CRISE DE RETENÇÃO ESPLÉNICA MAJOR NUM ADULTO COM DREPANOCITOSE | MAJOR ACUTE SPLENIC SEQUESTRATION CRISIS IN AN ADULT PATIENT WITH SICKLE-CELL DISEASE
Resumo
PT
A crise de retenção esplénica é uma complicação, frequentemente, fatal da drepanocitose. É rara em adultos, pela elevada
incidência de autoesplenectomia durante a infância. Heterozigóticos com traços de drepanocitose e de beta-talassémia têm
fenótipos menos graves, podendo manter um baço funcional até à idade adulta. Descrevemos um caso de crise de retenção
esplénica num homem de 19 anos, com concentração mínima de hemoglobina de 2,9g/dL, que resolveu após esplenectomia
emergente. Os poucos casos descritos na literatura acarretam uma mortalidade elevada. Um diagnóstico rápido e actuação
imediata são necessários para garantir a sobrevivência. É apresentada uma revisão da fisiopatologia e da abordagem tera-
pêutica desta entidade.
Palavras-chave: Anemia falciforme; Talassemia; Esplenomegália.
EN
Acute splenic sequestration crisis is an often fatal complication of sickle-cell disease, which is rare in adults due to high autosplenectomy
rates during childhood. Heterozygotes with sickle-cell and beta-thalassemia traits have milder phenotypes, carrying functional spleens onto
adulthood more frequently. Here we describe a case of acute splenic sequestration crisis in a 19 years-old male, with hemoglobin levels of
2,9g/dL, which was resolved after an emergency splenectomy. The few cases in adults described in the literature carry a high mortality rate.
Prompt diagnosis and immediate action are required to guarantee survival. A review of acute splenic sequestration crisis pathophysiology
and management is presented.
Key words: Sickle cell anemia; Thalassemia; Splenomegaly.
Texto Completo:
PDFReferências
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