Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Department of Hematology Oncology, Penn Presbyterian Medical Center, Philadelphia, PA, USA.
Hemoglobin. 2021 Jan;45(1):60-61. doi: 10.1080/03630269.2020.1868495. Epub 2021 Feb 15.
Acute splenic sequestration crisis (ASSC) is a life-threatening complication of sickle cell disease characterized by acute splenic enlargement and a rapid drop in hemoglobin (Hb) levels. Although ASSC is predominantly a disease of childhood, rare cases of ASSC have been reported in adults, frequently in association with heterozygous sickle cell disease. We describe a case of ASSC in a 29-year-old woman with homozygous Hb S (: c.20A>T) disease and suspected hereditary persistence of fetal Hb (HPFH), which was accompanied by marked leukocytosis.
急性脾隔离症危机（ASSC）是镰状细胞病的一种危及生命的并发症，其特征为急性脾肿大和血红蛋白（Hb）水平迅速下降。虽然 ASSC 主要发生在儿童期，但也有罕见的成人 ASSC 病例报告，常与杂合子镰状细胞病相关。我们描述了一例 29 岁女性的 ASSC 病例，该患者携带纯合 Hb S（: c.20A>T）疾病，疑似遗传性胎儿血红蛋白持续存在（HPFH），同时伴有明显的白细胞增多。
