Luo Yang, Zhang L I, Sun Yu-Jiao, DU Hua, Yang Gui-Lan
Department of Dermatology, Lanzhou General Hospital of Lanzhou Military Region, Lanzhou, Gansu 730050, P.R. China.
Exp Ther Med. 2016 Jun;11(6):2254-2258. doi: 10.3892/etm.2016.3183. Epub 2016 Mar 24.
Mycosis fungoides (MF), a low-malignant lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. The current study reported a case of syringotropic MF, a rare variant of MF, which presented with reactive B cell proliferation, lymphoid follicle formation, hair loss and lymphadenopathy. The clinical manifestations of the patient were MF-like lumps. Immunohistochemical staining of AE1/AE3 showed that there were abundant infiltrated lymphocytes surrounding the syringocystadenoma. In addition, the direction of the lymphocyte arrangement was consistent with the meandering direction of syringocystadenoma. The patient did not respond to 1-month narrowband (311-nm) ultraviolet therapy; however, a good response was obtained subsequent to one cycle of chemotherapy with vincristine sulfate, etoposide, L-asparaginase and prednisone acetate (know as the VELP regimen). After 7 days of VELP chemotherapy, the skin lesions were ameliorated, hair loss was improved and lymphadenopathy disappeared. No lymphadenopathy or new skin lesions were observed during 6 months of follow-up.
蕈样肉芽肿（MF）是一种低恶性淋巴增殖性疾病，是最常见的皮肤T细胞淋巴瘤类型。当前研究报告了1例向汗腺性MF，这是MF的一种罕见变异型，表现为反应性B细胞增殖、淋巴滤泡形成、脱发和淋巴结病。该患者的临床表现为MF样肿块。AE1/AE3免疫组化染色显示，汗腺囊腺瘤周围有大量浸润淋巴细胞。此外，淋巴细胞排列方向与汗腺囊腺瘤的迂曲方向一致。该患者对1个月的窄谱（311nm）紫外线治疗无反应；然而，在接受1个周期的硫酸长春新碱、依托泊苷、L-天冬酰胺酶和醋酸泼尼松（即VELP方案）化疗后取得了良好疗效。VELP化疗7天后，皮肤病变改善，脱发好转，淋巴结病消失。随访6个月期间未观察到淋巴结病或新的皮肤病变。
