Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell malignancy with features of both a myeloproliferative neoplasm and a myelodysplastic syndrome. We present a case of 72-year-old man with CMML who presented with generalized hemorrhagic papules and plaques which on histopathology showed a peculiar infiltrate of atypical mature histiocytes. The immunohistochemical markers for Langerhans cells, indeterminate dendritic cells, and plasmacytoid dendritic cells were negative. Next generation sequencing performed on the paraffin block of the leg biopsy specimen revealed identical ASXL1, SRSF2, and KRAS mutations as seen in the CMML clone of the peripheral blood. Along with recent literature, this case illustrates the spectrum of histiocytic and dendritic cell proliferations in CMML, many of which may be clonally related to the hematopoietic malignancy.
Keywords: chronic myelomonocytic leukemia; histiocytes; next generation sequencing; skin.
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