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