Koduri Prasad Rao, Kovarik Paula
Ann Hematol. 2006 Sep;85(9):633-5. doi: 10.1007/s00277-006-0125-1. Epub 2006 May 17.
Acute splenic sequestration crisis (ASSC) is a major cause of morbidity and mortality in children with sickle cell disease. Reports of ASSC in adults with sickle beta-thalassemia (S-beta(thal)) are rare and consist of isolated case reports comprising a total of seven patients, three of whom died during the crisis. We report a 22-year-old man with S-beta(thal) who developed ASSC 1 day after suffering multiple blunt trauma. Systemic inflammatory response to severe blunt trauma may have precipitated ASSC in our patient. ASSC in adults with S-beta(thal) is a potentially life-threatening complication with a high risk of recurrence. Splenectomy is recommended after the first attack of ASSC in adults with S-beta(thal).
急性脾滞留危象(ASSC)是镰状细胞病患儿发病和死亡的主要原因。关于成人镰状β地中海贫血(S-β(thal))患者发生ASSC的报道很少,仅有个别病例报告,总共7例患者,其中3例在危象期间死亡。我们报告1例22岁的S-β(thal)男性患者,在遭受多处钝器伤1天后发生ASSC。对严重钝器伤的全身炎症反应可能促使我们的患者发生了ASSC。成人S-β(thal)患者发生ASSC是一种潜在的危及生命的并发症,复发风险高。对于成人S-β(thal)患者,首次发生ASSC后建议行脾切除术。