Myeloproliferative Disorders Flashcards

1
Q

4 Myeloproliferative Disorders

A
  1. CML
  2. Polycythemia vera
  3. Essential Thrombocythemia
  4. Myelofibrosis
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2
Q

CML

A

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(-)

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3
Q

Polycythemia vera

A
mature myeloid cells, esp RBCs
increased granulocytes and platelets
Ax w/ JAK2 Kinase mutation
Hyperviscosity
Tx: blood letting; hydroxyurea --> hyperuricemia ; gout
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4
Q

Essential Thrombocythemia

A

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

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5
Q

Myelofibrosis

A

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

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