Iranian Journal of Allergy, Asthma and Immunology 2018. 17(1):85-93.

Good’s Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report
Marzieh Tavakol, Seyed Alireza Mahdaviani, Mir Reza Ghaemi, Mohammad Vaezi, Atosa Dorudinia, Hamidreza Jamaati, Ali Akbar Velayati

Abstract


Good’s syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner’s syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome. A 57-year-old man was admitted to our hospital with a history of thymectomy due to thymoma (Type A) 6 years ago. He developed weight loss and recurrent persistent diarrhea caused by isospora belli. His chest CT scan revealed bilateral bronchiectasis. His laboratory data showed hypogammaglobulinemia and he was treated by monthly IVIG with the diagnosis of good’s syndrome. Nevertheless he referred again with left sided loss of vision because of CMV retinitis and he also developed nail candidiasis. Good’s syndrome should be considered in every patient with a history of thymoma and recurrent infection. Immunologic evaluation of these patients including measurement of the serum level of immunoglobulin as well as B cell and T cell subgroups should be performed. Physicians must be aware and think about this entity in patients with adult onset immunodeficiency.


Keywords


Cytomegalovirus; Hypogammaglobulinemia; Retinitis; Thymoma

Full Text:

PDF

References


1. Masci AM, Palmieri G, Vitiello L, Montella L, Perna F, Orlandi P, et al. Clonal expansion of CD8+ BV8 T lymphocytes in bone marrow characterizes thymoma-associated B lymphopenia. Blood. 2003; 101(8):3106-8.

2. 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.

3. Engels EA. Epidemiology of thymoma and associated malignancies. Journal of thoracic oncology: J Thorac Oncol 2010; 5(10 Suppl 4):S26-5.

4. Agrawal R, Kanchana B, Venkatachalam G, Narayan U. Thymoma: Clinical experience from a tertiary care institute from North India. J Cancer Res Ther 2014; 10(1):214-5.

5. Suster S. Diagnosis of thymoma. J Clin Pathol 2006; 59(12):1238-44.

6. Agarwal S, Cunningham-Rundles C. Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. Ann Allergy Asthma Immunol 2007; 98(2):185-90.

7. 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.

8. Caperton C, Agrawal S, Gupta S. Good syndrome presenting with CD8+ T-Cell large granular lymphocyte leukemia. Oncotarget 2015; 6(34):36577-86.

9. Malphettes M, Gérard L, Galicier L, Boutboul D, Asli B, Szalat R, et al. Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. Clin Infect Dis 2015; 61(2):e13-e9.

10. Tarr PE, Sneller MC, Mechanic LJ, Economides A, Eger CM, Strober W, et al. Infections in patients with immunodeficiency with thymoma (Good syndrome): report of 5 cases and review of the literature. Medicine 2001; 80(2):123-33.

11. Detterbeck FC, Parsons AM. Thymic tumors. Ann Thorac Surg 2004; 77(5):1860-9.

12. Kelleher P, Misbah S. What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003; 56(1):12-6.

13. Kitamura A, Takiguchi Y, Tochigi N, Watanabe S-i, Sakao S, Kurosu K, et al. Durable hypogammaglobulinemia associated with thymoma (Good syndrome). Intern Med 2008; 48(19):1749-52.

14. Ezzie ME, Janssen WJ, O'Brien JM, Fox CC, Schwarz MI. Failure to Respond. N Engl J Med 2008; 358(1):70-4.

15. Khan S, Campbell A, Hunt C, Sewell WC. Lichen planus in a case of Good's syndrome (thymoma and immunodeficiency). Interact Cardiovasc Thorac Surg 2009; 9(2):345-6.

16. Lee SH, Lee S-M, Yang S-C, Yoo C-G, Kim YW, Shim Y-S, et al. A case of granulomatous lung disease in a patient with Good's syndrome. Korean J Intern Med 2008; 23(4):219-22.

