Haas R J, Janka G, Helmig M, Meister P
Onkologie. 1982 Feb;5(1):4-12. doi: 10.1159/000214958.
A retrospective study of 12 cases of so called histiocytosis-X and 3 cases of malignant histiocytosis was done. It was possible to establish the differences in clinical, morphologic and cytochemical findings of both diseases. The diagnosis of histiocytosis-X can be confirmed by multinucleated histiocytes interrupted by eosinophils and plasmacells. The histopathology of malignant histiocytosis is different and is characterized by atypical histiocytes. Erythrophagocytosis throughout the tissues is seen. Typical histochemistry (acid phosphatase and naphtol-AS-acetat-esterase) findings are also helpful for diagnosis. The treatment of both diseases should be continued at least six months after disappearance of clinical apparent lesions. Combination chemotherapy with vinblastine and prednisone is suggested. In cases of histiocytosis-X in isolated lesions curettage or irradiation may be adequate. Long term remissions and presumed cures of histiocytosis-X are possible in over 70% of the cases. A strict correlation between the prognosis and the degree of involvement is confirmed. Even in cases of malignant histiocytosis, previously reported as rapidly fatal disease, combination chemotherapy may produce complete long term remissions.
对12例所谓的组织细胞增多症-X和3例恶性组织细胞增多症进行了回顾性研究。得以明确这两种疾病在临床、形态学和细胞化学方面的差异。组织细胞增多症-X的诊断可通过多核组织细胞被嗜酸性粒细胞和浆细胞中断来证实。恶性组织细胞增多症的组织病理学不同，其特征为非典型组织细胞。可见整个组织中有吞噬红细胞现象。典型的细胞化学（酸性磷酸酶和萘酚-AS-乙酸酯酶）结果也有助于诊断。两种疾病的治疗应在临床明显病变消失后至少持续六个月。建议采用长春碱和泼尼松联合化疗。对于孤立性病变的组织细胞增多症-X病例，刮除术或放疗可能就足够了。超过70%的组织细胞增多症-X病例有可能实现长期缓解并假定治愈。预后与受累程度之间存在严格的相关性得到证实。即使在以前被报道为快速致命疾病的恶性组织细胞增多症病例中，联合化疗也可能产生完全的长期缓解。
