CAJ | 학술논문

目的探讨血管内NK细胞淋巴瘤(IVNKL)的临床病理特征及预后特点，以提高对这一罕见疾病的认识。方法采用HE、免疫组织化学染色（EliVision法）和原位杂交技术，结合临床资料对2例IVNKL进行分析，对文献中的7例及该组2例患者进行临床病理学特征分析和预后比较。结果2例患者分别为68岁女性和22岁男性，均表现为躯干和四肢皮疹伴发热，皮肤活检结果符合典型的血管内淋巴瘤，免疫表型证实为NK细胞来源，即CD3、CD56、颗粒酶B和EBV编码的小RNA探针(EBER)阳性。2例患者均于确诊2个月后死亡。汇总文献，9例患者中男4例，女5例，平均年龄45.7岁，中位数47岁，红斑皮疹是最常见的临床表现，7例患者病程中出现多系统受累，3例中枢神经系统受累。随访时间2～17个月，9例中6例死亡，中位生存时间为9个月，1年生存率为(35.6±18.6)％。多系统受累患者的死亡率( 6/7)略高于单纯皮疹者(0/2)，但差异无统计学意义(P =0.083)。结论IVNKL是一种罕见疾病，确诊需要依据典型组织学特点，并结合免疫表型分析和EBER原位杂交检测。该病预后较差，需尽早确诊、及时治疗，以避免多系统受累。
목적탐토혈관내NK세포림파류(IVNKL)적림상병리특정급예후특점，이제고대저일한견질병적인식。방법채용HE、면역조직화학염색（EliVision법）화원위잡교기술，결합림상자료대2례IVNKL진행분석，대문헌중적7례급해조2례환자진행림상병이학특정분석화예후비교。결과2례환자분별위68세녀성화22세남성，균표현위구간화사지피진반발열，피부활검결과부합전형적혈관내림파류，면역표형증실위NK세포래원，즉CD3、CD56、과립매B화EBV편마적소RNA탐침(EBER)양성。2례환자균우학진2개월후사망。회총문헌，9례환자중남4례，녀5례，평균년령45.7세，중위수47세，홍반피진시최상견적림상표현，7례환자병정중출현다계통수루，3례중추신경계통수루。수방시간2～17개월，9례중6례사망，중위생존시간위9개월，1년생존솔위(35.6±18.6)％。다계통수루환자적사망솔( 6/7)략고우단순피진자(0/2)，단차이무통계학의의(P =0.083)。결론IVNKL시일충한견질병，학진수요의거전형조직학특점，병결합면역표형분석화EBER원위잡교검측。해병예후교차，수진조학진、급시치료，이피면다계통수루。
Objective To study the clinicopathologic features and disease outcome of intravascular natural killer-cell lymphoma (IVNKL). Methods The histologic features, immunohistochemical findings and results of in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) were analyzed in 2 novel cases of IVNKL. Seven cases of IVNKL previously reported in the literature were reviewed. Results The patients were a 68-year-old woman and a 22-year-old man. They both presented with erythematous patches and nodules on their trunk and extremities. Skin biopsies confirmed the diagnosis of IVNKL. The tumor cells were positive for CD3, CD56, granzyme B and EBER. Both patients died 2 months after the diagnosis.Amongst the 9 reported cases, including those from the literature, the male was 4 cases, the female was 5 cases. The mean age of the patients was 45.7 years and the median age was 47 years. Skin lesions represented the commonest clinical manifestations. Multiple organ involvement was found in 7 cases and central nervous system was involved in 3 cases. Six patients died during 2 to 17 months of follow-up. The median survival was 9 months and the one-year survival rate was (35.6 ± 18.6)％. The clinical outcome of the patients with multiple organ involvement was worse than that with skin manifestations only. The difference however was not statistically significant ( P = 0.083 ). Conclusions IVNKL is a rare disease.Diagnosis should be made according to typical histologic findings, immunophenotype and EBER in-situ hybridization results. The overall prognosis of IVNKL is poor. Early diagnosis and treatment before multiorgan involvement may be helpful in improving the clinical outcome.