Department of Hepatobiliary Surgery, Wenzhou Central Hospital, The Dingli Clinical Institute of Wenzhou Medical University, Wenzhou, 325000, Zhejiang, People's Republic of China.
BMC Surg. 2022 Apr 9;22(1):136. doi: 10.1186/s12893-022-01580-5.
Hereditary spherocytosis (HS) complicated by splenic infarction is very rare, and it is even rarer to develop splenic infarction after infectious mononucleosis (IM) as a result of Epstein-Barr virus (EBV) infection. Therefore, misdiagnosis or missed diagnosis is prone to occur.
A 19-year-old Chinese female previously diagnosed with HS was admitted to our institution with persistent high fever and icterus. On admission, the physical examination showed anemia, jaundice, marked splenomegaly, obvious tenderness in the left upper abdomen (LUA). Peripheral blood film shows that spherical red blood cells accounted for about 6%, and Immunoglobulin M (IgM) antibodies specific to Epstein-Barr virus (EBV) viral capsid antigen were detected. An abdominal CT scan revealed a splenic infarction. The patient was diagnosed with HS with splenic infarction following EBV infection and underwent an emergency laparoscopic splenectomy (LS). Pathological analysis showed a splenic infarction with red pulp expansion, white pulp atrophy and a splenic sinus filled with red blood cells. After two months of follow-up visits, the patient showed no signs of relapse.
HS complicated by splenic infarction is very rare and mostly occurs in men under 20 years of age and is often accompanied by other diseases, such as sickle cell traits (SCT) or IM. Although symptomatic management may be sufficient, emergency laparoscopic splenectomy may be safe and effective when conservative treatment is ineffective.
遗传性球形红细胞增多症（HS）并发脾梗死非常罕见，而由 EBV 感染引起的传染性单核细胞增多症（IM）后发生脾梗死则更为罕见。因此，容易发生误诊或漏诊。
一名 19 岁的中国女性，既往诊断为 HS，因持续性高热和黄疸入院。入院时体格检查显示贫血、黄疸、明显脾肿大、左上腹（LUA）明显压痛。外周血涂片显示球形红细胞占 6%左右，检测到针对 EBV 病毒衣壳抗原的特异性 IgM 抗体。腹部 CT 扫描显示脾梗死。患者被诊断为 EBV 感染后并发 HS 伴脾梗死，并紧急行腹腔镜脾切除术（LS）。病理分析显示脾梗死，红髓扩张，白髓萎缩，脾窦充满红细胞。随访两个月后，患者无复发迹象。
HS 并发脾梗死非常罕见，多发生于 20 岁以下男性，常伴有其他疾病，如镰状细胞特征（SCT）或 IM。虽然对症治疗可能足够，但在保守治疗无效时，急诊腹腔镜脾切除术可能是安全有效的。
