[Systemic mastocytosis].

Abstract

UNLABELLED: HEMATOPOIESIS DISORDER: Systemic mastocytosis is a primary proliferation of mast cells in several noncutaneous tissues. It remains an uncommon disease, difficult to diagnosis and often missed. The spectrum of clinical signs is wide and the course and prognosis are quite variable. CLINICAL ASPECTS: Sudden release of mediators by mast cell degranulation lead manifestations of paroxysmal congestion. Pigmentary urticaria is the most frequent cutaneous form. Among visceral localizations, bone involvement is encountered in 90% of the cases, digestive tract and hematological involvement are less frequent. Respiratory and cardiovascular involvement is exceptional. DIAGNOSIS: Osteomedullary biopsy is required for diagnosis. Urinary histamine metabolites may be suggestive. PROGNOSIS: Prognosis depends on the presence of an associated hematological disorder, found in 30% of the cases. TREATMENT: Symptomatic treatment is the rule, aimed at blocking histamine release by mast cell degranulation and avoid the subsequent paroxysmal manifestations.