6.4 Myeloproliferative Disorders Flashcards
Myeloproliferative Disorder
Neoplastic proliferation of mature cells of myeloid lineage - disease will be named based on the predominant cell type
What is the typical patient age of Myeloproliferative Disorders?
Late adulthood
What are the common complications of Myeloproliferative Disorder?
- Hyperuricemia and gout
- Progression to marrow fibrosis
- Transformation to acute leukemia
Chronic Myeloid Leukemia
Proliferation of mature myeloid cells - especially granulocytes like basophils
Microscopic findings of CML
Increased granulocytes and precursor granulocytes
Translocation of CML
t(9;22) BCR-ABL fusion
What is the treatment of CML?
Imatinib
What is a common sign of CML?
Splenomegaly - enlarging spleen may indicate acceleration
How can CML be distinguished from a leukemoid reaction?
- CML granulocytes are LAP negative
- CML associated with increased basophils
- CML granulocytes will exhibit t(9;22) BCR-ABL
Polycythemia Vera
Proliferation of myeloid cells - mostly RBCs
What is the mutation that drives Polycythemia Vera?
JAK2
What are the complications of Polycythemia Vera?
Most are complications of hyper viscosity of the blood
- Blurry vision and headache
- Venous thrombosis in the liver
- Flushed face
What is the treatment for Polycythemia Vera?
Phlebotomy with hydroxyurea as a second line treatment
What are the EPO levels in PV?
EPO levels will be low
Essential Thrombocytopenia
Proliferation of myeloid cells - mainly platelets
