Stark B, Hershko C, Rosen N, Cividalli G, Karsai H, Soffer D
Cancer. 1984 Nov 15;54(10):2109-21. doi: 10.1002/1097-0142(19841115)54:10<2109::aid-cncr2820541010>3.0.co;2-m.
Eleven patients with familial hemophagocytic lymphohistiocytosis (FHLH) are described. They all belonged to four Jewish families of Iranian and Iraqi origin. Parental consanguinity was found in three families. The age of onset of disease ranged from 6 weeks to 36 months. All patients had fever, wasting, and enlargement of the liver and spleen. In addition, lymph-node enlargement and neurologic complications were common. The most consistent laboratory findings were pancytopenia, atypical lymphomonocytoid cells in the peripheral blood, abnormal liver function test results, and increased cerebrospinal fluid protein. The course was fatal in all patients. Nine of the 11 patients died within 2 weeks to 3 months of presentation, and 2 patients achieved temporary remissions but died of disease within 8 and 24 months, respectively. Response to antibiotic therapy or to the administration of corticosteroids and cytotoxic drugs was unimpressive. Pancytopenia complicated by sepsis or bleeding, hepatic failure, or encephalopathy were the terminal events. This report draws attention to the existence of FHLH in Jews of Iranian-Iraqi origin in whom parental consanguinity is very common.
本文描述了11例家族性噬血细胞性淋巴组织细胞增生症（FHLH）患者。他们均来自四个具有伊朗和伊拉克血统的犹太家庭。在三个家庭中发现了父母近亲结婚的情况。发病年龄从6周龄至36个月不等。所有患者均有发热、消瘦以及肝脏和脾脏肿大。此外，淋巴结肿大和神经系统并发症也很常见。最一致的实验室检查结果是全血细胞减少、外周血中非典型淋巴样单核细胞、肝功能检查结果异常以及脑脊液蛋白升高。所有患者的病程均为致命性。11例患者中有9例在就诊后2周内至3个月内死亡，2例患者分别在8个月和24个月内达到短暂缓解但最终死于该病。抗生素治疗或给予皮质类固醇和细胞毒性药物的反应不佳。全血细胞减少合并败血症或出血、肝衰竭或脑病是终末事件。本报告提请注意在父母近亲结婚非常普遍的伊朗 - 伊拉克裔犹太人中存在FHLH。
