Assuntos
Leucemia Neutrofílica Crônica , Aberrações Cromossômicas , Células Clonais/patologia , Progressão da Doença , Humanos , Leucemia Neutrofílica Crônica/genética , Leucemia Neutrofílica Crônica/patologia , Mieloma Múltiplo/patologia , Síndromes Mielodisplásicas/patologia , Neoplasias Primárias Múltiplas/patologia , Células-Tronco Neoplásicas/patologia , Neutrófilos/fisiologia , Paraproteinemias/patologiaAssuntos
Síndrome da Imunodeficiência Adquirida/complicações , Linfoma de Burkitt/etiologia , Neoplasias Hepáticas/etiologia , Adulto , Linfoma de Burkitt/patologia , Humanos , Fígado/patologia , Neoplasias Hepáticas/patologia , Masculino , Prognóstico , Transtornos Relacionados ao Uso de Substâncias/complicaçõesRESUMO
A 70-yr-old man with multiple myeloma IgD developed a plasma cell leukemia producing a serum IgD monoclonal peak and lambda light chains in the urine. When the serum and the urine were heated at 56 degrees C for 30 min both monoclonal bands disappeared. The precipitate failed to redissolve on heating to 100 degrees C. Ion exchange chromatography with a linear gradient of phosphate buffer, pH 8, 0.020-0.300 mol/l and column electrofocusing showed that the serum pyroglobulin was eluted with buffer concentration between 0.040-0.125 mol/l and had an isoelectric point of 5.02, while the pyroglobulin of the urine was eluted with 0.020-0.033 mol/l and had a pI = 7.16. The serum and urine pyroglobulins had a total antigenic community with their correspondent purified proteins. The isolated lambda chains did not change when they were heated at 56 degrees C for 30 min, nevertheless, the heated purified IgD shows some changes in its isoelectric point, molecular mass and antigenicity. These changes in the purified IgD suggest that the pyroprecipitability could be due to conformational features.
Assuntos
Imunoglobulina D , Leucemia Plasmocitária/metabolismo , Paraproteínas/metabolismo , Piroglobulinas/metabolismo , Idoso , Fenômenos Químicos , Físico-Química , Eletroforese , Temperatura Alta , Humanos , Imunoensaio , Cadeias lambda de Imunoglobulina , Focalização Isoelétrica , Leucemia Plasmocitária/etiologia , Leucemia Plasmocitária/imunologia , Masculino , Peso Molecular , Mieloma Múltiplo/complicações , Piroglobulinas/urinaRESUMO
Six cases of constitutional aplastic anemia (Fanconi's anemia), including four boys and two girls who were between 6 and 10 years of age, are presented. Congenital abnormalities were observed in all the patients, especially skin pigmentations, renal anomalies, and growth retardation. The initial symptoms were insidious, mainly related to the anemia. All patients were anemic and thrombocytopenic, but only three were leukopenic. Five cases showed increased levels of fetal hemoglobin, which was marked in two. Bone marrow obtained by aspiration and/or biopsy was hypocellular in all cases. Chromosomal studies showed breaks, ring- or cross-chromosomes, chromosome exchanges, and endoreduplications. Five cases were treated with androgens and a good response was obtained in all of them. Four patients died, and the remaining two are alive and well. The clinical and biological features of these patients are in accordance with those of previous reports. The importance of the androgen therapy is stressed; androgen dependence was observed in all cases.
