RESUMO
In connection with three observations, we discuss clinical and biological characteristics of "acquired haemophilia". We insist on therapeutics problems: in all the cases corticosteroid and or immunosuppressive drugs are indicated: haemorrhagic accidents treatment depend on the clinical state severity and on the inhibitor concentration: indications of substitutive therapy, plasmapheresis and activated concentrate are considered.
Assuntos
Antígenos/imunologia , Autoanticorpos/análise , Fator VIII/imunologia , Hemofilia A/imunologia , Idoso , Feminino , Hemofilia A/tratamento farmacológico , Hemofilia A/etiologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
The authors report the results of Dixon's assay modified by Follea in 24 cases of idiopathic thrombocytopenic purpura. The level of immunoglobulin G bound to platelet membrane was increased in 91 p. 100 of patients in the acute phase of the disease. An inverse correlation was demonstrated between platelet-bound antibody levels and platelet count as well as platelet survival. In all cases of refractory idiopathic thrombocytopenic purpura, the assay was positive and the mean level was higher. When the platelet count improved after prednisone therapy or after splenectomy, the level decreased. Platelet antibody determination seems to be useful for predicting the course of the disease. Patients with normal immunoglobulin G bound to platelet might have only C3, IgM or IgA. Dixon's assay is not specific to idiopathic thrombopenic purpura, since it is positive in other types of thrombocytopenia, but immunoglobulin G bound to platelet probably represents specific antiplatelet antibodies.
Assuntos
Plaquetas/imunologia , Imunoglobulina G/análise , Púrpura Trombocitopênica/imunologia , Humanos , Técnicas ImunoenzimáticasRESUMO
INTRODUCTION: The misleading aspects of appendicitis are multiple. We report an observation, original not only from a clinical and bacteriological point of view but also because of the presence of a portal vein thrombosis. OBSERVATION: A 48 year-old man was hospitalized for prolonged fever. Examination revealed a thrombosis of the portal vein. Several hemocultures were positive for Prevotella melaninogenica. There was no abnormality in blood crasis and/or thrombophilia. Since the digestive and endoscopic control was negative, as well as the scanographic and sonographic exploration of the appendix area, exploratory laparotomy was performed and revealed an abscess on the appendix, which was responsible for the clinical, biological and radiological images. Appendectomy led to complete, immediate and permanent regression of the fever. COMMENTS: The discovery of a Prevotella-type germ disputes the pathogenicity of such an anaerobic germ, at distance from a site where it is normally saprophyte.
Assuntos
Abscesso Abdominal/cirurgia , Apendicite/diagnóstico , Infecções por Bacteroidaceae/diagnóstico , Veia Porta , Prevotella melaninogenica , Sepse/diagnóstico , Trombose/diagnóstico , Abscesso Abdominal/diagnóstico , Apendicectomia , Apendicite/cirurgia , Infecções por Bacteroidaceae/cirurgia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Trombose/cirurgiaRESUMO
A 64-year old man presented with microcytic hypochromic aplastic acquired anemia without iron depletion. His bone marrow was hypercellular with dyserythropoiesis and no stainable iron deposits. 59 Fe incorporation by erythroblasts was reduced, and the karyotype revealed an aneuploidy with marker chromosome. After study of transferrin receptor with specific antibody, we conclude that the receptor presents a functional defect.
Assuntos
Anemia Hipocrômica/genética , Medula Óssea/patologia , Aberrações Cromossômicas/diagnóstico , Receptores de Superfície Celular/metabolismo , Anemia Hipocrômica/diagnóstico , Anemia Hipocrômica/metabolismo , Anticorpos Monoclonais , Exame de Medula Óssea , Transtornos Cromossômicos , Eritroblastos/metabolismo , Eritropoese , Humanos , Ferro/metabolismo , Masculino , Pessoa de Meia-Idade , Receptores da TransferrinaRESUMO
We report the case of a 51 years old woman with myositis, myasthenia gravis and thymoma. First apparent sign is myositis in 1976 but chest X ray show a mediastinal opacity and the patient reports an intermittent diplopia since 1973. The evolution of myositis occurs in two bouts in 1976 and 1981, Myasthenia gravis restricted to diplopia from 1973 to 1979 grow worse first alone then in association with increase of myositis signs in 1981. The mediastinal opacity seen on chest X ray in 1976 don't change and is revealed to be a thymoma at operation in 1981. After thymoma ablation myasthenic and myositis signs decrease. This pathologic association is found 24 times in literature and involves "giant cells" in muscle biopsy in about 50 p. 100 of cases and a myocarditis also with "giant cells". Those "giant cells" unusual in common myositis appears to have a prognostic value.