Papaioannou G, Speletas M, Kaloutsi V, Pavlitou-Tsiontsi A
Hematology and Immunology Department, Papageorgiou General Hospital, N. Eukarpia, 56429 Thessaloniki, Greece.
Ann Hematol. 2002 Dec;81(12):732-5. doi: 10.1007/s00277-002-0562-4. Epub 2002 Nov 14.
Histiocytic necrotizing lymphadenitis (HNL), or Kikuchi-Fujimoto disease, is a benign, self-limited disease that predominantly occurs in women. The etiology remains undetermined, although a viral or autoimmune hypothesis has been suggested. The disease usually emerges with cervical lymphadenopathy with or without fever. The diagnosis can be confirmed only by histological findings of lymph node biopsy, characterized by necrosis and histiocytic infiltration without neutrophils. We report a case of a 28-year-old woman with a medical history of two episodes of unexplained pulmonary embolisms (3 and 2 years previously) who was admitted to our hospital because of unilateral cervical lymphadenopathy and mild fever that presented 1 week before admission. A diagnosis of HNL was performed by lymph node biopsy. In parallel, whereas the laboratory tests for inherited thrombophilia were negative, a progressive elevated titer of anti-beta(2) glycoprotein I (GPI) antibodies was established. Because of persistent fever, the patient received a short course of corticosteroid therapy and she recovered completely from the HNL after 2 months. It is noteworthy that to date the patient has displayed an elevated titer of anti-beta(2) GPI antibodies (18 months after the recovery from the HNL). Thus, considering the previous history of venous thrombosis and the presence of antiphospholipid antibodies, the diagnosis of primary antiphospholipid syndrome associated with HNL was made. To our knowledge, this is the first report in the literature describing antiphospholipid syndrome associated with HNL. Moreover, a brief literature review is provided with emphasis on the etiology, clinical course, and pathogenesis of this rare disease entity.
组织细胞坏死性淋巴结炎（HNL），即菊池-藤本病，是一种主要发生于女性的良性自限性疾病。尽管有人提出病毒或自身免疫假说，但病因仍未明确。该病通常表现为伴有或不伴有发热的颈部淋巴结病。只有通过淋巴结活检的组织学检查结果才能确诊，其特征为坏死和组织细胞浸润而无中性粒细胞。我们报告一例28岁女性患者，有两次不明原因肺栓塞病史（分别在3年前和2年前），因入院前1周出现单侧颈部淋巴结病和低热而入住我院。通过淋巴结活检诊断为HNL。同时，遗传性血栓形成倾向的实验室检查结果为阴性，但抗β2糖蛋白I（GPI）抗体滴度呈进行性升高。由于持续发热，患者接受了短期糖皮质激素治疗，2个月后HNL完全康复。值得注意的是，迄今为止该患者的抗β2 GPI抗体滴度仍升高（从HNL康复后18个月）。因此，考虑到既往静脉血栓形成病史和抗磷脂抗体的存在，诊断为与HNL相关的原发性抗磷脂综合征。据我们所知，这是文献中首例描述与HNL相关的抗磷脂综合征的报告。此外，还提供了一篇简短的文献综述，重点介绍了这种罕见疾病实体的病因、临床过程和发病机制。
