Rademacher Sibylle, Anagnostopoulos Joannis, Luft Friedrich C, Kettritz Ralph
Department of Nephrology and Intensive Care Medicine, Virchow Klinikum , Charité Medical Faculty , Berlin , Germany.
Department of Pathology , Charité Medical Faculty , Berlin , Germany.
Clin Kidney J. 2014 Aug;7(4):402-5. doi: 10.1093/ckj/sfu031. Epub 2014 Apr 4.
Erdheim-Chester disease is a rare inflammatory condition characterized by a non-Langerhans histiocytic infiltration, involving the skeleton, nervous system, viscera, retroperitoneum and elsewhere. The aetiology is unknown. Positron emission tomography shows areas of involvement. We managed a dialysis patient with knee pain; a bone marrow specimen showed typical CD68 positive, but CD1a negative cells. We initiated interferon-α therapy although other options remain open. In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.
厄尔德海姆-切斯特病是一种罕见的炎症性疾病,其特征为非朗格汉斯组织细胞浸润,累及骨骼、神经系统、内脏、腹膜后及其他部位。病因不明。正电子发射断层扫描可显示受累区域。我们诊治了一名患有膝关节疼痛的透析患者;骨髓标本显示典型的CD68阳性但CD1a阴性细胞。尽管还有其他治疗选择,但我们开始了α干扰素治疗。在我们的患者中,继发性甲状旁腺功能亢进与肿瘤性钙化同时存在,为骨骼疼痛提供了一个有趣的额外鉴别诊断可能性。