SYSTEMIC MASTOCYTOSIS - STUDY OF 14 CASES

We have analyzed the clinical, analytical and evolutive data of 14 cas es of systemic mastocytosis (SM) diagnosed in our hospital between 199 1-1996 and we have compared these results with those of other publishe d series. Clinical parameters, analytical profiles, peripheral hematol ogic data, radiologic data and histological study were collected. Foll owing Metcalfe's criteria, the patients were clasified in 4 groups: a) group 1, indolent mastocytosis; b) group 2, hematologic disorders; c) group 3, aggressive lymphadenopathic mastocytosis with eosinophilia, and dl group 4, mastocytic leukemia. The average age at diagnosis was 52.4 years, range 25-83, and 64% were females. The mean follow-up was 2 yr. In most of the cases (71.4%) the initial complaint was urticaria pigmentosa. The predominant clinical features were pruriginous-eritem atous skin lesions (in 11 cases), and digestive symptoms (in 10 patien ts). The most usual biochemical disorder was the rise of serum alkalin e phosphatase level (in 8 patients), while lactate-dehydrogenase (LDH) was normal in all the cases. The most striking roentgenologic feature s were oteopenia, observed in 50% of our patients. Pheripherical hemat ological disorders were discovered in 8 patients (64.3%) and in one of them circulating mast-cells were observed. The bone marrow was involv ed in all patients (100%) and in two of them mielodysplasic features w ere found. The diagnostic of SM is difficult in the absence of skin le sions. The skin lesions are very common in systemic mastocytosis. Bone marrow involvement is constant, so its study has a high diagnostic re ntability.