Pathology
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Atlas of Bone Marrow pathology
Mojmir Moulis, Josef Feit et al.
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Atypical Chronic Myeloid Leukemia (aCML)
Introduction

Leukemia with both myelodysplastic and myeloproliferative features with predominant affection of the granulocytic lineage, BCR-ABL1 negative.

Clinical signs

BCR-ABL1 gene and PDGFRA and PDGFRB transformation are absent, mutation JAK2 V617F is sometimes present, 30% of cases show NRAS or KRAS mutation.

Clinical signs
  • leukocytosis more than 13×109/l, splenomegaly, BCR/ABL1 negativity, less than 20% blasts in peripheral blood or bone marrow, dysplastic changes in all three hematopoietic lineages, M:E ratio is usually higher than 10:1
  • prognosis: bad, median survival time is 14 – 29 months, 15 – 40%
    • of patients develop AML
  • symptomes resulting from anemia, sometimes thrombocytopenia, splenomegaly