Pekrun A, Linne S, Schröter W
Universitäts-Kinderklinik Göttingen.
Monatsschr Kinderheilkd. 1993 Jul;141(7):573-5.
A 14 year-old patient developed severe anemia and splenomegaly 2 days after the onset of a febrile upper airway infection. The hemoglobin concentration had dropped to 1.1 g/dl. Death occurred as consequence of the acute anemia and peripheral circulatory failure. The crisis was caused by an acute splenic sequestration. Hemoglobin SC disease could be identified as the underlying disorder. Hemoglobin SC disease usually has a milder course than sickle cell disease. However the patients may develop the same crisis-like symptoms. Splenic sequestration is caused by the occlusion of the splenic sinuses due to sickled and aggregated erythrocytes with subsequent trapping of large blood volumes and circulatory failure. Regular transfusions and/or splenectomy are recommended to prevent splenic sequestration crisis.
一名14岁患者在发热性上呼吸道感染发作2天后出现严重贫血和脾肿大。血红蛋白浓度降至1.1 g/dl。患者因急性贫血和外周循环衰竭死亡。此次危象由急性脾滞留引起。血红蛋白SC病可被确定为潜在疾病。血红蛋白SC病通常病程比镰状细胞病轻。然而，患者可能会出现相同的危象样症状。脾滞留是由于镰状和聚集的红细胞阻塞脾窦，随后大量血液被困，导致循环衰竭。建议定期输血和/或脾切除术以预防脾滞留危象。
