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Abstract：
UNASSIGNED: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal failure, and organomegaly) syndrome is a systemic inflammatory disorder and unique clinicopathological variant of idiopathic multicentric Castleman disease that was proposed in Japan. Prompt diagnosis is critical because TAFRO syndrome is a progressive and life threating disease. Some cases are refractory to immunosuppressive treatments. Renal impairment is frequently observed in patients with TAFRO syndrome, and some severe cases require hemodialysis. Histological evaluation is important to understand the pathophysiology of TAFRO syndrome. However, systemic histopathological evaluation through autopsy in TAFRO syndrome has been rarely reported previously.
UNASSIGNED: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome.
UNASSIGNED: Bacterial peritonitis, systemic cytomegalovirus infection, and Trichosporon asahii infection in the lungs were observed on autopsy. In addition, sepsis-related myocardial calcification was suspected. Management of infectious diseases is critical to reduce mortality in patients with TAFRO syndrome. Although the exact cause of MI could not be identified on autopsy, we considered embolization by fungal hyphae as a possible cause. Endothelial injury possibly caused by excessive secretion of IL-6 and VEGF contributed to renal impairment. Fibrotic changes in anterior mediastinal fat tissue could be a characteristic pathological finding in patients with TAFRO syndrome.
UNASSIGNED: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome.
UNASSIGNED: Bacterial peritonitis, systemic cytomegalovirus infection, and Trichosporon asahii infection in the lungs were observed on autopsy. In addition, sepsis-related myocardial calcification was suspected. Management of infectious diseases is critical to reduce mortality in patients with TAFRO syndrome. Although the exact cause of MI could not be identified on autopsy, we considered embolization by fungal hyphae as a possible cause. Endothelial injury possibly caused by excessive secretion of IL-6 and VEGF contributed to renal impairment. Fibrotic changes in anterior mediastinal fat tissue could be a characteristic pathological finding in patients with TAFRO syndrome.
摘要：
■TAFRO（血小板减少症，Anasarca,发烧,网织蛋白骨髓纤维化/肾衰竭，和器官肿大）综合征是一种全身性炎症性疾病，是日本提出的特发性多中心Castleman病的独特临床病理变异。及时诊断至关重要，因为TAFRO综合征是一种进行性和威胁生命的疾病。一些病例难以用免疫抑制治疗。在TAFRO综合征患者中经常观察到肾功能损害，一些严重的病例需要血液透析.组织学评价对了解TAFRO综合征的病理生理学具有重要意义。然而,以前很少有报道通过尸检对TAFRO综合征进行全身组织病理学评估.
一名46岁的日本男子，主要主诉发热和腹胀，通过影像学检查被诊断为TAFRO综合征，实验室发现,颈淋巴结和骨髓活检的病理结果。白细胞介素（IL）-6和血管内皮生长因子（VEGF）水平在血液和腹水中均显着升高。在第10天开始甲基强的松龙（mPSL）脉冲治疗，然后与PSL和环孢素A联合治疗。腹水的量对治疗没有反应。病人出现了无尿症,从第50天开始持续肾脏替代治疗.然而,患者在同一天突然出现与心肌梗死(MI)相关的心脏骤停.虽然急诊经皮冠状动脉介入治疗成功,尽管接受了重症监护，但患者在第52天死亡。进行尸检以确定MI的原因并确定TAFRO综合征的组织病理学特征。
■细菌性腹膜炎，系统性巨细胞病毒感染，尸检观察到肺中的曲孢菌感染。此外,怀疑与脓毒症相关的心肌钙化.传染病的管理对于降低TAFRO综合征患者的死亡率至关重要。尽管尸检无法确定MI的确切原因，我们认为真菌菌丝栓塞是可能的原因。IL-6和VEGF的过度分泌可能导致内皮损伤。前纵隔脂肪组织的纤维化改变可能是TAFRO综合征患者的特征性病理发现。
一名46岁的日本男子，主要主诉发热和腹胀，通过影像学检查被诊断为TAFRO综合征，实验室发现,颈淋巴结和骨髓活检的病理结果。白细胞介素（IL）-6和血管内皮生长因子（VEGF）水平在血液和腹水中均显着升高。在第10天开始甲基强的松龙（mPSL）脉冲治疗，然后与PSL和环孢素A联合治疗。腹水的量对治疗没有反应。病人出现了无尿症,从第50天开始持续肾脏替代治疗.然而,患者在同一天突然出现与心肌梗死(MI)相关的心脏骤停.虽然急诊经皮冠状动脉介入治疗成功,尽管接受了重症监护，但患者在第52天死亡。进行尸检以确定MI的原因并确定TAFRO综合征的组织病理学特征。
■细菌性腹膜炎，系统性巨细胞病毒感染，尸检观察到肺中的曲孢菌感染。此外,怀疑与脓毒症相关的心肌钙化.传染病的管理对于降低TAFRO综合征患者的死亡率至关重要。尽管尸检无法确定MI的确切原因，我们认为真菌菌丝栓塞是可能的原因。IL-6和VEGF的过度分泌可能导致内皮损伤。前纵隔脂肪组织的纤维化改变可能是TAFRO综合征患者的特征性病理发现。
