Egeler R M, Neglia J P, Aricò M, Favara B E, Heitger A, Nesbit M E
Sophia Children's Hospital, Rotterdam, The Netherlands.
Med Pediatr Oncol. 1994;23(2):81-5. doi: 10.1002/mpo.2950230204.
Langerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was developed with the cooperation of the Histiocyte Society. In 1991 the Histiocyte Society surveyed its members requesting information on cases in which LCH was associated with malignancy. The questionnaire was mailed to all members of the society and specifically requested information on the clinical and laboratory features of the cases, disease evolution, and response to therapy. Retrospective reporting was allowed. With this initial data, an ongoing registry of LCH patients with associated malignancy was begun of such cases, including evolution and response to therapy. Twenty-seven patients were enrolled during the first year of registry, of whom 4 patients had the association of LCH with a malignant lymphoma and 10 cases had an association of LCH with other types of solid tumor. The remaining 13 patients had the association of LCH with acute leukemia. In five cases, LCH was associated with acute lymphoblastic leukemia FAB L1 (ALL). In four cases the ALL preceded the LCH by 6 months to 1 year. In four of five patients the LCH was localized; in two instances the LCH was treated with chemotherapy. In all cases the leukemia was treated according to local standard ALL protocols and in one case autologous bone marrow transplantation (ABMT) was performed at relapse. Three patients are free of leukemia, one of whom has persistent localized LCH of the skin. Two patients died of the ALL, one of whom was free of the LCH at the time of death. In eight instances LCH was reported in association with acute myeloid leukemia (AML). Six of these patients had a generalized form of LCH. In seven the diagnosis of LCH preceded the diagnosis of leukemia by more than 2 years (median 4 years). In the remaining patient both diagnoses were made concurrently. In all seven cases in whom LCH was the initial diagnosis the treatment consisted of chemotherapy and/or radiotherapy. Seven patients died from the AML, five without evidence of LCH. The temporal patterns of the LCH-ALL and LCH-AML associations are distinct with ALL usually preceding the diagnosis of LCH and AML succeeding it.(ABSTRACT TRUNCATED AT 400 WORDS)
朗格汉斯细胞组织细胞增多症（LCH）与同一患者体内发生的恶性肿瘤并存的情况较为罕见，通常只见于个别病例报告。为了更好地界定这些事件的发生情况，在组织细胞协会的合作下建立了一个同步或异步LCH与恶性肿瘤病例登记处。1991年，组织细胞协会对其成员进行了调查，要求提供LCH与恶性肿瘤相关病例的信息。问卷被邮寄给协会的所有成员，特别要求提供病例的临床和实验室特征、疾病进展以及对治疗的反应等信息。允许进行回顾性报告。根据这些初始数据，开始了一个关于LCH合并恶性肿瘤患者的持续登记处，记录此类病例，包括疾病进展和对治疗的反应。登记处的第一年有27名患者登记，其中4例患者的LCH与恶性淋巴瘤相关，10例患者的LCH与其他类型的实体瘤相关。其余13例患者的LCH与急性白血病相关。在5例病例中，LCH与急性淋巴细胞白血病FAB L1（ALL）相关。在4例病例中，ALL在LCH之前出现6个月至1年。在5例患者中的4例中，LCH为局限性；2例患者的LCH接受了化疗。所有病例中的白血病均按照当地标准的ALL方案进行治疗，1例患者在复发时进行了自体骨髓移植（ABMT）。3例患者白血病已缓解，其中1例患者皮肤仍有持续性局限性LCH。2例患者死于ALL，其中1例患者在死亡时无LCH。在8例病例中，LCH与急性髓系白血病（AML）相关。这些患者中有6例为全身性LCH。在7例病例中，LCH的诊断先于白血病诊断2年以上（中位数为4年）。在其余1例患者中，两种诊断同时做出。在所有7例以LCH为初始诊断的病例中，治疗包括化疗和/或放疗。7例患者死于AML，5例无LCH证据。LCH-ALL和LCH-AML关联的时间模式不同，ALL通常先于LCH诊断，而AML则在其后。（摘要截选至400字）
