Andrews J, Buchanan G R
Clin Pediatr (Phila). 1984 Jun;23(6):354-5. doi: 10.1177/000992288402300612.
Acute splenic sequestration crisis ( ASSC ), a common complication in homozygous sickle cell anemia, has been described infrequently in sickle-hemoglobin C (SC) disease in the absence of high altitude exposure. In this report, we describe three children with hemoglobin SC disease who developed episodes of ASSC that were milder than those generally described in sickle cell anemia. In one patient, an antecedent triggering event was not recognized. The other two children may have had a predisposing cause for massive intrasplenic sickling in that one had an associated mononucleosis syndrome and the other an episode of hypovolemic shock following severe epistaxis. ASSC may occur in patients with SC disease and does not necessarily require a hypoxic trigger associated with high altitudes.
急性脾滞留危象（ASSC）是纯合子镰状细胞贫血的常见并发症，在未暴露于高海拔环境的镰状血红蛋白C（SC）病中鲜有报道。在本报告中，我们描述了三名患有血红蛋白SC病的儿童，他们发生的ASSC发作比镰状细胞贫血中通常描述的情况更为轻微。在一名患者中，未识别出先前的触发事件。另外两名儿童可能有导致脾内大量镰状化的易感因素，其中一名患有相关的单核细胞增多症综合征，另一名在严重鼻出血后发生了低血容量性休克。ASSC可能发生在SC病患者中，不一定需要与高海拔相关的缺氧触发因素。
