Egeler R M, Neglia J P, Puccetti D M, Brennan C A, Nesbit M E
Department of Pediatrics, University of Minnesota School of Medicine, Minneapolis 55455.
Cancer. 1993 Feb 1;71(3):865-73. doi: 10.1002/1097-0142(19930201)71:3<865::aid-cncr2820710334>3.0.co;2-0.
The association of Langerhans cell histiocytosis (LCH) with a malignant neoplasm is rare and generally has been the subject of isolated case reports.
A recent case of LCH seen at the University of Minnesota in combination with acute lymphoblastic leukemia led the authors to review their own charts from 1960 onward, in addition to the literature for other reported associations of LCH and malignant neoplasms.
In addition to the presented case and 3 cases from the files of the authors, the literature contained 87 reported cases. Of the 91 patients, 39 had LCH with malignant lymphoma (ML); 25 of these cases were Hodgkin disease. In 11 of these 39 patients, the LCH was diagnosed from 12 months to 33 years after the ML was diagnosed. In 62% of the patients with LCH-ML (24 patients), the diagnosis was made concurrently and the Langerhans cells were found in the same lymph nodes. In the remaining four patients, the diagnosis of LCH preceded that of ML by 6-24 months. In 22 patients, including 2 patients in the files of the authors, LCH was reported in association with leukemia; 16 (73%) of these cases were associated with acute nonlymphoblastic leukemia. In two cases the leukemia preceded the LCH. In 6 patients both diagnoses were made concurrently, and in 14 patients (64%) the diagnosis of LCH preceded the diagnosis of leukemia by 8 months to 17 years. In the remaining 30 patients, LCH was associated with a variety of solid tumors, including a lung carcinoma in 12 patients. In all of these 12 cases the LCH was confined to the lung, and in 75% (9 of 12) of patients the diagnoses were made concurrently. In the 16 patients in whom the LCH preceded the solid tumor, the malignant diseases in 69% (11 of 16) developed within the radiation field used for the treatment of the LCH.
The intimate and simultaneous association of LCH with ML and lung carcinomas suggests strongly that the process that leads to the association is a reactive one. However, in the patients with leukemia and the other solid tumors, the latency of the malignant neoplasm after the diagnosis of LCH is suggestive of a therapy-related process.
朗格汉斯细胞组织细胞增多症（LCH）与恶性肿瘤的关联较为罕见，通常只见于个别病例报告。
明尼苏达大学近期接诊了一例LCH合并急性淋巴细胞白血病的病例，促使作者回顾了自1960年以来他们自己的病历，并查阅了关于LCH与恶性肿瘤其他报道关联的文献。
除了所报道的病例以及作者档案中的3例病例外，文献中还包含87例报道病例。在这91例患者中，39例LCH合并恶性淋巴瘤（ML）；其中25例为霍奇金病。在这39例患者中，有11例在ML诊断后12个月至33年被诊断出LCH。在LCH - ML患者中，62%（24例患者）的诊断是同时做出的，且在同一淋巴结中发现了朗格汉斯细胞。在其余4例患者中，LCH的诊断比ML早6 - 24个月。在22例患者中，包括作者档案中的2例患者，报道了LCH与白血病相关；其中16例（73%）与急性非淋巴细胞白血病相关。在2例病例中白血病先于LCH出现。在6例患者中，两种诊断同时做出，在14例患者（64%）中，LCH的诊断比白血病的诊断早8个月至17年。在其余30例患者中，LCH与多种实体瘤相关，其中12例患者患有肺癌。在所有这12例病例中，LCH局限于肺部，75%（12例中的9例）患者的诊断是同时做出的。在LCH先于实体瘤出现的16例患者中，69%（16例中的11例）的恶性疾病发生在用于治疗LCH的放疗区域内。
LCH与ML和肺癌的密切且同时出现的关联强烈提示导致这种关联的过程是一种反应性过程。然而，在白血病和其他实体瘤患者中，LCH诊断后恶性肿瘤的潜伏期提示这是一个与治疗相关的过程。
