以发热伴关节肿痛为特征的组织细胞坏死性淋巴结炎1例报道

Histiocytic necrotizing lymphadenitis characterized by fever and joint pain:a case report

27岁女性,因间断性发热20^+d,膝关节肿痛7 d入院。患者持续发热,最高体温达40.1°C,双膝关节肿痛,双手及两大腿可见大量散在红色针尖样皮疹,淋巴结大、脾大,双手指间关节及双膝关节肿痛,淋巴结局限于双侧腋窝,质地软,局部压痛,活动度好。左侧腋窝淋巴结病理结果示:碎块状淋巴结组织,结构不完整,淋巴滤泡存在,滤泡减少或消失区见弥散成片组织细胞、免疫母细胞及少量小淋巴细胞增生,核分裂易见,其间有片状大小不等坏死灶,胞核崩解,并见较多纤维素样坏死血管,伴出血。免疫组化示CD3(弥漫+);CD20(滤泡+);CD79α(滤泡+);CD68(+);EMA(-);ALK(-);CD15(少量细胞+);CD30(+),CD4(-),CD5(+);CD10(-);bcl-2(+);CD21(+);粒酶B(-);TIA-1(-);EBV(-)Ki-67(+)。结合临床及免疫组化标记结果,明确为组织细胞坏死性淋巴结炎。患者停用抗菌药物,使用5%葡萄糖100 mL+氢化可的松琥珀酸钠200 mg静滴3 d后未见发热,腋窝淋巴结逐渐缩小。后改为甲泼尼龙8 mg/d,每日2次,每两周减一片,至最后停止使用,随访两年,多次复查血常规、肝肾功能在正常范围内,双侧膝关节不肿,可以自由行走,全身淋巴结无肿大。

A 27-year-old woman was admitted to a hospital due to intermittent fever for 20＋ days and swelling pain in knee joint for 7 days.Patients was with persistent fever,the highest temperature to 40.1°C,the double knee joint swelling pain,a large number of scattered red needle-like rash were visible on bilateral hands and legs,lymph-adenectasis,splenectasis,bilateral hands interphalangeal joint and knee joint swelling pain,lymph nodes were con-fined to the bilateral axillary with soft texture,local tenderness,and good activity.Pathological results of left axil-lary lymph node showed the following finding：fragmental lymph node tissue with incomplete structure,lymphoid follicles existed,there were diffuse histocytes,immunoblasts,and a few small lymphocytes proliferation in the re-duced or disappeared area of follicular,caryokinesis could be easily seen,there were patches of necrosis foci with varied size,nucleus disintegrated,fibrinoid necrosis blood vessel with bleeding could be seen.Immunohistochemical detection showed the following results：CD3 （diffuse ＋）;CD20 （follicular ＋）;CD79α（follicular ＋）;CD68 （＋）;EMA （-）;ALK （-）;CD15 （a small amount of cells ＋）;CD30 （＋）,CD4 （-）,CD5 （＋）;CD10 （-）;bcl-2 （＋）;CD21 （＋）;telomerase B （-）;TIA-1 （-）;EBV（-）Ki-67 （＋）.Through a combination of clinical and immunohistochemical detection results,the diagnosis of histiocytic necrotizing lymphadenitis was made.Patient＆nbsp;discontinued antimicrobial drugs,after she was treated with 5% glucose 100 mL ＋ hydrocortisone sodium succinate 200 mg intravenous drip for 3 days,patients had no fever,axillary lymph node gradually dwindled.Then patients was treated with methylprednisolone 8 mg/d,twice a day,reduced 1 tablet every two weeks,and stopped eventual-ly.Patients was followed up for two years,repeated examination of blood routine,liver and kidney function were in the normal range,bilateral knee didn’t swell,could walk freely,there appeared no enlargement of lymph node.