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ing promonocytes, and (ii) the white blood cell count in the circulation. The latter criterion, though, is artificial, since most CMML cases present with a white blood cell count of 8-14x10/L, often oscillating around the cut-off value of 13x10/L. All types of CMML show similarities with certain types of MDS. The paper by Valent et al. refers to a close relationship between MDS with multilineage dysplasia and dysplastic CMML-0, MDS with excess blasts-1 and dysplastic CMML-1, as well as MDS with excess blasts-2 and dysplastic CMML-2. In Figure 1, we try to illustrate the relationships within the large family of monocytoses. Valent et al. propose the term “oligomonocytic CMML” in order to emphasize, as also pointed out by others, that it may be appropriate to diagnose CMML based on bone marrow monocytosis and CMML-typical somatic mutations, even if marked monocytosis is missing in the peripheral blood. The group also points out that CMML can display two different types of acceleration and progression. On the one hand, dysplastic CMML can adopt typical features of MPN by showing accelerated proliferation with organomegaly and constitutional symptoms, without necessarily producing an excess of blasts. This evolution from a dysplastic to a proliferative type may be heralded by increasing white cell counts, often accompanied by splenic enlargement. On the other hand, progression of CMML in terms of increasing blast percentage, i.e. from CMML-0 to CMML-1 or CMML-2, often occurs without a marked increase in white cell count or development of organomegaly. To complicate matters, both types of evolution can occur simultaneously or sequentially, and can be caused (or at least accompanied) by clonal evolution in terms of new somatic mutations, increased variant allele frequencies, or acquisition of chromosomal aberrations. As disease evolution may be more prominent in the bone marrow or peripheral blood, the authors recommend classifying the disease according to the highest blast count detectable. Finally, the authors recommend complementing the group of CMML entities by including rare MPN-MDS variants, namely CMML with KITD816V systemic mastocytosis, and MDS-MPN with PDGFRA/B, FGFR1, or PCM-JAK2 rearrangements, because these rare entities are often associated with pronounced monocytosis. The paper by Valent et al. provides a comprehensive description of all the diagnostic tools needed to assign patients to the appropriate category within the heterogeneous group of non-clonal and clonal monocytoses. It summarizes our current knowledge and represents a starting point for future refinements of the classification of bone marrow disorders characterized by monocytosis.

p66Shc deficiency sets the scene for clinically aggressive chronic lymphocytic leukemia