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.
Asunto(s)
Anticuerpos Monoclonales/inmunología , Inmunoglobulinas/análisis , Linfoma/inmunología , Enfermedades Reumáticas/inmunología , Adulto , Biopsia , Médula Ósea/inmunología , Médula Ósea/patología , Citoplasma/inmunología , Femenino , Histocitoquímica , Humanos , Hiperplasia , Técnicas para Inmunoenzimas , Cadenas Pesadas de Inmunoglobulina/análisis , Cadenas Ligeras de Inmunoglobulina/análisis , Ganglios Linfáticos/inmunología , Ganglios Linfáticos/patología , Linfoma/complicaciones , Masculino , Persona de Mediana Edad , Células Plasmáticas/inmunología , Enfermedades Reumáticas/complicacionesAsunto(s)
Transfusión de Eritrocitos , Recambio Total de Sangre/métodos , Intercambio Plasmático , Porfirias/terapia , Enfermedad Aguda , Adulto , Envejecimiento Eritrocítico , Femenino , Humanos , Hepatopatías/complicaciones , Hepatopatías/terapia , Masculino , Persona de Mediana Edad , Fragilidad Osmótica , Porfirias/complicaciones , Porfirinas/sangre , Porfirinas/orina , Enfermedades de la Piel/complicaciones , Enfermedades de la Piel/terapiaRESUMEN
Forty-three non-HLA-matched donors provided 83 plateletpheresis products for 20 thrombocytopenic patients during a six-month period. The platelet product yield was 4.2 x 10(11) collected in 90 min. There was 26% cellular depletion of donor platelets and 20% depletion of donor lymphocyte per procedure. Males had a significantly greater lymphocyte depletion: 24% compared with 14% for females (P less than 0.05). There was no significant cellular depletion seen in 16 donors who underwent from two of a maximum of nine procedures. For these 16 donors, the time interval between procedures was a minimum of three days and a maximum of 100 days. Twelve refractory oncology patients received 49 plateletpheresis transfusions from 26 related donors. The mean corrected one-hour posttransfusion platelet increment was 18,300, and the mean corrected 20-hour posttransfusion platelet increment was 13,000. The results indicate that non-HLA-typed related plateletpheresis donors can safely undergo multiple procedures with the IBM 2997 Cell Separator and can effectively support their thrombocytopenic relatives who are unresponsive to random donor platelets.