摘要
目的归纳、分析种痘水疱病样皮肤T细胞淋巴瘤患者的临床、组织病理特点及治疗和预后,以提高临床医生对该病的诊治水平。方法分析3例患者的皮疹特点、伴发症状、辅助检查、组织病理、治疗及预后等临床资料,并复习相关文献。结果患者2男1女,发病年龄最早3岁,最晚10岁。皮损具有多形性,发作时伴高热、淋巴结及肝脾肿大。组织病理可见真皮至皮下脂肪层内的异型性淋巴样细胞围绕血管浸润,有明显的侵犯血管壁和脂肪小叶现象,伴有较多的EOS浸润;免疫组化示肿瘤细胞表达CD8,CD45RO,CD43,而不表达CD20,CD30,CD56。皮损组织基因重排检查TCR-γ(+)。Epstein-Barr病毒原位杂交(+)。外周血EOS明显升高,LDH酶稍高,EB病毒抗体(+)。例1行化疗,后死亡;例2给予强的松口服,例3接受防蚊子叮咬及抗病毒治疗,均长期缓解。结论种痘水疱病样皮肤T细胞淋巴瘤是一种新的恶性多系统疾病,发病可能与EBV感染以及蚊子叮咬有关。不同的患者恶性程度不一,治疗及预后亦不同。
Objective The purpose of this study was to describe 3 cases of hydroa vacciniforme - like T cell lymphoma, to discuss its clinical, histopathologic, and immunohistochemical features. Methods 3 patients with the disorder from PUMC hospital were enrolled in the study. The clinical and histiopathological features were analyzed. Results All the patients were children, 1 female and 2 were male. Skin lesions presented with polymorphous lesions accompanied with high fever and hepatospleno- megaly. Histopathological findings revealed that diffuse and dense infiltrate of lymphoid cells with anasplastic nuclei in the middle and low potion of the dermis as well as the subcutis;Noeplastic cells invaded blood vessels and subcutaneous fat, with an obviously angiocentric pattern. Moderate numbers of eosinophils were also present in the dermal infiltrates. The heteromorphic cells were s positive for CD8, CD45Ro and CD43, but negative for CD20,CD30 and CD56. The EBV serological tests showed positivity. The number of eosinophils, IgE and serum lactic dehydrogenase in the blood were elevated obviously. Monoclonal gene rearrangement of TCR -γ was found The EBER was detected by in situ hybridization. Conclusion Hydroa vacciniforme - like Cutaneous T cell lymphoma is a new kind of T cell lymphoma. This disorder may be related to the Epstein -Barr virus （EBV） infection and mos- quito bites. Different patients need different treatments and the prognoses are distinct.
出处
《中国皮肤性病学杂志》
CAS
北大核心
2008年第6期341-344,共4页
The Chinese Journal of Dermatovenereology
