HISTIOCYTOSIS X (EOSINOPHILIC GRANULOMA OF BONE, LETTERER-SIWE DISEASE, AND SCHUELLER-CHRISTIAN DISEASE). FURTHER OBSERVATIONS OF PATHOLOGICAL AND CLINICAL IMPORTANCE.
HISTIOCYTOSIS X (EOSINOPHILIC GRANULOMA OF BONE, LETTERER-SIWE DISEASE, AND SCHUELLER-CHRISTIAN DISEASE). FURTHER OBSERVATIONS OF PATHOLOGICAL AND CLINICAL IMPORTANCE.
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组织细胞增多症 X（骨嗜酸性肉芽肿、Letterer-SIWE 病和 Schueller-Christian 病）。
DOI：
10.2106/00004623-196446010-00007
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发表时间：
1964
期刊：
影响因子：
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通讯作者：
L. Lichtenstein
中科院分区：
文献类型：
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作者：
L. Lichtenstein
从1952年至今，对组织细胞增多症X的文献进行了综述。在此期间的临床和病理学观察倾向于证实组织细胞增多症X作为一个疾病分类实体的综合概念，包括骨嗜酸性肉芽肿、Letterer-Siwe病（当这是非肿瘤性时）和Schuller-Christian病。嗜酸性肉芽肿病变是对病原体的早期、相当迅速发展的反应的病理表现，这一印象得到了加强。这似乎是特别真实的慢性传播形式的疾病（Schuller-Christian病）。最近记录的经验增加了新的发病部位肝脏、肾脏、女性生殖器，可能还有脑神经和眼睛。总之，这种疾病的表现可能是相当多变的。特别值得注意的是大量关于肺嗜酸性肉芽肿的报道，这些肉芽肿在其他地方有或没有明显的病变。
强调明确区分急性（或亚急性）组织细胞增生症X和肿瘤性网状内皮组织增生症的实际重要性，两者都需要不同的治疗方法。从现有证据来看，急性播散性组织细胞增生症X应避免使用细胞毒性药物、放射性同位素等，因为它们可能严重损伤已受损的骨髓。在这些情况下，正如许多严重的慢性播散性组织细胞增生症X（Schuller-Christian病）病例一样，近年来的经验指出了类固醇在帮助患者度过危急情况方面的价值。同时，选定的抗生素在对抗继发性感染和X线治疗，特别是皮肤粘膜和骨骼病变的价值，已被进一步证实。
我们的资料显示：（1）慢性播散性组织细胞增生症X特异性累及肝脏，导致严重的肝内梗阻性黄疸;（2）骨嗜酸性肉芽肿向局部淋巴结的扩展（加强感染的概念）;（3）嗜酸性肉芽肿病灶的再现是慢性播散性组织细胞增生症X复发的病理表现（Schuller-Christian disease）长期存在。
The literature on histiocytosis X has been reviewed from 1952 to date. Clinical and pathological observations during this period tended to substantiate the integrated concept of histiocytosis X as a nosological entity embracing eosinophilic granuloma of bone, Letterer-Siwe disease (when this is non-neoplastic), and Schuller-Christian disease. The impression has been reinforced that the lesion of eosinophilic granuloma is the pathological expression of an early, rather rapidly developing reaction to an etiological agent. This appears to be especially true in the chronic disseminated form of the malady (Schuller-Christian disease). Recent recorded experience has added new sites of involvement—the liver, kidneys, female genitalia, and, possibly, the cranial nerves and eye, as well. Altogether, the manifestations of the disease may be quite protean. Especially noteworthy are the numerous reports on pulmonary eosinophilic granuloma, occurring with or without demonstrable lesions elsewhere.
Stress was placed on the practical importance of making a clear distinction between acute (or subacute) histiocytosis X and neoplastic reticuloendotheliosis, both of which require different therapy. From existing evidence it would appear that cytotoxic agents, radioactive isotopes, and the like should be avoided in acute disseminated histiocytosis X since they may seriously injure an already damaged bone marrow. In these circumstances, as in many severe cases of chronic disseminated histiocytosis X (Schuller-Christian disease), the experience of recent years points up the value of steroids in tiding patients over critical situations. Collaterally, the value of selected antibiotics in combating secondary infections and of roentgen therapy, especially for mucocutaneous and skeletal lesions, has been further documented.
Among the features brought out by our own material are: (1) specific involvement of the liver in chronic disseminated histiocytosis X, leading to serious intrahepatic obstructive jaundice; (2) extension of eosinophilic granuloma in bone to regional lymph nodes (reinforcing the concept of infection); and (3) reappearance of the lesion of eosinophilic granuloma as the pathological expression of recrudescence in chronic disseminated histiocytosis X (Schuller-Christian disease) of long standing.