[Clinical Anslysis of TAFRO Syndrome]

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Dec;31(6):1872-1877. doi: 10.19746/j.cnki.issn.1009-2137.2023.06.042.
[Article in Chinese]

Abstract

Objective: To investigate the clinical characteristics, diagnosis, and treatment of one patient with TAFRO syndrome, and to strengthen the understanding of this rare type.

Methods: The clinical manifestations, diagnosis and treatment process, and prognosis of the patient admitted in Gansu Provincial People's Hospital were retrospectively analyzed.

Results: Combined with laboratory tests, bone marrow examination, imaging, pathology, etc, the patient was diagnosed with TAFRO syndrome. After three cycles of treatment with pomalidomide (2-3 mg/d, d1-21), cyclophosphamide (300 mg/m2, 0.54 g once a week) and dexamethasone (20 mg/d, two days a week), platelet count, serum creatinine and procalcitonin returned to normal, the systemic edema disappeared, and the patient's condition was alleviated. The therapeutic effect was good.

Conclusion: TAFRO syndrome is rare, involves multiple systems, progresses rapidly, and has a worse prognosis. The choice of the "Pomalidomide+cyclophosphamide+dexamethasone" regimen is help to improve the survival prognosis of patient with TAFRO syndrome.

题目: TAFRO综合征临床分析.

目的: 探讨1例TAFRO综合征患者的临床特点及诊疗方案,加强对该类型罕见病的认识.

方法: 回顾性分析甘肃省人民医院收治的1例TAFRO综合征患者的临床表现、诊疗过程及预后.

结果: 结合实验室检查、骨髓学检查、影像学检查及病理学等检查,患者诊断为TAFRO综合征,给予泊马度胺(2-3 mg/d,d 1-21)、环磷酰胺(300 mg/m2,0.54 g每周一次)、地塞米松(20 mg/d,每周两天)方案治疗,3个周期后患者血小板计数、血肌酐、降钙素原等指标均恢复正常,全身水肿消褪,评估病情缓解,治疗效果好.

结论: TAFRO综合征罕见,多系统受累,进展快,预后差,“泊马度胺+环磷酰胺+地塞米松”方案治疗有助于改善TAFRO综合征患者的生存预后.

Keywords: TAFRO syndrome; cyclophosphamide; pomalidomide.

Publication types

  • English Abstract

MeSH terms

  • Castleman Disease* / diagnosis
  • Castleman Disease* / drug therapy
  • Cyclophosphamide / therapeutic use
  • Dexamethasone
  • Humans
  • Retrospective Studies
  • Thrombocytopenia*

Substances

  • pomalidomide
  • Dexamethasone
  • Cyclophosphamide

Supplementary concepts

  • Multi-centric Castleman's Disease