RESUMO
A case of alpha-chain disease (alpha-CD) is described presenting as a malabsorption syndrome complicated by severe kalipenic nephropathy. Antibiotics and cyclophosphamide were effective in controlling the malabsorption and thr renal dysfunction but did not affect the basic pathological lesion. Eventually the disease became generalized, affected lymph nodes, liver and bone marrow and caused multiple intestinal perforations. Detailed histological studies failed to demonstrate the development of reticulum-cell sarcoma as a terminal complication of the disease and are suggestive of a single malignant plasmacytic cell process.
Assuntos
Doença das Cadeias Pesadas/complicações , Hipopotassemia/etiologia , Cadeias Pesadas de Imunoglobulinas , Cadeias alfa de Imunoglobulina , Perfuração Intestinal/etiologia , Nefropatias/etiologia , Adulto , Antibacterianos/uso terapêutico , Humanos , Hipopotassemia/imunologia , Hipopotassemia/patologia , Perfuração Intestinal/imunologia , Perfuração Intestinal/patologia , Jejuno/imunologia , Jejuno/patologia , Nefropatias/imunologia , Nefropatias/patologia , MasculinoRESUMO
Clinical, immunological, and histological recovery in a patient with alpha-chain disease is described. The patient, a 27-year-old Greek man, presented with severe steatorrhoea, abdominal pain, oedema, and hypogammaglobulinaemia. Treatment with tetracycline produced only temporary remission. Intermittent therapy with prednisone and cyclophosphamide together with antibiotics was followed by clinical recovery, return of histological appearances of the small intestine to normal, and disappearance of free alpha-chain protein from the serum. The patient remained well one year later without treatment.
