- 期刊
被套區細胞淋巴瘤－看似不怎麼惡性，其實難纏的很，兼述非何杰金氏淋巴瘤的新進分類
Mantle Cell Lymphoma, It Appears Indolent but Actually Aggressive, also Summary the Classification of Non-Hodgkin's Lymphoma
摘要
被套區細胞淋巴瘤(Mantle cell lymphoma, MCL)是在九十年代後才有足夠證據確立爲非何杰金氏淋巴瘤(Non-Hodgkin's lymphoma)的一個亞型。在此之前多半歸類在瀰漫性淋巴球或小裂核細胞淋巴瘤裡。因此乍看之下並非很惡性，但臨床表現接近中度惡性的一群，但治療結果卻遠不如它們。在沒有治療的情形下，其中間存活期遠低於其他之低度惡性淋巴瘤；而傳統治療的效果，其無病存活率又遠低於其他中度惡性淋巴瘤（像瀰漫性大B細胞淋巴瘤）的一群，且無法痊癒。MCL的免疫表徵爲Cyclin D1陽性，此乃因爲染色體11及14之異位【t(11:14)】所致。其泛B抗原CD19、CD20及CD22爲陽性，但CD23則爲陰性。CD10(CALLA)通常爲陰性，但泛T抗原CDS反而爲陽性。臨床上，好發於男性，中間發生年齡爲58歲。理學特徵爲全身淋巴腫脹(71%-90%)，肝脾腫大(35%-55%)及骨髓的侵犯(53%-90%)，如果刻意去做腸胃檢查，會發現幾近百分之百的侵犯率。預後分析以被套細區胞淋巴瘤國際預後指標(MIPI)爲佳。傳統之治療無法痊癒MCL，因此在身體狀況許可下，在第一次完全緩解之後，應考慮幹細胞移植。根據追溯性的分析，自體移植與異體（近親、配對完全）移植的五年存活率沒有統計上的差異(47%VS49%)。
關鍵字
被套區細胞淋巴瘤並列摘要
Mantle cell lymphoma (MCL) is recognized ”with evidence” as a subtype of non-Hodgkin's lymphoma in early 1990s. It was categorized mostly in the group of diffuse small lymphocytic/small cleaved cell lymphomas before this recognition. Therefore, it might not seem so aggressive morphologically. But in fact, it behaved very differently from other diseases with similar morphology. The median survival without treatment is much shorter than other kinds of low grade lymphoma. When it is treated conventionally, the failure free survival is much shorter as compaired to aggressive lymphomas (e.g. diffuse large B cell lymphoma) and there is no cure at all. Genetic and immunophenotypic features are characterized by overexpression of Cyclin D1 secondary to translocation of t(11;14) (q13:q32). The pan B-antigens, CD19, CD20, CD22 are positive but CD23 is negative. CD10 (CALLA) is usually negative and coexpression of pan T antigen CD5 and its median age of occurrence is 58. It has male predominance clinically. The common signs of presentation are generalized lymphadenopathies (71%-90%), hepatosplenomegaly and bone marrow involvement (53%-90%). Involvement of G-I tract is strikingly high in nearly all cases. The prognostic evaluation by multivariate analysis showed the new mantle cell lymphoma international prognostic index (MIPI) was related to survival and worked better. MCL is incurable with conventional chemotherapy. So stem cell transplant should be considered on first remission on a medical fit patient. From retrospective reviews, there was no statistically significant difference in 5 year survival rates compared the autologous (47%) to allogeneic (49%) transplants.