樹(shù)突狀細(xì)胞在組織中大量積累為特征的腫瘤屬性罕見(jiàn)疾病,發(fā)病高峰年齡為1\~4歲,發(fā)病率為(4\~6)/100萬(wàn),成人發(fā)病率為(1\~2)/100萬(wàn)。由于其發(fā)病率低,臨床表現(xiàn)多種多樣,影像學(xué)表現(xiàn)無(wú)明顯特異性,為該類(lèi)腫瘤的明確診斷及早期治療帶來(lái)挑戰(zhàn)。本文報(bào)道8例骨朗格漢斯組織細(xì)胞增生癥,同時(shí)回顧近5年國(guó)內(nèi)外相關(guān)文獻(xiàn),對(duì)骨朗格漢斯組織細(xì)胞增生癥的臨床特點(diǎn)、病理特征、診斷、治療及預(yù)后進(jìn)行總結(jié)。-龍?jiān)雌诳W(wǎng)" />

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骨朗格漢斯組織細(xì)胞增生癥八例并文獻(xiàn)復(fù)習(xí)

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關(guān)鍵詞:骨朗格漢斯組織細(xì)胞增生癥;診斷;治療;免疫組織化學(xué)

中圖分類(lèi)號(hào):R365;R738.1 文獻(xiàn)標(biāo)識(shí)碼:B 文章編號(hào):1000-503X(2025)02-0325-08

DOI:10.3881/j.issn.1000-503X.16077

Langerhans Cell Histiocytosis of Bone: Report of Eight Cases and Review of the Literature

BI Ya,WU Dandan,YU Fangying,F(xiàn)ANG Zhenhong,HUANG Bo

DepartmentofRespiratoryndCriticalCareMedicine,AflitedHospitalofZunyiMedicalUniversity,Zunyi,Guizhou5630,China

Corresponding author:HUANG Bo Tel:0851-28609032,E-mail:13985229382@139.com

ABSTRACT:Langerhans cell histiocytosis of bone is a rare tumor disease characterized by the large accumulation of CD1a+ and CD207 + dendritic cells in tissues of unknown cause. It mainly occurs in children aged 1-4 years old,with incidences of4-6 per million in children and1-2 per million inadults.Due to its low incidence, diverse clinical manifestations,and noobvious specificityof imaging manifestations,the definitive diagnosis and early treatment of this type of tumor are challenging. In this paper,we report8 cases ofLangerhans cellhistiocytosis of boneand review therelevantliterature published inthe past five years to summarizethe clinical characteristics,pathological features,diagnosis,treatment,and prognosis of this disease.

Key words:Langerhans cell histiocytosis of bone;diagnosis;treatment; immunohistochemistry

Acta Acad Med Sin,2025,47(2):325-332

朗格漢斯組織細(xì)胞增生癥(Langerhanscellhistio-cytosis,LCH)是一種以朗格漢斯細(xì)胞增生為特征的一組病變實(shí)體,是一種介于良、惡性之間的組織細(xì)胞增殖性疾病[1]。(剩余13927字)

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