RESUMO
The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to increased resistance to APC, which in turn may contribute to the vaso-occlusive complications of SS disease.
Assuntos
Anemia Falciforme/sangue , Transtornos das Proteínas Sanguíneas/sangue , Proteína C , Adolescente , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/genética , Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/complicações , Transtornos das Proteínas Sanguíneas/complicações , Feminino , Homozigoto , Humanos , Masculino , Tempo de ProtrombinaRESUMO
The activated protein C resistance (APC-R) ratios in 50 patients with steady state homozygous sickle cell (SS) disease and 59 healthy AA controls was measured. There was a significant reduction in median APC-R ratio in sickle cell disease compared to controls. This reduction in APC-R ratio was not explained by (1) the presence of the factor V Leiden, found in only one of 165 patients with SS disease including those tested for APC-R, or (2) the presence of lupus anticoagulants. However, the raised levels of factor VIIIC in SS patients in this study may be contributing to the increased resistance to APC, which in turn may contribute to the vaso-occlusive complication of SS disease.(AU)
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Anemia Falciforme/sangue , Transtornos das Proteínas Sanguíneas/sangue , Proteína C , Anemia Falciforme/complicações , Anemia Falciforme/genética , Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/complicações , Transtornos das Proteínas Sanguíneas/complicações , Homozigoto , Tempo de ProtrombinaRESUMO
A 4-month-old girl who was examined because of splenomegaly had portal vein thrombosis, which apparently resulted from a combination of cytomegalovirus infection and deficiency of both protein S and protein C. The cytomegalovirus infection, by damaging endothelial cells, may have triggered a cascade of events that was ultimately expressed as portal vein thrombosis resulting from deficiency of proteins S and C.
Assuntos
Transtornos das Proteínas Sanguíneas/complicações , Infecções por Citomegalovirus/complicações , Veia Porta , Deficiência de Proteína C , Deficiência de Proteína S/complicações , Trombose/etiologia , Feminino , Humanos , LactenteRESUMO
Description of the first case of angio-immunoblastic lymphadenopathy with dysproteinemia diagnosed in a male patient 64 years old and which preceded the development of a lymphoma. The importance of this study is the association that may exist between certain drugs and the development of angioimmunoblastic lymphadenopathy, which occurred in our patient with the use of diphenylhydantoin. This disease can present itself up to 22 years after exposure to the agent.