Yip Kay Por, Ali Muzzammil, Avann Felicity, Ganguly Saibal
Intensive Care Unit, New Cross Hospital, Wolverhampton, UK.
J Intensive Care Soc. 2020 Feb;21(1):87-91. doi: 10.1177/1751143718809678. Epub 2018 Oct 31.
Haemophagocytic lymphohistiocytosis is an aggressive and life-threatening syndrome of excessive immune activation. It is associated with various aetiologies, including infections, collagen vascular diseases and malignancies. Pregnancy-induced immune dysregulation in genetically susceptible women may also play a critical role in haemophagocytic lymphohistiocytosis. Our case involves that of a 23-year-old pregnant woman who presented at 22 weeks gestation with tachycardia, swinging pyrexia, rigors and generalised myalgia. Refractory hypotension to intravenous fluids and rise in lactate level required admission to the intensive care unit for vasopressor support. Despite treatment with broad-spectrum antibiotics for presumed sepsis, she made little clinical improvement. Investigations for infection and rheumatological disease were unremarkable. A pronounced hyperferritinaemia, hypertriglyceridaemia and cytopenia raised the suspicion of haemophagocytic lymphohistiocytosis. Subsequent elevated CD25 levels helped establish the diagnosis. Treatment with corticosteroids and intravenous immunoglobulin provided a transient response in regard to temperature control and cardiovascular stability. The decision was made to treat her with anakinra, an interleukin-1 receptor antagonist. She responded well to this with a complete resolution of her symptoms and normalisation of her ferritin levels over the course of some weeks. Because of progressive slowing of foetal growth and abnormal umbilical artery Dopplers and cardiotocography, she eventually had an emergency caesarean section at 31 + 5 weeks. There were no foetal abnormalities.
噬血细胞性淋巴组织细胞增生症是一种因过度免疫激活而具有侵袭性且危及生命的综合征。它与多种病因相关，包括感染、胶原血管疾病和恶性肿瘤。在基因易感性女性中，妊娠引起的免疫失调在噬血细胞性淋巴组织细胞增生症中也可能起关键作用。我们的病例涉及一名23岁孕妇，她在妊娠22周时出现心动过速、弛张热、寒战和全身肌痛。对静脉输液难治性低血压以及乳酸水平升高，需要入住重症监护病房接受血管活性药物支持。尽管使用广谱抗生素治疗疑似败血症，但她临床改善甚微。感染和风湿性疾病的检查无异常。明显的高铁蛋白血症、高甘油三酯血症和血细胞减少症引发了对噬血细胞性淋巴组织细胞增生症的怀疑。随后升高的CD25水平有助于确诊。使用皮质类固醇和静脉注射免疫球蛋白治疗在体温控制和心血管稳定性方面产生了短暂反应。决定用阿那白滞素（一种白细胞介素-1受体拮抗剂）对她进行治疗。她对此反应良好，在几周内症状完全缓解且铁蛋白水平恢复正常。由于胎儿生长逐渐减缓以及脐动脉多普勒检查和胎心监护异常，她最终在31 + 5周时紧急剖宫产。胎儿无异常。
