Good’s Syndrome (GS): Thymoma with Immunodeficiency

  • Hans-Hartmut PeterEmail author
Part of the Rare Diseases of the Immune System book series (RDIS)


The association in adults of thymoma with hypogammaglobulinemia, low B-cell counts, and a variable degree of cellular immunodeficiency (low CD4 and NK cells) was first described by R.A. Good as a rare form of acquired combined immunodeficiency. Besides an increased susceptibility to respiratory infections with encapsulated bacteria, many patients suffer from chronic diarrhea, opportunistic infections (e.g., CMV, candida, tuberculosis, Pneumocystis), pure red cell aplasia (PRCA), and various autoimmune diseases notably myasthenia gravis (MG).


Combined immunodeficiency Thymoma Hypogammaglobulinemia Respiratory and gastrointestinal infections Pure red cell aplasia Autoimmunity Myasthenia gravis 


  1. 1.
    Good RA. Agammaglobulinaemia—a provocative experiment of nature. Bull Univ Minn. 1954;26:1–19.Google Scholar
  2. 2.
    Kelesidis T, Yang O. Good’s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135(3):347–63. ReviewCrossRefPubMedGoogle Scholar
  3. 3.
    Dong JP, Gao W, Teng GG, Tian Y, Wang HH. Characteristics of Good’s Syndrome in China: a systematic review. Chin Med J. 2017;130:1604–9.CrossRefGoogle Scholar
  4. 4.
    Masaoka A, Monden Y, Nakahara K, Tanioka T. Follow-up study of thymomas with special reference to their clinical stages. Cancer. 1981;48:2485–92.CrossRefGoogle Scholar
  5. 5.
    den Bakker MA, Roden AC, Marx A, Marino M. Histologic classification of thymoma: a practical guide for routine cases. J Thorac Oncol. 2014;9(9 Suppl 2):S125–30. ReviewCrossRefGoogle Scholar
  6. 6.
    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, Sève P. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev. 2016;15(1):82–92. ReviewCrossRefPubMedGoogle Scholar
  7. 7.
    Thongprayoon C, Tantrachoti P, Phatharacharukul P, Buranapraditkun S, Klaewsongkram J. Associated immunological disorders and cellular immune dysfunction in thymoma: a study of 87 cases from Thailand. Arch Immunol Ther Exp. 2013;61(1):85–93. Epub 2012 Dec 11CrossRefGoogle Scholar
  8. 8.
    Hayward AR, Paolucci P, Webster ADB, et al. Pre-B cell suppression by thymoma patients’ lymphocytes. Clin Exp Immunol. 1982;48:437–42.PubMedPubMedCentralGoogle Scholar
  9. 9.
    Yamazaki K, Watanabe N, Hasegawa A, Yamaguchi E, Miyamoto K, Sakiyama Y, Kawakami Y. Good’s syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis. Intern Med. 1994;33(8):496–500. ReviewCrossRefGoogle Scholar
  10. 10.
    Oritani K, Medina KL, Tomiyama Y, Ishikawa J, Okajima Y, Ogawa M, Yokota T, Aoyama K, Takahashi I, Kincade PW, Matsuzawa Y. Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors. Nat Med. 2000;6(6):659–66.CrossRefGoogle Scholar
  11. 11.
    Christopoulos P, Dopfer EP, Malkovsky M, Esser PR, Schaefer HE, Marx A, Kock S, Rupp N, Lorenz MR, Schwarz K, Harder J, Martin SF, Werner M, Bogdan C, IWW S, Fisch P. A novel thymoma-associated immunodeficiency with increased naive T cells and reduced CD247 expression. J Immunol. 2015;194(7):3045–53. Scholar
  12. 12.
    Sun X, Shi J, Wang M, Xu K, Xiao Y. Good’s syndrome patients hospitalized for infections: a single-center retrospective study. Medicine (Baltimore). 2015;94(47):e2090. Scholar
  13. 13.
    Kelleher P, Misbah SA. What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003;56(1):12–6.CrossRefGoogle Scholar
  14. 14.
    Joven MH, Palalay MP, Sonido CY. Case report and literature review on Good's syndrome, a form of acquired immunodeficiency associated with thymomas. Hawaii J Med Public Health. 2013;72(2):56–62.PubMedPubMedCentralGoogle Scholar
  15. 15.
    Ide S, Koga T, Rikimaru T, Katsuki Y, Oizumi K. Good’s syndrome presenting with cytomegalovirus pneumonia. Intern Med. 2000;39(12):1094–6.CrossRefGoogle Scholar
  16. 16.
    Wargo JJ, Kim AH, Hart A, Berg A. It took a village: Good’s syndrome. Am J Med. 2015;128(7):699–701. Scholar
  17. 17.
    Tachdjian R, Keller JJ, Pfeffer M. A bad case of Good’s syndrome. Infect Dis Ther. 2014;3(2):333–7. Scholar
  18. 18.
    Ueno S, Sekimoto-Tsuboi S, Ishiguro Y, Koinuma T, Eguchi H, Machida Y, HattoriN MH. Good's syndrome with opportunistic infection of the central nervous system: a case report. BMC Neurol. 2015;15:150. Scholar
  19. 19.
