Gammon Bryan, Guitart Joan
Department of Dermatology, Northwestern University, Chicago, Illinois 60611, USA.
Arch Dermatol. 2012 Sep;148(9):1040-4. doi: 10.1001/archdermatol.2012.1545.
Follicular helper T cells are a subset of helper T cells that facilitate B-cell recruitment and maturation. Rare cases of cutaneous T-cell lymphoma manifesting as de novo tumor lesions in intertriginous skin contain an infiltrate rich in B cells. These cases may represent malignant counterparts of skin-homing follicular helper T cells.
Two men and 1 woman (age range, 35-58 years) were seen with predominantly intertriginous tumor-stage cutaneous T-cell lymphoma lesions characterized by the absence of epidermotropism and the presence of a mixed infiltrate rich in B cells. Two of the patients died of the disease less than 3 years from the initial diagnosis. The surviving patient has aggressive disease and underwent hematopoietic stem cell transplantation. Two of the patients had a prominent CXCL13+, Bcl6/CD3+, and programmed death protein 1-positive follicular helper T-cell population.
The intertriginous tumor variant of cutaneous T-cell lymphoma is heterogeneous but may be associated in some cases with a follicular helper T-cell immunophenotype. These patients may follow an aggressive clinical course. Tumor progression in sanctuary sites on patients receiving phototherapy may manifest as a similar clinical phenotype. Further characterization of the disease process is needed to confirm this observation.
滤泡辅助性T细胞是辅助性T细胞的一个亚群，可促进B细胞募集和成熟。罕见的皮肤T细胞淋巴瘤病例表现为间擦部位皮肤出现原发性肿瘤病变，其中含有丰富的B细胞浸润。这些病例可能代表皮肤归巢滤泡辅助性T细胞的恶性对应物。
我们观察到2名男性和1名女性（年龄范围35 - 58岁），主要表现为间擦部位肿瘤期皮肤T细胞淋巴瘤病变，其特征为无亲表皮性且存在富含B细胞的混合浸润。2例患者在初次诊断后不到3年死于该病。幸存患者患有侵袭性疾病并接受了造血干细胞移植。2例患者有显著的CXCL13 +、Bcl6/CD3 +和程序性死亡蛋白1阳性的滤泡辅助性T细胞群体。
皮肤T细胞淋巴瘤的间擦部位肿瘤变体具有异质性，但在某些情况下可能与滤泡辅助性T细胞免疫表型相关。这些患者可能有侵袭性临床病程。接受光疗患者的免疫赦免部位肿瘤进展可能表现为类似的临床表型。需要对疾病过程进行进一步表征以证实这一观察结果。
