[Improvement of tissue fibrosis in TAFRO syndrome by prednisolone monotherapy].

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI:10.11406/rinketsu.65.237

Takao Kashiwagi, Yuki Kashiwagi, Tomohide Suzuki, Manami Shingu, Sayaka Kashiwagi, Tamaki Hirata, Junko Hori, Keikichi Miyata, Miho Takechi, Minoru Kishi, Takamasa Ohnishi, Yoshio Katayama, Masahide Iwai, Toshimitsu Matsui

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Abstract

We report the case of a 48-year-old man who presented with fatigue and weight loss. A local physician observed elevated alkaline phosphatase levels, anemia, thrombocytopenia, and renal dysfunction. Fever also appeared, and the patient was admitted to our hospital. Computed tomography revealed hepatosplenomegaly, pleural and ascitic fluid, and left axillary lymphadenopathy. Bone marrow biopsy indicated hyperplasia with increased megakaryocytes and reticulin fibrosis. Axillary lymph node biopsy showed Castleman's disease-like features. Liver biopsy revealed proliferation of reticulin fibrosis. Therefore, TAFRO syndrome was diagnosed and treatment with 1 mg/kg prednisolone was started. Anemia and thrombocytopenia improved, and after 24 weeks of treatment, serum hyaluronic acid and type IV collagen decreased to the normal range. Bone marrow biopsy after 18 weeks of treatment showed decreased reticular fibers. In TAFRO syndrome, improvement of liver and bone marrow fibrosis can be expected with adequate intervention, and serum hyaluronic acid and type IV collagen are useful for evaluating fibrosis.

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[泼尼松龙单药疗法改善 TAFRO 综合征的组织纤维化]。

我们报告了一例 48 岁男性的病例，患者出现乏力和体重减轻。当地医生发现他的碱性磷酸酶水平升高、贫血、血小板减少和肾功能不全。患者还出现了发热，于是被送入我院。计算机断层扫描显示肝脾肿大、胸腔积液和腹水，以及左侧腋窝淋巴结肿大。骨髓活检显示骨髓增生，巨核细胞增多，网状纤维化。腋窝淋巴结活检显示出类似卡斯特曼病的特征。肝脏活检显示网织纤维化增生。因此，诊断为 TAFRO 综合征，并开始使用 1 毫克/千克泼尼松龙进行治疗。贫血和血小板减少得到改善，治疗 24 周后，血清透明质酸和 IV 型胶原降至正常范围。治疗 18 周后，骨髓活检显示网状纤维减少。在TAFRO综合征中，通过适当干预，肝脏和骨髓纤维化可望得到改善，血清透明质酸和IV型胶原蛋白可用于评估纤维化。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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[Rinsho ketsueki] The Japanese journal of clinical hematology

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