论文部分内容阅读
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 improve dermatologists’ understanding of chronic natural killer lymphoproliferative disorders (NK-CLPD) and to summarize their clinical features, diagnosis and treatment. Methods One case of NK-CLPD, which was characterized by erythema multiforme, was firstly reported in China. The clinical manifestation, histopathology were analyzed. Relevant literatures at home and abroad were reviewed. Results The patients were young and middle-aged women, with onset of 11 months. The clinical manifestations were fever and generalized erythema multiforme rash. According to the course of the disease, histopathological examination of lesions, blood images, immunofluorescence by flow cytometry, And virological tests can be clear NK-CLPD diagnosis, the main treatment of glucocorticoid-based, the effect of hormone therapy in patients with good. Conclusion NK-CLPD is a rare NK cell disease characterized by chronic expansion of mature NK cells in peripheral blood. Large particles of lymphocytes were found in peripheral blood and bone marrow. The typical immunophenotypes were CD2 ~ +, sCD3 ~ -, CD16 ~ + and weakly expressed CD56. NK-CLPD, characterized by erythema multiforme-like rash, has not been reported at home and abroad. The disease is inactive duration, some patients have a better prognosis, but the possibility of conversion to invasive NK cell leukemia.