- 期刊
Indeterminate Cell Histiocytosis: A Case Report
未明確細胞組織球增生症一病例報告
摘要
未明確細胞組織球增生症（ICH）是一種罕見的細胞組織球增生病變，其細胞的特性介於Langerhans氏細胞與非Langerhans氏細胞之間。本文報導一位36歲女性，近六個多月來全身泛發性出現不痛不養、堅實、帶微黃或紅棕顏色，觸摸的到之皮下及表皮腫瘤。組織病理學檢查、免疫組織學檢查和電子顯微鏡檢查可見ICH的特徵，即：（1）組織球細胞增生散在於真皮層中，並無epidermotropism或細胞atypia現象；（2）腫瘤細胞S-100及CD1a免疫染色呈陽性，及局部Factor XIIIa及CD68免疫染色呈陽性；（3）電子顯微鏡下找不到Birbeck顆粒。藉由流體細胞儀器檢測出病人週邊血中，標誌有CD34陽性之細胞較正常人為高。病人對於光化學治療的反應良好。
關鍵字
未明確細胞組織球增生症並列摘要
Indeterminate cell histiocytosis is a rare neoplasm composed of cells with mixed characteristics of Langerhans cells and non-Langerhans cells. An otherwise healthy, 36-year-old woman presented with asymptomatic generalized papules and nodules that had appeared on all four extremities, the trunk, and cheeks in the previous 6 months. The lesions were firm, painless, non-pruritic, and slightly flesh-yellow or reddish-brown in color. Histopathologic, immunohistochemical examination and electron microscopic studies showed characteristic findings of indeterminate cell histiocytosis: diffuse proliferative histiocytes infiltrating the dermis without epidermotropism or atypia; neoplastic cells expressing markers characteristic of both Langerhans cells (CD1a, S-100) and focal monocytes / macrophages (Factor XIIIa, CD68); and no Birbeck granules within the cytoplasm of the neoplastic cells. Flow cytometry revealed more CD34+ cells in the peripheral blood of the patient than in peripheral blood from a control. Interestingly, the patient responded favorably to psoralen ultraviolet A-range treatment. Herein, we present this case and review the literature.