Response of urticaria pigmentosa to cladribine in a patient with systemic mastocytosis

Cladribine therapy for systemic mastocytosis.

Patients with systemic mastocytosis (SM) can suffer from disabling symptoms related to mast cell mediator release or mast cell infiltration, requiring mast cell eradication. In the present absence of any curative therapy, a recent case report describing the efficacy of cladribine showed promising results. In a pilot study, the efficacy of cladribine (0.10-0.13 mg/kg in a 2-hour infusion, days 1...

Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease.

OBJECTIVE To determine clinical correlates of urticaria pigmentosa (UP) regression in adult patients with systemic mastocytosis (SM). DESIGN Cohort study of the natural history of mastocytosis. SETTING National Institutes of Health Clinical Center. PATIENTS In a study of adult patients referred to the National Institutes of Health after 1980 and observed for a minimum of 10 years, 12 of 1...

Mastocytosis (urticaria pigmentosa) of skin, stomach, and gut with malabsorption.

Combined Mastocytoma-hemangioma in a Patient with Urticaria Pigmentosa.

A patient with long-standing urticaria pigmentosa presented with a pea-sized reddish to purplish papule on the posterior part of the right ear. Histopathologic examination revealed numerous dilated vascular structures in the upper dermis and mast cell infiltrations throughout the whole dermis, consistent with combined mastocytoma-hemangioma. The mast cells were strongly positive with Giemsa stain.

Indolent systemic mastocytosis in a patient with ileocolitis

Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor cell. It is a rare disorder with unknown incidence in Greece, with an estimate of 2 cases per year in Great Britain. We present a case of an asymptomatic, 72-year-old man who was found to have ileocolitis on endoscopy. Histology revealed mast cells in lamina propria >15 HPF and biochemistry showed high levels of seru...