van der Deure J, Kardos G, von Blomberg-van der Flier B M
Afd. Kindergeneeskunde, Academisch Ziekenhuis Vrije Universiteit. Amsterdam.
Ned Tijdschr Geneeskd. 1997 Oct 11;141(41):1973-5.
A lymph node biopsy sample from a boy aged with fever, pneumonia, hepatosplenomegaly, lymphadenopathy and pancytopenia, showed histiocytosis with erythrophagocytosis, compatible with the haemophagocytic syndrome. Treatment consisted of dexamethasone and etoposide, with cyclosporine added in a later phase. During the subsequent remission phase, bone marrow transplantation was carried out. Haemophagocytic syndrome is a rare condition, characterized by fever, pancytopenia, hepatosplenomegaly and characteristic laboratory findings (including a high interferon-gamma level) and morbid-anatomical findings (haemophagocytic histiocytic cells in bone marrow, lymph nodes, liver and spleen, but also in the CNS, kidneys and lungs). Recent pathophysiological discoveries indicate an enhanced T-cell response, leading to hypercytokinaemia. As a rule the patient dies from multiorgan failure and diffuse intravascular coagulation. Bone marrow transplantation is the treatment of choice.
一名患有发热、肺炎、肝脾肿大、淋巴结病和全血细胞减少症的男孩的淋巴结活检样本显示有组织细胞增多伴红细胞吞噬现象，符合噬血细胞综合征。治疗包括地塞米松和依托泊苷，后期加用环孢素。在随后的缓解期进行了骨髓移植。噬血细胞综合征是一种罕见疾病，其特征为发热、全血细胞减少、肝脾肿大以及特征性的实验室检查结果（包括高干扰素-γ水平）和病理解剖学发现（骨髓、淋巴结、肝脏和脾脏中存在噬血细胞组织细胞，中枢神经系统、肾脏和肺部也有）。最近的病理生理学发现表明T细胞反应增强，导致细胞因子血症。通常患者死于多器官功能衰竭和弥散性血管内凝血。骨髓移植是首选的治疗方法。
