Clinical analysis of six cases of TAFRO syndrome
10.3760/cma.j.cn141217-20240826-00258
- VernacularTitle:TAFRO综合征6例临床分析
- Author:
Sumiao LIU
1
;
Qianyu GUO
;
Pengyan QIAO
;
Yinfeng CUI
;
Ke XU
;
Liyun ZHANG
Author Information
1. 山西白求恩医院（山西医学科学院 同济山西医院） 山西医科大学第三医院风湿免疫科，太原 030032
- Publication Type:Journal Article
- Keywords:
TAFRO syndrome;
Autoimmune diseases;
Connective tissue disease
- From:
Chinese Journal of Rheumatology
2025;29(2):118-122
- CountryChina
- Language:Chinese
-
Abstract:
Objective:The clinicalpathological features of TAFRO syndrome were analyzed to clarify the similarities and differences between TAFRO syndrome and autoimmune diseases and to establish differential diagnosis.Methods:Six patients diagnosed with TAFRO syndrome in Shanxi Bethune Hospital from January 2014 to March 2022 were collected. The clinical, examination, pathology and treatment of TAFRO syndrome were analyzed and compared with autoimmune diseases, especially systemic lupus erythematosus and Sj?gren′s syndrome.Results:Among the 6 patients, 4 were males and 2 were females, with an average age of (57.5 ±9.8) years. All the 6 patients had fever, edema (including chest and abdominal effusion and systemic edema), thrombocytopenia (3 main criteria) and more than 2 secondary criteria.ESR and CRP were significantly elevated in 6 patients. There were 1 case of elevated IgA and IgG (IgA 4.10 g/L, IgG19.05 g/L), 1 case of elevated igg (IgG 19.33 g/L), 3 cases of normal and 1 case of undetected. Serum IgG4 was negative in 4 cases and undetected in 2 cases. Autoantibodies: 4 cases were ANA positive, including 1 case with anti-SSA/Ro52(+), anti-SSA/Ro60(+), anti-SSB (+), 1 case with anti-SSA /Ro60(+), and 2 untested. Bone marrow cytological examination was performed in 6 cases, all of which showed active hyperplasia, 2 cases showed elevated megakaryocytes, and 1 case was accompanied by interstitial fibrosis. Pathological examination of lymph nodes: 5 cases were consistent with Castleman′s disease, and 1 case was suggestive of reactive hyperplasia of lymph nodes. Conclusion:Although the diagnostic criteria of TAFRO syndrome should exclude autoimmune diseases, TAFRO syndrome and autoimmune diseases can coexist, and the connective tissue disease complicated with TAFRO syndrome has its specific clinical characteristics and treatment plan, which needs to be identified clinically.
