Familial hemophagocytic lymphohistiocytosis (FHLH) in Israel. I. Description of 11 patients of Iranian—Iraqi origin and review of the literature
Familial hemophagocytic lymphohistiocytosis (FHLH) in Israel. I. Description of 11 patients of Iranian—Iraqi origin and review of the literature
复制标题
以色列家族性噬血细胞性淋巴组织细胞增多症 (FHLH) I. 11 名伊朗-伊拉克裔患者的描述及文献综述
作者：
B. Stark;C. Hershko;Nehama Rosen;G. Cividalli;H. Karsai;D. Soffer
本文报告11例家族性噬血细胞性淋巴组织细胞增生症。他们都属于伊朗和伊拉克血统的四个犹太家庭。在三个家庭中发现了父母的血缘关系。发病年龄从6周到36个月不等。所有患者均有发热、消瘦、肝脾肿大等症状。此外，淋巴结肿大和神经系统并发症也很常见。最一致的实验室表现是全血细胞减少，外周血中不典型的淋巴单核细胞样细胞，肝功能检查结果异常，以及脑脊液蛋白增加。所有患者的病程都是致命的。11名患者中有9名在发病后2周至3个月内死亡，2名患者暂时缓解，但分别在8个月和24个月内死于疾病。对抗生素治疗或给予皮质类固醇和细胞毒性药物的反应平平。全血细胞减少合并败血症或出血、肝功能衰竭或脑病是终末期事件。本报告提请注意伊朗裔伊拉克裔犹太人中存在FHLH型血统，他们的父母血缘关系非常普遍。
Eleven patients with familial hemophagocytic lymphohistiocytøsis (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.
组织细胞疾病：病理生理学分析。
影响因子：
39.2
作者：
Groopman,JE;Golde,DW
通讯作者：
Golde,DW