Mast cell leukemia morphologic illustration of a rare entity

eJHaem

article

Chauncey R. Syposs¹  Andrew G. Evans¹  John M. Bennett¹  Jane L. Liesveld²  Siba El Hussein¹ 

[1] Department of Pathology, University of Rochester Medical Center;Department of Medicine and The James P. Wilmot Cancer Center, University of Rochester Medical Center

关键词: acute basophilic leukemia;    leukemia;    mast cell leukemia;    myelomastocytic leukemia;    systemic mastocytosis;   

DOI  :  10.1002/jha2.571

来源: Wiley

【 摘 要 】

A 72-year-old woman with no oncologic past medical history presentedto the emergency department with several weeks of progressivefatigue, dyspnea, night sweats, intermittent rash, and a 30 lb unintended weight loss. Initial imaging demonstrated splenomegaly (upto 22.6 cm) and attenuated bone marrow lesions, suspicious for bonemarrow involvement by an occult malignancy. Her complete bloodcount was significant for mild anemia (hemoglobin 10.0 g/dl), thrombocytopenia (platelets 103 × 109/L), with a normal white blood cell countof 10.0 × 109/L. Serum tryptase and histamine levels were markedlyelevated, >400 ug/L (normal range ≤ 10.9 ug/L) and 51,930 nmol/L(normal range 180–1800 nmol/L), respectively. Peripheral blood smearreview demonstrated 20% immature cells, medium-to-large in size,with abundant cytoplasm, prominent metachromatic granules, roundnuclei and immature chromatin (Figure 1A). Flow cytometry analysisof the blood revealed an aberrant mast cell population expressingCD2 (40%), CD13, CD25, CD33, and CD117; while negative for theexpression of CD34, HLA-DR, cytoplasmic MPO, and other lymphoid and monocytic markers. A bone marrow biopsy demonstratedextensive involvement by leukemic mast cells (Figure 1B–D), withcharacteristically round rather than spindle-shaped nuclear contours,abundant cytoplasm, some granulated and vacuolated, and immaturechromatin, with frequent mitotic figures (Figure 1D).

【 授权许可】

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