Atypical chronic myelogenous leukemia (aCML), BCR/ABL1 negative is a rare disorder classified into the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN), according to the 2016 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia. It is, by definition, a BCR-ABL1-negative clonal disorder sharing myelodysplastic and myeloproliferative features.
Diagnostic Criteria of Atypical Chronic Myeloid Leukemia (aCML)
|Monocyte count||<10% of leukocytes|
|Multilineage dysplasia||Dyserythropoiesis and dysmegakaryopoiesis may be present|
|Immature granulocytes||≥ 10% leukocytes|
|Blasts||<20% (blood and bone marrow)|
|Bone marrow cellularity||Hypercellular bone marrow|
|Other molecular characteristics||No rearrangements of PDGFRA, PDGFRB, FGFR1, and PCM1-JAK2
Emphasis on molecular changes (ETNK1, SETBP1)
|Other factors||WHO criteria for other MPNs not met|
FAB, French-American-British; WHO, World Health Organization; Ph, Philadelphia; MPN, myeloproliferative neoplasm; MPN, myeloproliferative neoplasm.
- Diamantopoulos PT, Viniou NA. Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches. Front Oncol. 2021 Nov 17;11:722507. [Medline]
- Belkhair J, Raissi A, Elyahyaoui H, Ameur MA, Chakour M. Atypical chronic myeloid leukemia BCR-ABL 1 negative: A case report and literature review. Leuk Res Rep. 2019 May 25;12:100172. [Medline]
Created Jun 24, 2022.