Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection
Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection
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DOI：
10.1111/j.1365-2141.2004.04993.x
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发表时间：
2004-07-01
影响因子：
6.5
通讯作者：
Pizzolo, G
中科院分区：
文献类型：
--
作者：
Ambrosetti, A;Zanotti, R;Pizzolo, G
唾液腺粘膜相关淋巴组织（MALT）淋巴瘤（SGML）是罕见的，因为是关于他们的行为的数据。我们分析了1985年3月至2003年4月诊断的33例SGML的临床特征，特别是与舍格伦综合征（SS）和丙型肝炎病毒（HCV）感染的相关性。有5名男性和28名女性，中位年龄为61岁。在介绍，12/33（36%）有多个唾液腺或粘膜受累，4骨髓浸润。安阿伯分期为IE 15例（46%），IIE 4例（12%），IV 14例（42%）。15例患者有SS病史（46%），2例其他自身免疫性疾病，7例HCV感染。无一例同时感染SS和HCV。在29例接受治疗的患者中，17例接受手术或局部放疗; 69%达到完全缓解。4例（12%）发生组织学转化。5例患者死亡（3例淋巴瘤，2例不相关原因）。5年总生存率（OS）、病因特异性生存率和无进展生存率分别为85 +/-8%、94 +/- 6%和65 +/- 10%。总体而言，尽管诊断时已处于晚期，但病程缓慢，局部治疗通常似乎足够。影响OS的唯一预后因素是组织学转化和年龄。在我们的研究中，SGML与自身免疫性疾病或HCV感染的密切相关性（73%）证实了它们在这些淋巴瘤发病机制中的可能作用。
Salivary gland mucosa-associated lymphoid tissue (MALT) lymphomas (SGML) are rare, as are data concerning their behaviour. We analysed clinical features at presentation, particularly the association with Sjoegren syndrome (SS) and hepatitis C virus (HCV) infection, and outcome in 33 cases of SGML diagnosed between March 1985 and April 2003. There were five males and 28 females, with a median age of 61 years. At presentation, 12/33 (36%) had multiple salivary glands or mucosal involvement and four had bone marrow infiltration. Ann Arbor stage was IE in 15 (46%), IIE in four (12%) and IV in 14 patients (42%). Fifteen patients had a history of SS (46%), two of other autoimmune diseases, seven of HCV infection. No case had both SS and HCV. Of the 29 treated patients, 17 received surgery or local radiotherapy; 69% achieved complete remission. Histological transformation occurred in four (12%). Five patients died (three of lymphoma, two of unrelated causes). The 5 year-overall survival (OS), cause-specific survival and progression-free survival was 85 +/- 8%, 94 +/- 6% and 65 +/- 10% respectively. Overall, the disease course was indolent, despite the advanced stage at diagnosis, and local therapy often appeared to be adequate. The only prognostic factors influencing OS were histological transformation and age. The close association of SGML with either autoimmune diseases or HCV infection in our series (73%) confirms their possible role in the pathogenesis of these lymphomas.