Giona F, Caruso R, Testi A M, Moleti M L, Malagnino F, Martelli M, Ruco L, Giannetti G P, Annibali S, Mandelli F
Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy.
Cancer. 1997 Nov 1;80(9):1786-91.
Langerhans' cell histiocytosis (LCH) is a rare disorder of uncertain etiology, characterized by a wide clinical spectrum and varied behavior.
This retrospective study analyzed 11 adult patients with a diagnosis of LCH observed at the study institution between April 1988 and March 1993.
Based on the sites and extent of disease at diagnosis, patients were divided into four categories. Group A was comprised of four patients with unifocal bone disease who had surgical curettage. At last follow-up only 1 patient was in continuous complete response (CCR) at 29+ months. The other 3 patients recurred at 3, 12, and 30 months, respectively, after surgery and at last follow-up were found to be in CR at 16+, 48+, and 124+ months, respectively, after therapy with vinblastine (VBL) and high dose methylprednisolone (HDMP). Group B was comprised of three patients with multifocal bone disease. Two of these patients received VBL + HDMP; at last follow-up, 1 patient was in CCR 8 months after completion of therapy, and the other developed progressive disease 11 months later. The third patient was treated with interferon (IFN) and at last follow-up was in CCR at 35+ months. Group C was comprised of 2 patients with bone and visceral disease who were treated with etoposide (VP-16) + HDMP; at last follow-up, 1 patient was in CCR at 42+ months and the other patient, who had isolated vulvar recurrence 16 months later, was in CR with treatment with local IFN. Group D was comprised of two patients with lung and lymph node involvement, one of whom was treated with VP-16 + HDMP and the other with cyclophosphamide, doxorubicin, vincristine, and prednisone; at last follow-up, both were in CCR at 30+ and 71+ months, respectively.
VBL + HDMP showed efficacy in patients with bone disease, in particular those treated for recurrent LCH after surgery. Therapy with VP-16 and HDMP was successfully employed in patients with visceral disease. IFN was effective both for localized disease and in patients with multiple bone lesions.
朗格汉斯细胞组织细胞增多症（LCH）是一种病因不明的罕见疾病，临床谱广泛，表现多样。
这项回顾性研究分析了1988年4月至1993年3月在研究机构观察到的11例诊断为LCH的成年患者。
根据诊断时疾病的部位和范围，将患者分为四类。A组由4例单灶性骨病患者组成，他们接受了手术刮除术。在最后一次随访时，仅1例患者在29 +个月时处于持续完全缓解（CCR）状态。其他3例患者分别在术后3、12和30个月复发，在最后一次随访时，经长春花碱（VBL）和大剂量甲基强的松龙（HDMP）治疗后，分别在16 +、48 +和124 +个月时处于CR状态。B组由3例多灶性骨病患者组成。其中2例患者接受了VBL + HDMP治疗；在最后一次随访时，1例患者在完成治疗后8个月处于CCR状态，另1例患者在11个月后出现疾病进展。第3例患者接受了干扰素（IFN）治疗，在最后一次随访时，在35 +个月时处于CCR状态。C组由2例骨和内脏疾病患者组成，他们接受了依托泊苷（VP - 16）+ HDMP治疗；在最后一次随访时，1例患者在42 +个月时处于CCR状态，另1例患者在16个月后出现孤立的外阴复发，经局部IFN治疗后处于CR状态。D组由2例肺部和淋巴结受累患者组成，其中1例接受了VP - 16 + HDMP治疗，另1例接受了环磷酰胺、阿霉素、长春新碱和泼尼松治疗；在最后一次随访时，两人分别在30 +和71 +个月时处于CCR状态。
VBL + HDMP对骨病患者有效，尤其是对术后复发性LCH患者。VP - 16和HDMP联合治疗在内脏疾病患者中取得了成功。IFN对局限性疾病和多骨病变患者均有效。
