Myeloproliferative Disorders Flashcards
4 Myeloproliferative Disorders
- CML
- Polycythemia vera
- Essential Thrombocythemia
- Myelofibrosis
CML
chronic myeloid leukemia
t(9;22) –> BCR-ABL, Ph chromosome
increase tyrosine kinase activity –> overgrowth
splenomegaly –> expanded red pulp
Transformation to acute leukemia –> AML or ALL
LAP(-)
Polycythemia vera
mature myeloid cells, esp RBCs increased granulocytes and platelets Ax w/ JAK2 Kinase mutation Hyperviscosity Tx: blood letting; hydroxyurea --> hyperuricemia ; gout
Essential Thrombocythemia
Neoplastic prolif of mature myeloid cells, esp platelets
Increased RBC and granulocytes
Ax w/ JAK2 kinase mutation + MPL tyrosine kinase
Dx of exclusion
thromboses erythromelalgia
no risk for gout
Myelofibrosis
extensive collagen deposition by non-neoplastic fibrosis
esp megakaryocytes
Ax w/ JAK2 kinase mutation + MPL
produce excess PDGF –> marrow fibrosis, myelofibrosis
splenomegaly d/t extramedullary hematopoiesis
bone marrow contains–> reticulin gates
Leukoerythroblastic smear: cells can’t leave spleen, see nucleated RBCs
Tear drop cells