Arewa O P, Ajadi A A
Department of Haematology and Immunology, College of Health Sciences, Niger Delta University, Wilberforce Island Bayelsa State, Nigeria.
West Afr J Med. 2011 Jan-Feb;30(1):66-8. doi: 10.4314/wajm.v30i1.69922.
Haemophagocytic syndrome (HPS) is a rare condition that has been documented in association with viral, bacterial, fungal and parasitic infections as well as a wide spectrum of malignant neoplasms and autoimmune diseases. Whereas HPS is a known cause of anaemia in HIV infection, its occurrence is uncommon. Its occurrence in pregnancy is even rarer and very few such cases have been reported.
Full clinical evaluation and investigations including bone marrow aspiration cytology were done to elucidate the cause of the anaemia.
Evaluation revealed severe anaemia, increased serum bilirubin HIV positivity and a low CD4 count. The final diagnosis confirmed the case to be haemophagocytic syndrome in pregnancy. A 31-year-old primigravida presented at 21 weeks of gestation with a two-week history of fever, jaundice and abdominal pain. She responded to treatment.
Although a rare condition, this case highlights the importance of a high index of suspicion for the syndrome in HIV positive pregnant women with persistent Coombs negative haemolytic anaemia. Full recovery could be expected following prompt institution of HAART and delivery.
噬血细胞综合征(HPS)是一种罕见疾病,已被证明与病毒、细菌、真菌和寄生虫感染以及多种恶性肿瘤和自身免疫性疾病有关。虽然HPS是HIV感染中贫血的已知病因,但其发生率并不常见。其在妊娠期间的发生更为罕见,仅有极少数此类病例被报道。
进行了全面的临床评估和检查,包括骨髓穿刺细胞学检查,以阐明贫血的病因。
评估显示严重贫血、血清胆红素升高、HIV阳性以及CD4计数低。最终诊断证实该病例为妊娠合并噬血细胞综合征。一名31岁初产妇在妊娠21周时出现,有两周的发热、黄疸和腹痛病史。她对治疗有反应。
尽管这种情况罕见,但该病例凸显了对患有持续性库姆斯试验阴性溶血性贫血的HIV阳性孕妇高度怀疑该综合征的重要性。及时开始高效抗逆转录病毒治疗(HAART)并分娩后有望完全康复。