and platelets also increase
-assoc w/ JAK2 kinase mutation, which drive the disease

Question 7

PV symptoms

Answer 7

1. blurry vision and headache
2. increased risk of venous thrombosis, can -> blood chiari syndrome
3. flushed face due to congestion
4. itching after bathing due to extra mast cells

Question 8

PV Tx

Answer 8

1. phlebotomy
2. hydroxyurea

Question 9

CONT

Answer 9

WITH PATHOMA p58