A Splenectomy in Plasma Exchange Refractory Thrombotic Thrombocytopenia Purpura / 대한진단검사의학회지
The Korean Journal of Laboratory Medicine
;
: 119-122, 2006.
Artigo
em Coreano
| WPRIM
| ID: wpr-216289
ABSTRACT
Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and variable abnormalities in renal function and mental status. The pathogenesis of TTP is related to an inhibitor or deficiency of the von Willebrand factor (vWF)-cleaving protease (a disintegrin and metalloprotease with thrombospondin type 1 repeats; ADAMTS-13) that cleaves the large vWF multimers. Uncleaved, large vWF molecules are present in TTP and induce thrombosis in small vessels. Even though plasma exchange was proven effective in TTP, 20-40% of the cases showed refractory to plasma exchange. We describe a 41 years old female with plasma exchange refractory TTP who was completely recovered from anemia, thrombocytopenia, and accompanying symptoms following splenectomy.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Plasma
/
Troca Plasmática
/
Púrpura
/
Púrpura Trombocitopênica Trombótica
/
Esplenectomia
/
Trombocitopenia
/
Trombose
/
Fator de von Willebrand
/
Trombospondinas
/
Febre
Limite:
Adulto
/
Feminino
/
Humanos
Idioma:
Coreano
Revista:
The Korean Journal of Laboratory Medicine
Ano de publicação:
2006
Tipo de documento:
Artigo
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