Systemic mastocytosis – a clinical case report

• Autor(a) principal: Melo, Marco
• Data de Publicação: 2003
• Outros Autores: Deus, Adelaide, Aragão, António, Magalhães, Eduardo, Santos, Fernando, Saldanha, Maria Helena
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://revista.spmi.pt/index.php/rpmi/article/view/1793
• topic: anemia; trombocitopenia; mastocitose sistémica; Anaemia; Thrombocytopenia; systemic mastocytosis

The A.A. describe a clinical case concerning an adult male, 51 years old, suffering from easytiredness, dry cough, nocturnal fever along with oedema and pigmented lesions on both legs, of 15 days duration. In his past history he had suffered an episode of histaminic shock in 1992.Clinical findings: slightly raised body temperature, paleness of the skin and mucosae, bilateral axillary and inguinal adenopathic conglomerates coexisting with painless and homogeneous hepatic and splenic enlargement. Bothlegs were covered with brown macular diffuse lesions, compatible with pigmented urticaria.Laboratory findings revealed the presence of macrocytic anaemia thrombocytopenia; hypergammaglobulinemia IgG; negative tumour markers; thoracic and abdominal computerized axial tomography showed “(...) slight bilateralpleural effusion, homogeneous hepatic andsplenic enlargement with the presence of enlarged lumbar and aortic lymph nodes”. Bone marrow biopsy result revealed “ a medullary invasion by fibroblastic proliferation nodes associated with a lymphocytic and mast cells infiltration, allowing the diagnosis of Systemic mastocytosis “. Cutaneous and hepatic biopsies also showed the involvement of these organs in the pathologic process.The patient was treated with methylprednisolone and cyclosporine A with remarkable reduction of symptoms.