以皮肤多形红斑为表现的慢性NK细胞淋巴增殖性疾病1例国内首报  

Cutaneous Erythema Multiforme Associated with Chronic Natural Killer Lymphoproliferative Disorders: the First Case Reported in China

作　　者：齐思思[1] 赵颖[1] 朱敏[1] 杨勤萍[1] 

机构地区：[1]复旦大学附属华山医院,上海200040

出　　处：《中国皮肤性病学杂志》2016年第2期114-117,共4页The Chinese Journal of Dermatovenereology

摘　　要：目的提高皮肤科医生对慢性NK细胞淋巴增殖性疾病(chronic natural killer lymphoproliferative disorders,NK-CLPD)的认识,总结其临床特点、诊断及治疗。方法国内首次报道1例以皮肤多形红斑样皮疹为表现的NK-CLPD,分析其临床表现、组织病理,并复习国内外相关文献。结果患者为中青年女性,发病11个月,临床表现为发热及全身泛发性多形红斑样皮疹,根据病程、皮损组织病理学检查、血象、流式细胞术免疫分型结果、骨髓穿刺及病毒学检验可明确NK-CLPD诊断,治疗主要以糖皮质激素为主,患者对激素治疗效果好。结论 NK-CLPD是罕见的NK细胞疾病,以外周血成熟NK细胞慢性扩增为特点。外周血和骨髓可见到大颗粒淋巴细胞,典型免疫表型为CD2^+,sCD3^-,CD16^+,弱表达CD56。以多形红斑样皮疹为表现的NK-CLPD国内外尚未见报道。该病呈惰性病程,部分患者预后较好,但有转变为侵袭性NK细胞白血病的可能。Objective To investigate the clinical manifestations, diagnosis and treatment of chronic natural killer lymphoproliferative disorders （NK-CLPD） , and to raise awareness of the disease. Methods A case of cutaneous erythema multiforme associated with NK-CLPD was reported. The clinical data and histopathogical features of this case were analyzed. The relevant literatures were reviewed while the discussions were conducted. Results The middle aged woman was diagnosed with NK-CLPD by duration （ 11 months）, clinical manifestations, complete blood count, immunophenotype of peripheral blood, bone marrow biopsy, histopathology of the skin and EBV test were carried out. By the end of follow-up, the patient receiving glucocorticoid was in stable condition. Conclusion NK-CLPD is a rare lymphoproliferative disorder that derived from nature NK cell. The typical immunophenotype is CD2^＋ , sCD3^- , CD16^＋ , with weak expression of CD56. This is the first case of cutaneous erythema muhiforme associated with NK-CLPD in China. The clinical courses of most patients are inert. Few patients can transform into aggressive NK cell leukemia.

关 键 词：NK细胞 慢性NK细胞淋巴增殖性疾病 多形红斑 

分 类 号：R551.2[医药卫生—血液循环系统疾病]