RESUMO
We report a case of primary extramedullary plasmacytoma (PEMP), IgA kappa type, with a long clinical history, in a 64-year-old Japanese woman. In this case, PEMP occurred primarily in the jejunum and then recurred in several organs, specifically the spleen and stomach, and in the subcutaneous tissue, over a period of about 16 years. However, examination of bone marrow aspirates on several occasions continued to show no plasmacytoma involvement. The patient is still living. Immunohistochemical examinations revealed monoclonal IgA kappa immunoglobulin in the cytoplasm of infiltrating plasma cells in all surgical specimens examined. Immunoelectrophoresis revealed serum M-component only when the patient had a splenic tumor. Soon after splenectomy, the serum M-component disappeared. PEMP of the small intestine is rare.
Assuntos
Neoplasias do Jejuno/imunologia , Plasmocitoma/imunologia , Feminino , Humanos , Imunoglobulina A/metabolismo , Cadeias kappa de Imunoglobulina/metabolismo , Imuno-Histoquímica , Neoplasias do Jejuno/patologia , Pessoa de Meia-Idade , Plasmocitoma/patologiaAssuntos
Ácidos Araquidônicos/farmacologia , Transtornos da Coagulação Sanguínea/sangue , Calcimicina/farmacologia , Agregação Plaquetária/efeitos dos fármacos , Adulto , Ácidos Araquidônicos/sangue , Transtornos da Coagulação Sanguínea/genética , Plaquetas/metabolismo , Plaquetas/ultraestrutura , Cálcio/metabolismo , Feminino , HumanosRESUMO
Two cases of von Willebrand disease (vWD) associated with familial thrombocytopenia were reported. The proband (daughter) and her father showed thrombocytopenia with large platelets and decreased von Willebrand factor activity (VIIIR:WF). Factor VIII procoagulant activity (VIII:C) and factor VIII-related antigen (VIIIR:AG) were normal, but both patients revealed an increased ristocetin-induced platelet aggregation and a qualitative abnormality of the factor VIII protein, which was characterized by fast electrophoretic mobility of VIIIR:AG and an abnormal elution of factor VIII-related activities on Sepharose 2B. DDAVP was hemostatically effective even in this thrombocytopenic patient undergoing a dental extraction.
Assuntos
Agregação Plaquetária/efeitos dos fármacos , Trombocitopenia/genética , Doenças de von Willebrand/genética , Adulto , Plaquetas/fisiologia , Criança , Fator VIII/metabolismo , Feminino , Humanos , Masculino , Linhagem , Ristocetina/farmacologia , Trombocitopenia/complicações , Trombocitopenia/fisiopatologia , Doenças de von Willebrand/complicações , Doenças de von Willebrand/fisiopatologiaRESUMO
Using ferritin as surface marker, the localization of the surface immunoglobulin (Ig) was studied on peripheral lymphocytes from normal human individuals and patients with macroglobulinaemia Waldenström by scanning immunoelectron microscopy. Normal IgG-, IgM-lymphocytes and pathological IgM-lymphocytes were then compared with regard to their topographical differences. In all cells examined, IgG- and IgM-conjugated ferritin particles were detected all over the cell surface, but the distribution of the former on the normal IgG-lymphocytes was slightly more diffuse than that of the latter on the normal and pathological IgM-lymphocytes. Furthermore, in the pathological IgM-lymphocytes, the clustered IgM-conjugated ferritin particles were found in great number on the microvilli. Normal IgG-lymphocytes were almost always characterized by short rod-like microvilli standing densely and vertically on the cell surface. Some of normal IgM-lymphocytes had a similar appearance to those of normal IgG-lymphocytes (type A) but others (type B) had tilted rod-like microvilli or wide plate-like processes on their surface. As for IgM-lymphocytes of macroglobulinaemia, most lymphocytes had tilted rodlike mirovilli and wide plate-like processes similar to type B, whereas a minor population of the pathological lymphocytes carried long, thin rod-like microvilli standing vertically on the surface.
