Yamashita Hiroyuki, Takahashi Yuko, Kano Toshikazu, Kaneko Hiroshi, Mimori Akio
Division of Rheumatic Diseases, National Center for Global Health and Medicine.
Nihon Rinsho Meneki Gakkai Kaishi. 2012;35(2):136-43. doi: 10.2177/jsci.35.136.
We classified underlying diseases presenting as inflammation of unknown origin in division of rheumatic diseases. On the other hand, patients with malignant lymphoma (ML) varying in cause and symptoms are often admitted to our Rheumatology Division as cases of malignant disease presenting with fever of unknown origin.
Clinical records over 5 years were reviewed for 246 cases of undiagnosed inflammatory diseases at hospitalization.
The 246 patients included 76 patients with infection, 116 with rheumatic disease, 8 with solid cancer, 9 with drug-induced fever, 10 with other disorders, and 27 with suspected ML. Of theses, malignant lymphoma (ML) was diagnosed in 20 patients (5 Mature T-cell and NK-cell neoplasms, 3 Hodgkin lymphomas, and 12 Mature B cell neoplasms including 5 Intravascular large B-cell lymphoma (IVLBCL)). The reasons for hospitalization were underlying collagen disease in 6 patients, medical workup for fever of unknown origin in 8 patients, and symptoms suggestive of collagen disease in 6. Nearly all of these patients presented with fever and other B symptoms, frequently with concurrent hypoxemia and hemophagocytic syndrome in IVLBCL. For diagnostic purposes, biopsy sites were determined by 18 fluorodexyglucose-positron emission tomography and computed tomography (FDG-PET/CT) in 13 patients, and findings on random skin biopsies were diagnostic in 3 of 5 patients with IVLBCL. Laboratory data on patients with B-cell lymphoma or peripheral T-cell lymphoma revealed tendencies for thrombocytopenia, elevated serum LDH, and increased soluble interleukin-2 receptor (sIL-2R). High serum ferritin values were more frequent in IVLBCL than in other B-cell lymphomas.
ML ranked first among causes of inflammation of unknown origin at the division of Rheumatic Diseases, and FDG-PET/CT proved to be useful for biopsy site selection. IVLBCL was not uncommon, and our results show the usefulness of clinical features and random skin biopsies for rapid diagnosis.
我们对风湿性疾病科中表现为不明原因炎症的基础疾病进行了分类。另一方面，病因和症状各异的恶性淋巴瘤（ML）患者常以不明原因发热的恶性疾病病例被收治入我们的风湿科。
回顾了5年期间246例住院时未确诊的炎症性疾病患者的临床记录。
246例患者中，76例为感染，116例为风湿性疾病，8例为实体癌，9例为药物性发热，10例为其他疾病，27例疑似ML。其中，20例患者被诊断为恶性淋巴瘤（ML）（5例成熟T细胞和NK细胞肿瘤，3例霍奇金淋巴瘤，12例成熟B细胞肿瘤，包括5例血管内大B细胞淋巴瘤（IVLBCL））。住院原因包括6例潜在的胶原病，8例因不明原因发热进行医学检查，6例有提示胶原病的症状。几乎所有这些患者都有发热和其他B症状，IVLBCL患者常并发低氧血症和噬血细胞综合征。为了诊断，13例患者通过18氟脱氧葡萄糖 - 正电子发射断层扫描和计算机断层扫描（FDG - PET/CT）确定活检部位，5例IVLBCL患者中有3例通过随机皮肤活检结果确诊。B细胞淋巴瘤或外周T细胞淋巴瘤患者的实验室数据显示有血小板减少、血清乳酸脱氢酶升高和可溶性白细胞介素 - 2受体（sIL - 2R）增加的趋势。IVLBCL患者血清铁蛋白值高的情况比其他B细胞淋巴瘤更常见。
在风湿性疾病科不明原因炎症的病因中，ML位居首位，FDG - PET/CT被证明对活检部位的选择有用。IVLBCL并不罕见，我们的结果表明临床特征和随机皮肤活检对快速诊断有用。
