RESUMEN
We have described a patient with simultaneous onset of antibody-positive immune thrombocytopenic purpura and ulcerative colitis, an association that must be extremely rare. Although the colitis responded to steroid therapy, the thrombocytopenia required splenectomy. The antibody was monomeric, with HLA specificity against A3 and B8 platelets.
Asunto(s)
Colitis Ulcerosa/complicaciones , Púrpura Trombocitopénica/inmunología , Anciano , Anticuerpos/análisis , Plaquetas/inmunología , Humanos , Masculino , Púrpura Trombocitopénica/complicaciones , Púrpura Trombocitopénica/cirugía , EsplenectomíaRESUMEN
Three patients with erythropoietic protoporphyria were treated with combined plasmapheresis and neocyte exchange transfusions that induced and maintained clinical and biochemical remissions. Red cell and plasma porphyrin levels normalized and patients developed a more normal tolerance to ultraviolet exposure. This treatment modality was an effective method of reversing other newly described abnormalities such as excessive erythropoiesis, abnormal RBC osmotic fragilities, elevated plasma viscosities, and hepatic and renal impairments.
Asunto(s)
Transfusión Sanguínea , Transfusión de Eritrocitos , Eritropoyesis , Plasmaféresis , Porfirias/terapia , Porfirinas/sangre , Protoporfirinas/sangre , Adulto , Viscosidad Sanguínea , Eritrocitos/análisis , Femenino , Humanos , Masculino , Fragilidad Osmótica , Rayos UltravioletaRESUMEN
Lymphocytes were obtained from two patients with paroxysmal nocturnal hemoglobinuria as well as from apparently healthy controls and from patients with acute lymphoblastic leukemia and chronic lymphocytic leukemia. Subsequently, several aspects of zinc metabolism were studied in vitro in short-term cultures of these lymphocytes in order to assess lymphocyte functional capacity. The results of mitogen stimulation and zinc uptake studies for lymphocytes from donors with paroxysmal nocturnal hemoglobinuria were similar to those obtained for leukemic lymphocytes. The results of studies to determine the inducibility of the low molecular weight zinc-binding protein, metallothionein, by zinc were complicated by the decrease in overall protein synthesis in lymphocytes from donors in the paroxysmal nocturnal hemoglobinuria. It is proposed that paroxysmal nocturnal hemoglobinuria is indeed a clonal disorder and the relationship between lymphocytes in this disorder and leukemic lymphocytes is discussed.
RESUMEN
Tissue specimens from five patients with rheumatic disease who developed lymphadenopathy were studied by an immunoperoxidase method; available biopsy material was examined for cytoplasmic immunoglobulin determinants. Three patients had follicular hyperplasia of lymph nodes with polyclonal patterns. Two patients with Sjogren's syndrome had monoclonal patterns; both of these patients subsequently developed lymphoma with similar monoclonal patterns. Implications of the monoclonal patterns with regard to the biology of these lymphoproliferative disorders are discussed.