[Systemic mastocytosis. Study of 14 cases]. / Mastocitosis sistémica. Estudio de 14 casos.

We have analyzed the clinical, analytical and evolutive data of 14 cases of systemic mastocytosis (SM) diagnosed in our hospital between 1991-1996 and we have compared these results with those of other published series. Clinical parameters, analytical profiles, peripheral hematologic data, radiologic data and histological study were collected. Following Metcalfe's criteria, the patients were classified in 4 groups: a) group 1, indolent mastocytosis; b) group 2, hematologic disorders; c) group 3, aggressive lymphadenopathic mastocytosis with eosinophilia, and d) 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-eritematous skin lesions (in 11 cases), and digestive symptoms (in 10 patients). The most usual biochemical disorder was the rise of serum alkaline phosphatase level (in 8 patients), while lactate-dehydrogenase (LDH) was normal in all the cases. The most striking roentgenologic features were oteopenia, observed in 50% of our patients. Pheripherical hematological disorders were discovered in 8 patients (64.3%) and in one of them circulating mast-cells were observed. The bone marrow was involved in all patients (100%) and in two of them mielodysplasic features were found. The diagnostic of SM is difficult in the absence of skin lesions. The skin lesions are very common in systemic mastocytosis. Bone marrow involvement is constant, so its study has a high diagnostic rentability.

[Primary T-cell lymphoma in the central nervous system of immunocompetent patients. Clinical and pathological study of two cases]. / Linfoma primario de células T del sistema nervioso central en pacientes inmunocompetentes. Estudio clinicopatológico de dos casos.

Primary T-cell lymphoma in the central nervous system has rarely been described in the literature. We report 2 such cases in immunocompetent patients. The first presented with subacute encephalopathy affecting both memory and speech. A CT-scan of the head showed a contrast-enhanced mass around the third ventricle. The second presented with 2 partial seizures with secondary generalization; the CT-scan in this case showed a right temporal lesion. Both underwent surgery for total removal of the masses followed by cranial irradiation. Tissue examination confirmed the diagnosis. The first patient died 14 months after diagnosis. In the second case lymphoma recurred 8 months after surgery.