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
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