Kunisada M, Adachi A, Matsumoto S, Ogawa Y, Horikawa T, Iwatsuki K
Department of Dermatology, Hyogo Prefectural Kakogawa Hospital, Kakogawa, Japan.
Int J Dermatol. 2003 Sep;42(9):710-4. doi: 10.1046/j.1365-4362.2003.01757.x.
We report a case of an Epstein-Barr virus (EBV)-associated nasal-type natural killer cell lymphoma (NKCL) preceded by benign panniculitis, which arose in a 48-year-old woman with an asymptomatic human T-cell leukemia/lymphoma virus type-1 (HTLV-1) infection. A biopsy of the initial panniculitis lesion demonstrated lobular panniculitis with a germinal center composed of benign mononuclear cells with a phenotype of CD4+CD45RO+CD5sCD3+ cCD3 epsilon + T-cell intracellular antigen-1 (TIA-1)- and granzyme B-. One year after oral prednisolone therapy, the patient developed subcutaneous nodules composed of atypical lymphoid cells with a phenotype of CD4-CD45RO+CD56+sCD3-cCD3 epsilon + (TIA-1)+ and granzyme B+. In the initial panniculitis lesion, neither EBV-encoded RNA (EBER-1) nor clonal proliferation of EBV-infected cells was identified. In later lesions, however, a large number of atypical cells were positive for EBER-1, and a clonal expansion of EBV-infected cells was detected. No clonal rearrangement of T-cell receptor-alpha, -beta, or -gamma genes was found in either specimen. This patient was an asymptomatic carrier of human T-cell leukemia/lymphoma virus type-1 (HTLV-1) without clonal integration of proviral HTLV-1 in neither the peripheral blood nor the skin lesions. These observations suggest that EBV-associated NKCL occurred subsequently in the clinical course of benign panniculitis under the influence of immunosuppression caused by prednisolone treatment and HTLV-1 infection.
我们报告一例在良性脂膜炎后发生的与爱泼斯坦-巴尔病毒(EBV)相关的鼻型自然杀伤细胞淋巴瘤(NKCL),该病例发生在一名48岁无症状感染1型人类T细胞白血病/淋巴瘤病毒(HTLV-1)的女性身上。最初脂膜炎病变的活检显示小叶性脂膜炎,其生发中心由具有CD4+CD45RO+CD5sCD3+cCD3ε+T细胞细胞内抗原-1(TIA-1)-和颗粒酶B-表型的良性单核细胞组成。口服泼尼松龙治疗一年后,患者出现由非典型淋巴细胞组成的皮下结节,其表型为CD4-CD45RO+CD56+sCD3-cCD3ε+(TIA-1)+和颗粒酶B+。在最初的脂膜炎病变中,未发现EBV编码的RNA(EBER-1)或EBV感染细胞的克隆增殖。然而,在后来的病变中,大量非典型细胞EBER-1呈阳性,并且检测到EBV感染细胞的克隆扩增。在两个标本中均未发现T细胞受体α、β或γ基因的克隆重排。该患者是1型人类T细胞白血病/淋巴瘤病毒(HTLV-1)的无症状携带者,在外周血和皮肤病变中均未发现前病毒HTLV-1的克隆整合。这些观察结果表明,在泼尼松龙治疗和HTLV-1感染引起的免疫抑制影响下,EBV相关的NKCL在良性脂膜炎的临床过程中随后发生。