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卡波西氏肉瘤-成大皮膚部11年間(1988至1998)二十二病例研究-

Kaposi's Sarcoma -A Review of 22 Cases in NCKUH from 1988 to 1998-

摘要


卡波西氏肉瘤(Kaposi’s sarcoma,以下簡稱KS)在台灣已不算罕見的疾病,但目前為止國內僅有數篇病例報告,並沒有較大系列的臨床報告。因我們彙整成大醫院自1988年到1998年這十一年內經病理證實為KS的病例共22個,採回顧性研究分別就流行病學、臨床表現、治療及預後四部分來討論。本研究的22位KS病人,包括男性20人,女性2人,年齡分佈為25-80歲。臨床類型包括傳統型9人,醫源性免疫抑制有關型7人,AIDS相關之流行型6人。22例中以聚合酵素鍊反應偵測HHV-8核酸序列者計10例,各亞型皆有,反應全為陽性。屬免疫抑制有關型的7人,其CD4淋巴球數目並不比傳統病人之CD4淋巴球數目少,但臨床病灶分佈範圍似乎較為廣泛(除了四肢之外,也會侵犯至軀幹)。這類病人中有4位病人長期服用中藥、黑藥丸,其中有3位併有嚴重的全身性皮癬菌感染。與non-AIDS KS比較起來,ADIS-KS患者有以下之特色:發病年齡較輕(38.5歲vs 64.1歲),皮膚病灶分佈較廣泛(100% vs 44%),易侵犯頭頸部(83% vs 0%)及口腔黏膜(67% vs0%),發病時CD4淋巴球數目較低(16.2/mm vs 431.3/mm),治療反應較差。KS侵犯皮膚以外部位包括口腔黏膜4例,肺部2例,及腸胃道1例,皆是AIDS病患。治療上,病灶分佈範圍較侷限性時,採用局部性治療,一般反應都良好,反之則用全身性治療。2位non-AIDS KS和1位AIDS-KS以paclitaxel(Taxol)治療,效果顯著。

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並列摘要


Kaposi’s sarcoma(KS) is no longer considered a rare disease in Taiwan, there have been only scattered case reports. In this retrospective study, we review all patients with pathology-proven KS diagnosed at Department of Dermatology, National Cheng Kung University Hospital from 1988to 1998, and report the epidemiology, clinical presentation, treatment and prognosis of this malignancy. The present series consists of 22 patients of KS, including 20 males and 2 females, aged between 25 and 80 years. There were 9 classic KS, 7 iatrogenic immunosuppression-related KS, and 6 AIDS-related KS. KS from ten of the 22 patients were analyzed for the presence of HHV-8 sequence by polymerase chain reaction. The results revealed that all 10 cases of KS, including all 3 types, were positive for the HHV-8.Of the iatrogenic group, 4 had history of chronic herb drug (often containing corticosteroids) use which constitutes a common form of steroid abuse in Taiwan. Three of these 4 patients manifested generalized dermatophytosis. Compared with classical KS, KS in these group has higher tendency to involve truncal parts, although their CD4+ lymphocyte counts were still within normal range. Compared with the non-AIDS KS groups, AIDS-KS group showed younger age at onset (38.5 yr vs 64.1 yr),more widespread skin lesions (100% vs 44%), and frequent involvement of the head and neck (83% vs 0%), and oral mucosa(67% vs 0%),lower CD4+ lymphocyte counts (16/mm3 vs 431/mm3), and poorer response to treatment. Extracutaneous lesions were only seen in AIDS patients, involving oral mucosa in 4 patients, pulmonary in 2 and GI tract in 1. In general, limited disease in the present series responded to local treatment while generalized or aggressive disease required systemic treatment. In 2 non-AIDS patients and 1AIDS patient, chemotherapy with paclitaxel (taxol) was effective.

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