Chronic Myelomonocytic Leukemia (CMML) Variants

Chronic Myelomonocytic Leukemia (CMML) has a number of variants that need to be considered when making the diagnosis.

Patient selection: meets criteria for CMML

Variant	Criteria
oligomonocytic	absolute monocyte count < 1 * 10^9 in peripheral blood
systemic mastocytosis with CMML (SM-CMML)	fulfills WHO criteria for systemic mastocytosis
CMML with myeloid neoplasm expressing classic MPN driver	fulfills WHO criteria for MPN (CML, PMF, etc) AND has molecular driver
CMML with classic MPN driver	does not fulfill criteria for MPN but has molecular drivers
CMML with concomitant lymphoid neiplasma	fulfills WHO criteria for lymphoid neoplasm

where:

• Most patients with SM-CMML express KIT D816V.

• Classic MPN drivers: JAK2 V617F, BCR-ANL1, rearranged PDGFRA/B or FGDR1.

• The presence of CMML and MPN is an MDS/MPN overlap syndrome.

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