CMML


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  • Clinical

    • median age 70
    • monocytosis
    • can have organomegaly
    • 15-30% develop AML

  • Criteria

    • persistent monocytosis > 1E9/L
    • rule out CML, PDGFRA/B, AML
      • lacks Ph chr, BCR-ABL1
      • lack PDGFRA, PDGFRB
      • blast < 20%
    • has dysplasia or one of the following
      • cytogenetics
      • monocytosis over 3 months with no other cause

  • Types

    • CMML1: blood blasts < 5%, marrow blasts < 10%
    • CMML2: blood blasts 5-19%, marrow blasts 10-19%, or has Auer rods

  • Morphology

    • blood: monocytosis, abnormal monocytes, can have eosinophilia
    • marrow
      • usually hypercellular
      • monocytic proliferation
      • dysplasia: granulocytic (most), erythroid (half), megakaryocytic (80%)
      • blasts usually < 5%
      • can have increased reticulin, plasmacytoid dendritic cell nodules

  • Immunophenotype

    • myelomonocytic: CD33, CD13
    • plasmacytoid dendritic cells: CD123, CD43, CD33 (weak), CD4

  • Molecular

    • +8, del 7q, 12p
    • RAS mutation
    • JAK2 V617F is uncommon (vs atypical CML)
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