Myeloproliferative Disorders

Question 1

Myeloproliferative Disorders in General

Answer 1

Hyperuricemia / Gout
Marrow Fibrosis
Transform to Acute Leukemia
Cells of all Myeloid Lineages are raised

Question 2

Chronic Myeloid Leukemia

Answer 2

mature myeloid cells, mainly granulocytes
Basophils are elevated
9;22 translocation leads to BCR-ABL fusion with increased Tyrosine Kinase activity
Imatinib blocks tyrosine kinase activity
Splenomegaly usually preceded Acute Leukemia - AML or ALL

Question 3

CML distinguished from Leukemoid Reaction

Answer 3

CML Granulocytes are LAP Negative
(Leukocyte Alkaline Phosphotase)
CML is associated with increased Basophils
CML granulocytes exhibit 9;22 translocation

Question 4

Polycythemia Vera

Answer 4

Mature Myeloid Cells - RBCs
JAK2 Kinase Mutation
Hyper-viscosity (blurry vision, headache, venous thrombosis, Flushed Face, Itching)
Budd-chiari Syndrome
Itching after Bathing (mast cells)
Phlebotomy or Hydroxyurea

Question 5

Essential Thrombocythemia

Answer 5

Mature Myeloid Cells - Platelets
JAK2 Kinase Mutation
Risk of Bleeding or Thrombosis
Usually not marrow fibrosis or acute leukemia
No significant risk of gout