Wollina U, Hahnfeld S, Kosmehl H
Department of Dermatology, Friedrich-Schiller-University Jena, Germany.
J Cancer Res Clin Oncol. 1999;125(5):305-8. doi: 10.1007/s004320050278.
Marginal zone lymphoma (MZL) is a distinct entity among B-cell lymphomas. We report on a 53-year-old woman who developed disseminated primary cutaneous MZL with secondary lymph node involvement and perinodular spreading. The tumor cell phenotype was characterized as CD20/CD79a/kappa/lambda+/ bcl-2-positive, CD3/5/15/39/bcl-1-negative. Ki-67 was expressed by 20-35% of tumor cells. There was no evidence of systemic (including bone marrow) involvement. The diagnosis of MZL with plasmacellular differentiation (Stage IVa) was made. The patient was treated with interferon alpha2a injected s.c. at 9x10(6) U 3 days a week for 1 year. During this time the skin lesions completely disappeared. No evidence of lymph node or extracutaneous disease was found. The patient remains in complete remission. Side effects were only of grade I (WHO); the Karnovsky index was 90%. As shown for other types of primary cutaneous B-cell lymphoma, prolonged interferon alpha monotherapy may be effective in controlling the disease and/or inducing complete remission in MZL.
边缘区淋巴瘤（MZL）是B细胞淋巴瘤中的一种独特类型。我们报告了一名53岁女性，她患播散性原发性皮肤MZL并伴有继发性淋巴结受累及结节周围扩散。肿瘤细胞表型特征为CD20/CD79a/κ/λ+/bcl-2阳性，CD3/5/15/39/bcl-1阴性。20%-35%的肿瘤细胞表达Ki-67。无全身（包括骨髓）受累证据。诊断为伴有浆细胞分化的MZL（IVa期）。患者接受皮下注射干扰素α2a治疗，剂量为9×10⁶ U，每周3天，共1年。在此期间，皮肤病变完全消失。未发现淋巴结或皮肤外疾病证据。患者仍处于完全缓解状态。副作用仅为I级（世界卫生组织标准）；卡诺夫斯基指数为90%。正如其他类型的原发性皮肤B细胞淋巴瘤所示，延长干扰素α单药治疗可能对控制MZL疾病和/或诱导完全缓解有效。
