摘要
目的分析种痘水疱病样淋巴瘤3例患儿特点,探讨该病的诊断与治疗,提高临床医生对其认识。方法总结2007年11月至2011年10月中南大学湘雅医院诊治的3例种痘水疱病样淋巴瘤患儿的临床表现、实验室检查、诊治经过,并结合相关文献进行分析。结果 (1)主要临床表现为多形性皮疹、间歇性发热、肝脾淋巴结肿大及凹陷性水肿。(2)实验室检查:3例患儿血象分类均以淋巴细胞为主,2例免疫组化示CD45RO(+)、CD56(+)、TIA-1(+)、Ki67(>30%),1例免疫组化示CD3ε(+)、CD56(+)、TIA-1(+)、Ki67(>30%)、EBER(原位杂交)(+)。皮肤活检:2例示皮肤种痘水疱病样T细胞淋巴瘤,1例示皮肤T细胞非霍奇金恶性淋巴瘤,倾向外周T细胞性。(3)治疗及预后:病例1放弃治疗;病例2丙种球蛋白及激素治疗2周后皮疹明显改善,予以化疗(柔红霉素+门冬酰胺酶+地塞米松+长春新碱),骨髓抑制期后因消化道出血死亡;病例3因侵犯多器官致全身衰竭死亡。结论种痘水疱病样淋巴瘤确诊主要依据皮损部位组织病理活检及免疫组化,发病与EB病毒感染有关。不同疗法的治疗反应存在个体差异,应综合患儿对治疗的反应及其他因素,采取个性化治疗。本病病情进展迅速,恶性程度高,预后差。
Objective By analyzing the oharacteristics of hydroa vacciniforme-like lymphoma in children, to discuss the diagnosis and treatment of the disease and to improve of awareness of doctors about the hydroa vacciniforme-like lympho- ma. Methods The clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in 3 children with hydroa vacciniforme-like lymphoma were summerized and analyzed with literature review. Results （1） The main manifestations were cutaneous rash, intermittent fever, hepatosplenomegaly/lymphadenectasis and pit- ting edema. （2）Laboratory examinations：the peripheral hemogram revealed moderate elevation of lymphocytes. Immuno- histochemisty of 2 cases were proved CD45RO （＋）, CD56 （＋）, TIA-1 （＋） and Ki67 （〉30%）. One cases showed CD3 e （＋）, CD56 （＋）, TIA- 1 （＋）, Ki67 （〉30%） and EBER （＋）. Two of 3 cases in skin biopsy displayed the result of hydra vac- ciniforme-like lymphoma, and one case was the cutaneous T ceils non-hodgkin＇s malignant lymphoma, tending to periph- eral T cell. （3）Therapy and prognosis：case 1 gave up treatment. After c-type globulin and hormone therapy for 2 weeks, the rash of case 2 obviously improved. Then given chemotherapy（Daunorubicin ＋ Asparaginase ＋ Dexamethasone ＋ Vin- cristine）, the child had gastrointestinal bleeding and died after bone marrow suppression period. Case 3 died of systemic exhaustion. Conclusion Hydroa vacciniforme-like lymphoma is identified by the main skin biopsy of affected tissue and immunohistochenfistry assay ; EBV infection may be related to the development of the disease. The reaction of differ- ent treatments has individual difference. The children should be given personalized treatment by comprehensively consid- ering their response to the treatment and other factors. In a word, this disease has high malignant degree and bad prognosis.
出处
《中国实用儿科杂志》
CSCD
北大核心
2013年第8期606-609,共4页
Chinese Journal of Practical Pediatrics
关键词
种痘水疱病样
淋巴瘤
EB病毒
儿童
hydroa vacciniforme-like
lymphoma
Epstein-Barr virus
child
