Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, 600 Yishan Road, Shanghai 200233, China.
Oncologist. 2013;18(1):19-24. doi: 10.1634/theoncologist.2012-0234. Epub 2013 Jan 8.
Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) share similar clinical features and mechanisms. In very rare circumstances, the two diseases coexist in the same patient. Here we report such a patient, who was first diagnosed with Hand-Schüller-Christian disease (HSC), a type of LCH. Several years later, the patient presented with severe exophthalmos and osteosclerosis on radiograph. New biopsy revealed ECD. We also analyze 54 cases of LCH and 6 cases of ECD diagnosed in our hospital, as well as their progression during a follow-up period of 8 years. In five cases of HSC (9.3% of LCH), a triad of central diabetes insipidus, hyperprolactinemia, and pituitary stalk thickening on magnetic resonance imaging (MRI) preceded the typical bone lesions by 4-9 years. In addition, LCH was featured as elevated plasma alkaline phosphatase (ALP), which was normal in ECD. Combined with a literature review, several features are summarized to differentiate ECD from HSC. In patients with diabetes insipidus, concomitant hyperprolactinemia and pituitary stalk thickening on MRI indicate a possible HSC. Additionally, if osteosclerosis is observed in a patient with LCH, the coexistence of ECD should be considered.
朗格汉斯细胞组织细胞增生症（LCH）和 Erdheim-Chester 病（ECD）具有相似的临床特征和发病机制。在极少数情况下，这两种疾病会同时存在于同一位患者身上。我们在此报告一例患者，该患者最初被诊断为 Hand-Schüller-Christian 病（HSC），这是 LCH 的一种类型。数年后，该患者出现严重的眼球突出和影像学上的骨硬化。新的活检显示为 ECD。我们还分析了我院诊断的 54 例 LCH 和 6 例 ECD 病例，以及它们在 8 年的随访期间的进展情况。在 5 例 HSC（LCH 的 9.3%）中，磁共振成像（MRI）上的正中糖尿病、高泌乳素血症和垂体柄增粗三联征比典型的骨病变早出现 4-9 年。此外，LCH 的特征是血浆碱性磷酸酶（ALP）升高，而 ECD 则正常。结合文献复习，总结了一些特征来区分 ECD 和 HSC。在患有尿崩症的患者中，如果同时存在高泌乳素血症和 MRI 上的垂体柄增粗，则提示可能为 HSC。此外，如果在 LCH 患者中观察到骨硬化，则应考虑同时存在 ECD。
