Sasidharan P K, Prasanth Varghese C, Sandeep P, Sreejith R, Shaan Mohammed, Shiji P V, Satish H, Feroz M
Department of Medicine, Calicut Medical College, Calicut 673008, Kerala, India.
Natl Med J India. 2011 Jan-Feb;24(1):19-20.
Haemophagocytic syndrome is a life-threatening systemic illness characterized by an uncontrolled inflammatory response. Patients present with fever, hepatosplenomegaly, jaundice and liver dysfunction, neurological manifestations and often pancytopenla. Bone marrow, lymph node, hepatic or splenic biopsy shows macrophages with Ingested blood cells or their precursors. Laboratory markers include elevated triglycerides and ferritin, low fibrinogen with normal or low erythrocyte sedimentation rate (ESR). Familial haemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder. Secondary haemophagocytic syndrome results from infections, malignancy and collagen vascular disorders. We describe a young girl with primary haemophagocytic syndrome.
噬血细胞综合征是一种危及生命的全身性疾病，其特征为不受控制的炎症反应。患者表现为发热、肝脾肿大、黄疸和肝功能障碍、神经表现，且常伴有全血细胞减少。骨髓、淋巴结、肝脏或脾脏活检显示巨噬细胞吞噬了血细胞或其前体。实验室指标包括甘油三酯和铁蛋白升高、纤维蛋白原降低，红细胞沉降率（ESR）正常或降低。家族性噬血细胞性淋巴组织细胞增生症（HLH）是一种常染色体隐性疾病。继发性噬血细胞综合征由感染、恶性肿瘤和胶原血管疾病引起。我们描述了一名患有原发性噬血细胞综合征的年轻女孩。