17.Notarangelo, L.D., Primary immunodeficiencies. J Allergy and Clin Immunology, 2010. 125(2): p. S182-S194.

18. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol 2008; 142(4):505-14.

19. Wargo JJ, Kim AH, Hart A, Berg A. It Took a Village: Good's Syndrome. Am J Med 2015; 128(7):699-701.

20. Hirokawa M, Sawada K-i, Fujishima N, Nakao S, Urabe A, Dan K, et al. Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group. Haematologica 2008; 93(1):27-33.

21. Rawat A, Dhir V, Gupta A, Suri D, Burad DK, Nada R, et al. Good's Syndrome Presenting with Recurrent Giardiasis. J Clin Immunol 2014; 34(7):751-2.

22 Nakagawa Y, Murakami K, Hirashita Y, Ogawa R, Hisamatsu A, Mizukami K, et al. A case of Good syndrome with refractory gastrointestinal ulcers. Endoscopy 2012; 44(Suppl 2):E246-E7.

23. Ogoshi T, Ishimoto H, Yatera K, Oda K, Akata K, Yamasaki K, et al. A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. Intern Med 2011; 51(9):1087-91.

24. Hoffacker V, Schultz A, Tiesinga JJ, Gold R, Schalke B, Nix W, et al. Thymomas alter the T-cell subset composition in the blood: a potential mechanism for thymoma-associated autoimmune disease. Blood 2000; 96(12):3872-9.

25. Christopoulos P, Dopfer EP, Malkovsky M, Esser PR, Schaefer H-E, Marx A, et al. A novel thymoma-associated immunodeficiency with increased naive T cells and reduced CD247 expression. J Immunol 2015; 194(7):3045-53.

26. Kisand K, Wolff ASB, Podkrajšek KT, Tserel L, Link M, Kisand KV, et al. Chronic mucocutaneous candidiasis in APECED or thymoma patients correlates with autoimmunity to Th17-associated cytokines. J Exp Med 2010; 207(2):299-308.

27. Lougaris V, Vitali M, Baronio M, Tampella G, Plebani A. BAFF-R mutations in Good's syndrome. Clin Immunol 2014; 153(1):91-3.

28. Sáenz-Cuesta M, Martínez-Pomar N, de Gracia J, Echaniz P, Villegas E, Prada Á, et al. TACI mutation in Good's Syndrome: in search of a genetic basis. Clin Immunol 2012; 145(1):27-30.

29. Tomaszek S, Wigle DA, Keshavjee S, Fischer S. Thymomas: review of current clinical practice. Ann Thorac Surg 2009; 87(6):1973-80.

30. Idress A, Wahla AS. Good syndrome, a rare disease that physicians cannot afford to overlook; case report and review of literature. J Pak Med Assoc 2013; 63(12):1541-3.

31. 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.

32. Assi AC, Lightman S. Cytomegalovirus retinitis in patients with Good syndrome. Arch Ophthalmol 2002; 120(4):510-2.

33 Park DH, Kim SY, Shin JP. Bilateral cytomegalovirus retinitis with unilateral optic neuritis in Good syndrome. Jpn J Ophthalmol 2010; 54(3):246-8.

34. Yong DS, Tsang M, Chan EY, Tse D. Good's syndrome in a patient with cytomegalovirus retinitis. Hong Kong Med J 2008; 14(2):142-4.

35. Downes KM, Tarasewicz D, Weisberg LJ, Cunningham ET. Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients—case report and comprehensive review of the literature. J Ophthalmic Inflamm Infect 2016; 6(1):3.

36. Taylor GH. Cytomegalovirus. Am Fam Physician 2003; 67(3):519-24.

37. Jansen A, van Deuren M, Miller J, Litzman J, de Gracia J, Sáenz-Cuesta M, et al. Prognosis of Good syndrome mortality and morbidity of thymoma associated immunodeficiency in perspective. Clin Immunol. 2016; 171:12-7.


Refbacks

  • There are currently no refbacks.


Creative Commons Attribution-NonCommercial 3.0

This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.