atypical CML


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  • Criteria

    • lacks Ph Chr, BCR-ABL1
    • leukocytosis (WBC >=13E9) due to neutrophils and their precursors with dysgranulopoiesis
    • neutrophil precursors (promyelocytes, myelocytes, metamyelocytes) >= 10% of leukocytes
    • minimal basophila (<2%) and monocytosis (<10%)
    • hypercellular marrow with granulocytic proliferation and dysplasia +/- dysplasia in other lineages
    • rule out PDGFRA/B, AML
      • lack PDGFRA, PDGFRB
      • blast < 20%
  • Morphology

    • blood
      • neutrophilia
      • increased neutrophil precursors, usually 10-20%
      • pronounced dysgranulopoiesis: Pelger Huet, clumped chromatin, abnormal granulation
      • minimal basophilia or monocytosis
      • blasts usually < 5%
    • marrow
      • hypercellular
      • dysgranulopoiesis
      • some megakaryocytic dysplasia
      • erythroid dysplasia in half
  • Molecular

    • most common: +8, del 20q
    • lacks BCR-ABL1