Kerley Robert Noel, Kelly Raymond Michael, Cahill Mary Rose, Kenny Louise Clare
University College Cork, National University of Ireland, Cork, Ireland.
Cork University Hospital, University College Cork, Cork City, Ireland.
BMJ Case Rep. 2017 Apr 22;2017:bcr-2017-219516. doi: 10.1136/bcr-2017-219516.
Haemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, haematological disorder, which can be clinically challenging to diagnose and manage. We report a case of HLH in a previously healthy 33-year-old primigravida. The patient presented at 22 weeks gestation with dyspnoea, abdominal pain, anaemia, thrombocytopenia and elevated liver enzymes suggestive of HELLP syndrome.HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets is considered a severe form of pre-eclampsia. Despite delivery of the fetus, her condition deteriorated over 3-4 days with high-grade fever, worsening thrombocytopenia and anaemia requiring transfusion support. A bone marrow biopsy showed haemophagocytosis and a diagnosis of HLH was made. Partial remission was achieved with etoposide-based chemotherapy and complete remission following bone marrow transplantation. Eleven months post-transplant, the disease aggressively recurred, and the patient died within 3 weeks of relapse.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、可能致命的血液系统疾病，其诊断和管理在临床上具有挑战性。我们报告一例33岁初产妇的HLH病例，该患者此前身体健康。患者在妊娠22周时出现呼吸困难、腹痛、贫血、血小板减少以及肝酶升高，提示为HELLP综合征。HELLP综合征以溶血、肝酶升高和血小板减少为特征，被认为是子痫前期的一种严重形式。尽管分娩了胎儿，但她的病情在3 - 4天内恶化，出现高热、血小板减少和贫血加重，需要输血支持。骨髓活检显示噬血细胞现象，诊断为HLH。基于依托泊苷的化疗使病情部分缓解，骨髓移植后完全缓解。移植后11个月，疾病迅速复发，患者在复发后3周内死亡。
