FAMILIAL HAEMOPHAGOCYTIC RETICULOSIS
FAMILIAL HAEMOPHAGOCYTIC RETICULOSIS
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DOI：
10.1136/adc.27.136.519
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发表时间：
1952-01-01
影响因子：
5.2
通讯作者：
CLAIREAUX, AE
中科院分区：
文献类型：
--
作者：
FARQUHAR, JW;CLAIREAUX, AE
一种罕见且总是致命的疾病，其特征是进行性红细胞减少，伴有或不伴有循环中粒细胞和血小板的抑制，尽管骨髓具有高反应性，以前曾在成人中描述过这种情况。肝、脾、淋巴结肿大，全身中毒深，可能出现复发性发热。病程很少超过几个月，并可能出现终末期黄疸和紫癜。Scott和Robb-Smith(1939)，Anderson(1944)和Asher(1946)记录了这样的病例。我们不想将网状内皮系统的命名进一步复杂化，但我们确实认为Robb-Smith(1938)为这种情况建议的组织细胞性髓质网状增生症的名称，虽然恰当地描述了淋巴结中的细胞损害，但当考虑到整个疾病过程时，它不如噬血细胞网状增生症合适。后者不仅表明所涉及的系统，而且还指示异常的过程，从而引导疾病可能追求的临床过程，而其他网状病的名称通常是同名的或纯粹的组织学名称。
A rare and invariably fatalcondition, characterized by progressive erythropenia with or without depression of the circulating granulocytes and platelets despite a highly reactive marrow, has previously been described in adults. The liver, spleen and lymph nodes are enlarged, general intoxication is profound, and there may be a relapsing fever. The course seldom exceeds a few months, and terminally jaundice and purpura may appear. Such cases have been recorded by Scott and Robb-Smith (1939), Anderson (1944) and Asher (1946).We have no desire to complicate further the nomenclature of the reticulo-endothelial system, but we do feel that the name histiocytic medullary reticulosis suggested for this condition by Robb-Smith (1938), although aptly describing the cellular lesion in the lymph nodes, is less appropriate than haemophagocytic reticulosis when the whole disease process is considered. The latter not only indicates the system involved, but also the abnormal process and thus gives a lead to the clinical course the disease may pursue, whereas the names of other reticuloses are often eponymous or purely histological.