Daneshbod K, Kissane J M
Department of Pathology, Pahlavi University, Shiraz, Iran.
Am J Clin Pathol. 1976 May;65(5):601-11. doi: 10.1093/ajcp/65.5.601.
Nine examples of polyostotic histiocytosis without visceral involvement with long-term follow-up are presented. Only one patient had the complete Hand-Schüller-Christian disease triad of multiple osteolytic lesions, diabetes insipidus, and exophthalmos. Three of the nine patients had solitary osseous lesions when first seen but other lesions developed within ten years. The other six had multiple lesions when first seen. In no case was progression to systemic histiocytosis observed. Eight patients were well and in good health seven to 18 years after initial diagnosis. One died indirectly of his disease. Low-dose radiation therapy seems to accelerate healing of lesions.
本文展示了9例无内脏受累的多骨型组织细胞增多症的长期随访病例。仅1例患者出现了多发性溶骨性病变、尿崩症和突眼这一完整的汉-许-克病三联征。9例患者中有3例初诊时为单发性骨病变，但在10年内出现了其他病变。另外6例初诊时就有多处病变。未观察到进展为系统性组织细胞增多症的病例。8例患者在初次诊断后7至18年情况良好、身体健康。1例患者间接死于其疾病。低剂量放射治疗似乎能加速病变的愈合。
