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原发性胃T细胞淋巴瘤:两例报告并文献复习

Primary gastric T-cell lymphomas: report of two cases and a review of the literature.

作者信息

Horie R, Yatomi Y, Wakabayashi T, Ohno Y, Eriguchi M, Higashihara M, Nakahara K, Watanabe T

机构信息

Department of Pathology, Institute of Medical Science, University of Tokyo, Japan.

出版信息

Jpn J Clin Oncol. 1999 Mar;29(3):171-8. doi: 10.1093/jjco/29.3.171.

Abstract

To understand more fully the clinicopathological features of primary gastric T-cell lymphomas (PGTL), we report two cases of PGTL and review the literature. The present cases were not associated with human T-cell leukemia virus type 1 (HTLV-1) and were at clinical stage IIE. In both cases, T-cell origin of the lymphoma cells was diagnosed immunohistochemically. The clinical courses of these two cases were different: one followed a very aggressive clinical course and the patient died 6 months after the diagnosis, whereas the other patient survived more than 2 years without adjuvant chemotherapy. Clinicopathological features of 23 patients with PGTL are summarized with regard to their differences from primary small intestinal T-cell lymphomas (PSITL) and by association with HTLV-1. The median age at onset of PGTL was 58 years. The gender ratio was male-dominant (M:F = 2.3:1). About two-thirds (10 of 17) of PGTL cases had evidence of HTLV-1 infection. The most common presenting symptom for PGTL was upper abdominal discomfort and/or pain (76%), whereas that in PSITL was weight loss (61%) and diarrhea (42%). Typical lesions for PGTL were large ulcerations at the corpus to antrum. Neoplastic cells had no typical morphological characteristics for PGTL including HTLV-1-associated cases. CD3+4+8- was the most frequently observed surface phenotype of PGTL cells. Laboratory findings at diagnosis were not informative. Most patients were treated by gastrectomy with or without chemotherapy. PGTL, excluding that with HTLV-1, showed better prognosis than PSITL, although PGTL with HTLV-1 had a poorer prognosis.

摘要

为更全面地了解原发性胃T细胞淋巴瘤(PGTL)的临床病理特征,我们报告2例PGTL病例并复习相关文献。目前这2例病例均与1型人类T细胞白血病病毒(HTLV-1)无关,临床分期为IIE期。2例病例均通过免疫组化诊断为淋巴瘤细胞起源于T细胞。这2例病例的临床病程不同:1例临床病程非常凶险,诊断后6个月死亡,而另1例患者未接受辅助化疗存活超过2年。总结了23例PGTL患者的临床病理特征,包括其与原发性小肠T细胞淋巴瘤(PSITL)的差异以及与HTLV-1的相关性。PGTL发病的中位年龄为58岁。性别比以男性为主(男:女 = 2.3:1)。约三分之二(17例中的10例)的PGTL病例有HTLV-1感染证据。PGTL最常见的首发症状是上腹部不适和/或疼痛(76%),而PSITL的首发症状是体重减轻(61%)和腹泻(42%)。PGTL的典型病变是胃体至胃窦的大溃疡。肿瘤细胞没有PGTL的典型形态特征,包括与HTLV-1相关的病例。CD3+4+8-是PGTL细胞最常观察到的表面表型。诊断时的实验室检查结果无诊断意义。大多数患者接受了胃切除术,部分患者接受了化疗。排除与HTLV-1相关的PGTL,其预后比PSITL好,尽管与HTLV-1相关的PGTL预后较差。

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