Chung Hee-Jung, Chi Hyun-sook, Cho Young-Uk, Jang Seongsoo, Park Chan-Jeoung
Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.
Korean J Lab Med. 2007 Jun;27(3):182-7. doi: 10.3343/kjlm.2007.27.3.182.
Fibrin-ring granuloma (FRG), which can be found in bone marrow or the liver, is a subtype of epithelioid granuloma characterized by a central fat vacuole and annular peripheral fibrinoid materials. FRG has been proven to be associated with many etiologies such as several infectious organisms (Coxiella burnett; Epstein-Barr Virus, EBV; cytomegalovirus, CMV; and hepatitis A virus), allopurinol induced hepatitis, Hodgkin's lymphoma, and peripheral T-cell lymphoma.
We retrospectively reviewed 24 patients diagnosed with FRG by bone marrow biopsy at a single institute between 1995 and 2004. We reviewed clinical symptoms and laboratory findings of the patients, classified them by etiology, and compared prognosis of each group.
The most common cause of FRG was acute or chronic EBV infection. Chronic or acute EBV infection was associated with 41.4% of patients (10/24). Of the remaining patients, 33.3% (8/24) were leukemia or lymphoma patients after chemotherapy, 4.2% (1/24) was a patient with hepatic failure, and 20.8% (5/24) were diagnosed as fever of unknown origin. The most common symptom and clinical finding were fever and cytopenia. EBV-associated group comprised chronic active EBV infection, EBV-associated hemophagocytic histiocytosis, acute EBV infection, EBV-associated lymphoproliferative disease, and Langerhans' cell histiocytosis. The EBV-associated group showed a lower survival probability compared with the non-EBV group (P<0.05).
Patients with bone marrow fibrin ring granuloma accompanied by fever require an active workup to find out the cause of infectious agents including EBV infection particularly due to their poor prognosis.
纤维蛋白环肉芽肿（FRG）可出现在骨髓或肝脏中，是上皮样肉芽肿的一种亚型，其特征为中央脂肪空泡和环形周边纤维蛋白样物质。FRG已被证明与多种病因相关，如几种感染性生物体（伯氏考克斯体；爱泼斯坦-巴尔病毒，EBV；巨细胞病毒，CMV；以及甲型肝炎病毒）、别嘌醇诱导的肝炎、霍奇金淋巴瘤和外周T细胞淋巴瘤。
我们回顾性分析了1995年至2004年间在单一机构通过骨髓活检确诊为FRG的24例患者。我们回顾了患者的临床症状和实验室检查结果，按病因进行分类，并比较了每组的预后。
FRG最常见的病因是急性或慢性EBV感染。慢性或急性EBV感染与41.4%的患者（10/24）相关。其余患者中，33.3%（8/24）为化疗后的白血病或淋巴瘤患者，4.2%（1/24）为肝功能衰竭患者，20.8%（5/24）被诊断为不明原因发热。最常见的症状和临床发现是发热和血细胞减少。EBV相关组包括慢性活动性EBV感染、EBV相关噬血细胞性组织细胞增生症、急性EBV感染、EBV相关淋巴增殖性疾病和朗格汉斯细胞组织细胞增生症。与非EBV组相比，EBV相关组的生存概率较低（P<0.05）。
伴有发热的骨髓纤维蛋白环肉芽肿患者需要积极检查以找出包括EBV感染在内的感染病原体原因，特别是鉴于其预后较差。
