Huang Yenlin, Moreau Anne, Dupuis Jehan, Streubel Berthold, Petit Barbara, Le Gouill Steven, Martin-Garcia Nadine, Copie-Bergman Christiane, Gaillard Fanny, Qubaja Marwan, Fabiani Bettina, Roncador Giovanna, Haioun Corinne, Delfau-Larue Marie-Hélène, Marafioti Teresa, Chott Andreas, Gaulard Philippe
INSERM, Unité 955, Equipe 9, France.
Am J Surg Pathol. 2009 May;33(5):682-90. doi: 10.1097/PAS.0b013e3181971591.
Rare cases of peripheral T-cell lymphomas with follicular growth pattern (PTCL-F) have been recently reported, and their association with t(5;9)(q33;q22) involving ITK and SYK has been suggested. However, the clinicopathologic aspects of PTCL-F are poorly described and the normal cell counterpart of this subgroup of lymphoma is still unknown. Therefore, we analyzed the pathologic, phenotypic, and cytogenetic features of a series of 30 patients (range: 33 to 88 y) that showed histopathologic features of PTCL-F in at least 1 biopsy (n=30), either at initial presentation (n=26) or at relapse (n=4). Neoplastic cells were medium-sized clear cells that were CD4+ (24/27, 89%), CD10+ (21/29, 72%), BCL-6+ (14/19, 74%), and expressed programed death-1 (27/27, 100%), CXCL13 (23/27, 85%), and ICOS (11/11, 100%), markers of follicular helper T cells (TFH). Four of 22 patients (18%) had t(5;9)(q33;q22) detected by fluorescence in situ hybridization. Patients with clinical data available had multiple lymphadenopathies (25/28, 89%), stage III to IV diseases (17/26, 65%), B symptoms (7/27, 26%), and skin lesions (6/23, 26%). Three patients with sequential biopsies disclosed clinical and histopathologic features of angioimmunoblastic T-cell lymphoma at initial presentation. Our results show that this rare form of PTCL-F (1) has an immunophenotype indicative of derivation from TFH cells, (2) is associated with t(5;9) in a proportion of cases, and (3) shows some overlapping features with angioimmunoblastic T-cell lymphoma, raising the question of a possible relationship.
近期有外周T细胞淋巴瘤伴滤泡生长模式（PTCL-F）的罕见病例报道，且提示其与涉及ITK和SYK的t(5;9)(q33;q22)有关。然而，PTCL-F的临床病理特征描述较少，该淋巴瘤亚组的正常细胞对应物仍不清楚。因此，我们分析了30例患者（年龄范围：33至88岁）的病理、表型和细胞遗传学特征，这些患者在至少1次活检（n = 30）中表现出PTCL-F的组织病理学特征，其中26例为初诊时发现，4例为复发时发现。肿瘤细胞为中等大小的透明细胞，CD4+(24/27, 89%)、CD10+(21/29, 72%)、BCL-6+(14/19, 74%)，并表达程序性死亡-1(27/27, 100%)、CXCL13(23/27, 85%)和ICOS(11/11, 100%)，这些均为滤泡辅助性T细胞（TFH）的标志物。22例患者中有4例（18%）通过荧光原位杂交检测到t(5;9)(q33;q22)。有临床资料的患者有多处淋巴结肿大（25/28, 89%）、Ⅲ至Ⅳ期疾病（17/26, 65%）、B症状（7/27, 26%）和皮肤病变（6/23, 26%）。3例接受序贯活检的患者在初诊时表现出血管免疫母细胞性T细胞淋巴瘤的临床和组织病理学特征。我们的结果表明，这种罕见形式的PTCL-F：（1）具有提示起源于TFH细胞的免疫表型；（2）部分病例与t(5;9)有关；（3）与血管免疫母细胞性T细胞淋巴瘤有一些重叠特征，这就引发了两者可能存在关系的问题。
