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

被引:5
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
相关论文
共 36 条
  • [1] PURE RED CELL APLASIA AND THYMOMA - LOSS OF SERUM INHIBITOR OF ERYTHROPOIESIS FOLLOWING THYMECTOMY
    ALMONDHIRY, H
    ZANJANI, ED
    SPIVACK, M
    ZALUSKY, R
    GORDON, AS
    [J]. BLOOD-THE JOURNAL OF HEMATOLOGY, 1971, 38 (05): : 576 - +
  • [2] Thymoma associated with autoimmune diseases: 85 cases and literature review
    Bernard, C.
    Frih, H.
    Pasquet, F.
    Kerever, S.
    Jamilloux, Y.
    Tronc, F.
    Guibert, B.
    Isaac, S.
    Devouassoux, M.
    Chalabreysse, L.
    Broussolle, C.
    Petiot, P.
    Girard, N.
    Seve, P.
    [J]. AUTOIMMUNITY REVIEWS, 2016, 15 (01) : 82 - 92
  • [3] The pathophysiology of pure red cell aplasia: Implications for therapy
    Charles, RJ
    Sabo, KM
    Kidd, PG
    Abkowitz, JL
    [J]. BLOOD, 1996, 87 (11) : 4831 - 4838
  • [4] DANDREA V, 1993, THYMUS, V21, P1
  • [5] The Masaoka-Koga Stage Classification for Thymic Malignancies Clarification and Definition of Terms
    Detterbeck, Frank C.
    Nicholson, Andrew G.
    Kondo, Kazuya
    Van Schil, Paul
    Moran, Cesar
    [J]. JOURNAL OF THORACIC ONCOLOGY, 2011, 6 (07) : S1710 - S1716
  • [6] Epidemiology of Thymoma and Associated Malignancies
    Engels, Eric A.
    [J]. JOURNAL OF THORACIC ONCOLOGY, 2010, 5 : S260 - S265
  • [7] The Management of Thymoma: A Systematic Review and Practice Guideline
    Falkson, Conrad B.
    Bezjak, Andrea
    Darling, Gail
    Gregg, Richard
    Malthaner, Richard
    Maziak, Donna E.
    Yu, Edward
    Smith, Christopher A.
    McNair, Sheila
    Ung, Yee C.
    Evans, William K.
    [J]. JOURNAL OF THORACIC ONCOLOGY, 2009, 4 (07) : 911 - 919
  • [8] The thymus, thymoma and myasthenia gravis
    Fujii, Yoshitaka
    [J]. SURGERY TODAY, 2013, 43 (05) : 461 - 466
  • [9] Long-term response and outcome following immuno-suppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group
    Hirokawa, Makoto
    Sawada, Ken-ichi
    Fujishima, Naohito
    Nakao, Shinji
    Urabe, Akio
    Dan, Kazuo
    Fujisawa, Shin
    Yonemura, Yuji
    Kawano, Fumio
    Omine, Mitsuhiro
    Ozawa, Keiya
    [J]. HAEMATOLOGICA, 2007, 93 (01) : 27 - 33
  • [10] Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group
    Hirokawa, Makoto
    Sawada, Kenichi
    Fujishima, Naohito
    Teramura, Masanao
    Bessho, Masami
    Dan, Kazuo
    Tsurumi, Hisashi
    Nakao, Shinji
    Urabe, Akio
    Fujisawa, Shin
    Yonemura, Yuji
    Kawano, Fumio
    Oshimi, Kazuo
    Sugimoto, Koichi
    Matsuda, Akira
    Karasawa, Masamitsu
    Arai, Ayako
    Komatsu, Norio
    Harigae, Hideo
    Omine, Mitsuhiro
    Ozawa, Keiya
    Kurokawa, Mineo
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 2015, 169 (06) : 879 - 886