Pure Red Cell Aplasia and Other Haematological Diseases Associated With Thymoma: A Case Series and Systematic Review

被引:6
作者
Yen, Chih-Chieh [1 ,2 ]
Huang, Wei-Li [3 ]
Li, Sin-Syue [4 ]
Chen, Ya-Ping [4 ]
Tseng, Yau-Lin [3 ]
Yen, Yi-Ting [3 ]
Chu, Chang-Yao [5 ]
Hsu, Ya-Ting [4 ]
Chen, Tsai-Yun [4 ]
机构
[1] Natl Cheng Kung Univ Hosp, Douliou Branch, Dept Internal Med, Div Haematol Oncol, Touliu, Yunlin, Taiwan
[2] Natl Cheng Kung Univ, Inst Clin Med, Sch Med, Tainan, Taiwan
[3] Natl Cheng Kung Univ, Natl Cheng Kung Univ Hosp, Div Thorac Surg, Dept Surg,Coll Med, Tainan, Taiwan
[4] Natl Cheng Kung Univ, Natl Cheng Kung Univ Hosp, Div Haematol, Dept Internal Med,Coll Med, Tainan, Taiwan
[5] Chi Mei Med Ctr, Dept Pathol, Tainan, Taiwan
关键词
thymoma; thymectomy; systematic review; pure red cell aplasia; autoimmune disease; Good's syndrome; AUTOIMMUNE-DISEASES; MYASTHENIA-GRAVIS; THYMECTOMY; CLASSIFICATION; INHIBITOR; SURVIVAL; THYMUS; TUMOR;
D O I
10.3389/fmed.2021.759914
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background: Thymoma-associated haematological diseases (HDs), such as pure red cell aplasia (PRCA) and Good's syndrome, are extremely rare, and due to the paucity of large-scale studies, the characteristics, remission after thymectomy, and long-term evaluation remain undetermined.Methods: We retrospectively assessed patients with thymoma and associated HDs from Jan 2005 to Dec 2020. All patients received thymectomy and/or additional treatments for HDs. A comparison with thymoma-associated myasthenic gravis (MG), and a systematic review from PubMed/MEDLINE and Embase were conducted.Results: In the median follow-up of 56 months, 130 patients were enrolled. Patients with thymoma-associated MG (n = 46) and HDs [n = 8; PRCA (n = 5), PRCA and Good's syndrome (n = 2) and autoimmune haemolytic anaemia (n = 1)] were evaluated. Patients with MG had a significantly higher remission rate after thymectomy (50 vs. 17%; p = 0.0378) as compared to those with other autoimmune diseases. Two of seven patients with PRCA experienced remission with thymectomy alone, and an additional two patients achieved remission with thymectomy plus immunosuppressive therapy (IST). In the systematic review, 60 studies (case reports, n = 46; case series including the present study, n = 14) were evaluated. Forty-four percent of patients were diagnosed with PRCA after thymoma, and 61% achieved remission with thymectomy plus IST; however, Good's syndrome was unaffected.Conclusions: Our study indicates that patients with thymoma-associated autoimmune diseases other than MG have a lower remission rate than those with MG. Remission of thymoma-associated PRCA can be achieved by thymectomy and IST. This study provides insight into extremely rare but puzzling autoimmune manifestations.
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页数:10
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