Mast cell leukemia associated with undefined morphology and chronic basophilic leukemia

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

Mast Cells as Targets for the Therapy of Inflammatory Bowel Disease

The etiology and pathogenesis of inflammatory bowel disease (IBD) is poorly understood. However, numerous studies have demonstrated that immunological and inflammatory responses are activated during this disease. A better understanding of these events will help identify appropriate therapeutic interventions. Mast cell hyperplasia is a prominent feature of inflamed intestinal tissue in IBD. Intestinal mast cells are heterogeneous and at least two populations are present in the human intestine. The authors' objective is to explore mast cell properties, activation and mediators that are involved in the induction, maintenance and perpetuation of inflammatory lesions in the intestine. Although some therapies used in IBD can modulate mast cell activities, whether these actions are important in the beneficial effects of the drugs is unknown. Future drug development targeted to the inhibition of mast cells might be of therapeutic value. However, a cascade of different cellular events are involved in IBD development. The complexity of the disease raises difficulties in the development of therapies. Multiple drugs, selective for different phases of the disease or acting on different cells, might be most appropriate, rather than a single, all-encompassing therapeutic agent