Background: Mast cell leukemia (MCL) is rare type of neoplasia with an incidence of 1% in a large series of 342\nadult patients with systemic mastocytosis (SM). Chronic basophilic leukemia (CBL) is an extremely rare type of\nleukemia with appearance of 7 cases in the literature.\nCase presentation: A 73 year-old female patient who presented with weaknes, had a prolonged duration of\nhematologic remission after treatment of her CBL by hydroxyurea (HU). Evolution of SM occurring as a second\nneoplasia concurrently with relapse of de novo CBL was demonstrated by mast cells (MCs) infiltration in the bone\nmarrow (BM) biopsy and smear and increase in tryptase level. Transformation to MCL with simultaneous occurrance\nof accelerated phase of CBL were documented by the appearance of MCs in both BM and peripheral blood (PB)\nsmears, antigen expressions detected by flow cytometry and spesific stains. Sequence analysis of c-kit gene revealed\nc-kit exon 11 K550N mutation. Undefined associations of MCL with different mast cell morphology, increase in IL-6\nlevel and accelerated phase of de novo CBL was described.\nConclusion: Elevations in CRP and IL-6 levels occurring with increases in basophil counts to high levels revealed\nthat febrile episodes with abdominal pain seen in our patient were induced by increase in IL-6 levels released from\nneoplastic basophils. Neoplastic basophils with diffuse and coarse basophilic granules possibly mimic neutrophils\nwith toxic granules and cause wrong characterization of neoplastic basophils as neutrophils by the automated\nblood cell counters and misleaded physicians.
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