    Sveinsson O, Matell H, Herrman L. Progressive multifocal leukoencephalopathy in a patient with Good's syndrome. BMJ Case Rep. 2013;2013:pii: bcr2013009763. Scholar
  20. 20.
    Sasson SC, Davies S, Chan R, Davies L, Garsia R. Cerebral toxoplasmosis in a patient with myasthenia gravis and thymoma with immunodeficiency/Good's syndrome: a case report. BMC Infect Dis. 2016;16(1):457. Scholar
  21. 21.
    Akinosoglou K, Melachrinou M, Siagris D, Koletsis E, Marangos M, Gogos CA, Solomou EE. Good’s syndrome and pure white cell aplasia complicated by cryptococcus infection: a case report and review of the literature. J Clin Immunol. 2014;34(3):283–8. Scholar
  22. 22.
    Disselhorst MJ, Dickhoff C, Alhan C. Good’s syndrome: an uncommon cause of therapy-resistant diarrhoea. Neth J Med. 2016;74(7):309–12.PubMedGoogle Scholar
  23. 23.
    Rawat A, Dhir V, Gupta A, Suri D, Burad DK, Nada R, Singh S. Good’s syndrome presenting with recurrent giardiasis. J Clin Immunol. 2014;34(7):751–2. Scholar
  24. 24.
    Verne GN, Amann ST, Cosgrove C, Cerda JJ. Chronic diarrhea associated with thymoma and hypogammaglobulinemia (Good’s syndrome). South Med J. 1997;90(4):444–6.CrossRefGoogle Scholar
  25. 25.
    Murray WD, Webb JN. Thymoma associated with hypogammaglobulinaemia and pure red cell aplasia. Am J Med. 1966;41:974–80.CrossRefGoogle Scholar
  26. 26.
    Taniguchi T, Usami N, Kawaguchi K, Yokoi K. Good syndrome accompanied by pure red cell aplasia. Interact Cardiovasc Thorac Surg. 2009;9(4):750–2. Scholar
  27. 27.
    Kuribayashi K, Fujimi A, Kobune M, Takimoto R, Kikuchi S, Iyama S, Kato J, Niitsu Y, Watanabe N. Pure red cell aplasia associated with Good's syndrome accompanied by decreased stem cell factor production in the bone marrow. Intern Med. 2010;49(5):377–82.CrossRefGoogle Scholar
  28. 28.
    Nitta H, Harada Y, Okikawa Y, Fujii M, Arihiro K, Kimura A, Harada H. Good’s syndrome-associated pure red cell aplasia with myelodysplastic syndrome. Intern Med. 2011;50(18):2011–4.CrossRefGoogle Scholar
  29. 29.
    Hashizume T. Good’s syndrome and pernicious anemia. Intern Med. 2002;41(11):1062–4.CrossRefGoogle Scholar
  30. 30.
    Kaku Y, Shimamoto N, Matsunaga H, Makiura M, Fujisawa A, Morita K. Oral erosive lichen planus and alopecia areata with Good's syndrome (thymoma with hypogammaglobulinemia). Eur J Dermatol. 2011;21(1):124–5. Scholar
  31. 31.
    Hanafusa T, Umegaki N, Yamaguchi Y, Katayama I. Good's syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus. J Dermatol. 2010;37(2):171–4. Scholar
  32. 32.
    Ohuchi M, Inoue S, Hanaoka J, Igarashi T, Tezuka N, Ozaki Y, et al. Good syndrome coexisting with leukopenia. Ann Thorac Surg. 2007;84:2095–7. Scholar
  33. 33.
    Kristiansen HA, Spetalen S, Fløisand Y, Heldal D. Eltrombopag in Good’s Syndrome. Case Rep Hematol. 2014;2014:172139. Scholar
  34. 34.
    Soppi E, Eskola J, Veromaa T, et al. Thymoma with immunodeficiency (Good’s syndrome) associated with myasthenia gravis and benign IgG gammopathy. Arch Intern Med. 1985;145:1704–7.CrossRefGoogle Scholar
  35. 35.
    Zonios D, Sheikh V, Sereti I. Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells. Arthritis Res Ther. 2012;14(4):222. Scholar
  36. 36.
    Glück J, Rymarczyk B, Paluch U, Rogala B, Brzoza Z. A case of Good’s syndrome diagnosed after more than 20 years since onset of myasthenia in a patient with psoriasis. Neurol Sci. 2016;37(7):1179–80. Scholar
  37. 37.
    Habib AM, Thornton H, Sewell WC, Loubani M. Good's syndrome: Is thymectomy the solution? Case report and literature review. Asian Cardiovasc Thorac Ann. 2016;24(7):712–4. Scholar
  38. 38.
    Antar AI, Otrock ZK, Kharfan-Dabaja MA, Mahfouz RA, Alameddine RS, El-Majzoub NM, Salem ZM. Thymoma with concomitant pure red cell aplasia, Good's syndrome and myasthenia gravis responding to rituximab. Indian J Hematol Blood Transfus. 2016;32(Suppl 1):219–22. Scholar
  39. 39.
    Ternavasio-de la Vega HG, Velasco-Tirado V, Pozo-Rosado L, Soler-Fernández MC, Pérez-Andres M, Orfao A, Sánchez-Sánchez R, González-Villaron L. Persistence of immunological alterations after thymectomy in Good's syndrome: a clue to its pathogenesis. Cytometry B Clin Cytom. 2011;80(5):339–42. Scholar
  40. 40.
    Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993;86(1):31–42.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Center for Chronic ImmunodeficiencyUniversity Medical Center FreiburgFreiburgGermany

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