The authors describe a rare case of systemic mastocytosis without skin infiltration. The first symptom were episodes of flushing in the face and upper trunk. From the case-history it was assumed that the condition had a several year's benign course, the diagnosis was established in the stage of malignant acceleration of the disease from bone marrow biopsy and liver biopsy. Atypical mastocytes displayed metachromasia with toluidine blue and positive naphthol-AS-D chloroacetate esterase. Symptomatic treatment with sodium cromoglycate reduced temporarily the frequency of flushing episodes, but it was not possible to arrest the increase of the mastocyte mass by cytostatic treatment.
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