同时存在的活动性和神经退行性朗格汉斯细胞组织细胞增多症的识别与治疗：一例报告

Recognition and treatment of concurrent active and neurodegenerative langerhans cell histiocytosis: a case report.

作者信息

Ehrhardt Matthew J, Karst Jeffrey, Donohoue Patricia A, Maheshwari Mohit, McClain Kenneth L, Bingen Kristin, Kelly Michael E

机构信息

*Midwest Center for Cancer and Blood Disorders †Medical College of Wisconsin and Children's Hospital of Wisconsin ‡Department of Psychology, Marquette University, Milwaukee, WI §Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, TX.

出版信息

J Pediatr Hematol Oncol. 2015 Jan;37(1):e37-40. doi: 10.1097/MPH.0000000000000085.

DOI:10.1097/MPH.0000000000000085

PMID:24322503

Abstract

Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation with subsequent tissue damage often requiring chemotherapy. Neurodegenerative LCH presents with neuromuscular, cognitive, and behavioral alterations typically occurring years after diagnosis of active LCH. We present a male child with a 4-year history of growth arrest, polyuria, polydipsia, recurrent otitis media, and seborrheic dermatitis. Cutaneous biopsies confirmed LCH and chemotherapy was initiated. During treatment for active LCH he developed neuropsychiatric decline. White matter changes on brain MRI were consistent with neurodegenerative LCH. Treatment was changed to cytarabine and intravenous immunoglobulin. After 1 year of therapy the patient experienced neuropsychological improvement.

摘要

朗格汉斯细胞组织细胞增多症（LCH）是一种树突状细胞增殖紊乱疾病，随后常需化疗来治疗组织损伤。神经退行性LCH表现为神经肌肉、认知和行为改变，通常在活动性LCH诊断数年之后出现。我们报告一名男童，有4年生长停滞、多尿、烦渴、复发性中耳炎和脂溢性皮炎病史。皮肤活检确诊为LCH并开始化疗。在治疗活动性LCH期间，他出现了神经精神功能衰退。脑部MRI的白质改变与神经退行性LCH一致。治疗改为使用阿糖胞苷和静脉注射免疫球蛋白。治疗1年后，患者神经心理状况有所改善。