Koduri Prasad Rao, Nathan Sunita
The Division of Hematology-Oncology, Cook County Hospital, Chicago, IL 60612, USA.
Ann Hematol. 2006 Apr;85(4):239-43. doi: 10.1007/s00277-005-0061-5. Epub 2006 Feb 7.
Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0-21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality-a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.
急性脾滞留危象（ASSC）是一种潜在的危及生命的并发症，也是镰状细胞病患儿的主要死因之一。在血红蛋白S-C病成人患者中很少有相关报道，且这些患者的自然病史尚未得到充分研究。我们在此描述了1972年至2000年间接受治疗的9例血红蛋白S-C病成人患者ASSC的临床病理特征，并对其进行了平均9年（范围0至21年）的随访。ASSC的特征为急性左上腹疼痛、脾肿大、发热以及伴有活跃红细胞生成的血细胞比容迅速下降。在ASSC期间，血红蛋白较稳定状态值平均下降4.8 g/dl（范围3.0至6.7 g/dl）。2例患者对浓缩红细胞输血无反应，需要紧急脾切除术。有1例死亡病例——一名76岁女性，入院36小时后死亡。分别对随访2年、3年、16年、18年和21年的5例患者进行观察，未出现ASSC复发情况。成人ASSC是血红蛋白S-C病的一种严重且偶有致命的并发症。血红蛋白S-C病患者在八十岁之前可能仍有发生ASSC的风险。
