Good’s syndrome (thymoma associated hypogammalobulinaemia) – 2 case reports

Česká verzia

Authors: P. Králíčková ¹; R. Slezák ²; M. Vošmik ³ ; P. Malá ³; H. Hornychová ⁴; J. Brožík ⁵; B. Melichar ⁶
Authors place of work: Ústav klinické imunologie a alergologie Lékařské fakulty UK a FN Hradec Králové, přednosta prof. RNDr. Jan Krejsek, CSc. ¹; Stomatologická klinika Lékařské fakulty UK a FN Hradec Králové, přednosta doc. MUDr. Radovan Slezák, CSc. ²; Klinika onkologie a radioterapie Lékařské fakulty UK a FN Hradec Králové, přednosta prof. MUDr. Jiří Petera, Ph. D. ³; Fingerlandův ústav patologie Lékařské fakulty UK a FN Hradec Králové, přednosta prof. MUDr. Aleš Ryška, Ph. D. ⁴; Radiologická klinika, Fakultní nemocnice a Lékařská fakulta UK v Hradci Králové, prof. MUDr. Pavel Eliáš, CSc. ⁵; Onkologická klinika, Fakultní nemocnice a Lékařská fakulta Olomouc, prof. MUDr. Bohuslav Melichar, Ph. D. ⁶
Published in the journal: Vnitř Lék 2012; 58(2): 154-161
Category: Case Reports

Summary

Although tumours of the thymus are rare, they are common among neoplasms of the anterior superior mediastinum. They usually exhibit indolent behavior, but do have the capacity to invade surrouding structures. Their metastatic potential is low. Paraneoplastic complications including autoimmune disorders (frequently myastenia, cytopenia) or combined immunodeficiency are of clinical significance. Here we report two case reports of thymoma patients associated with secondary immunodeficiency known as Good’s syndrom. The first case exhibited as symptomatic combinated immunodeficiency with oral lichen planus. Thymoma findings was accidental. Severity of immunodeficiency required long-term intravenous immunoglobulins supplementations, even after complete surgical resection. On the other hand, the second case manifested by signs of advanced local tumour growth. Antibiotic prophylaxis was selected as immunodeficiency treatment.

Key words:
thymoma – combined immunodeficiency – Good’s syndrom – lichen planus

Zdroje

1. Babičková L, Skřičková J. Thymom. In: Adam Z, Vorlíček J, Vaníček J et al. Diagnostické a léčebné postupy u maligních chorob. Praha: Grada 2004; 69–73.

2. Tomaszek S, Wigle DA, Keshavjee S et al. Thymomas: Review of current clinical practice. Ann Thorac Surg 2009; 87: 1973–1980.

3. Wright CD. Management of thymomas. Crit Rev Oncol Hematol 2008; 65: 109–120.

4. Rajan A, Giaccone G. Treatment of advanced thymoma and thymic carcinoma. Curr Treat Options Oncol 2008; 9: 277–287.

5. Slováček L, Jebavý L, Kačerovský J. Thymom v klinické praxi. Interní medicína pro praxi 2005; 5: 410–411.

6. Kelesidis T, Yang O. Goods syndrome remains a mystery after 55 years: A systemic review of scientific evidence. Clin Immunol 2010; 135: 347–363.

7. Dřízhal I, Paulusová V. Orální lichen planus – současné poznatky. Čes Stom 2008; 108: 35–38.

8. Kaku Y, Shimamoto N, Matsunaga H et al. Oral erosive lichen planus and alopecia areata with Good’s syndrome (thymoma with hypogammaglobulinaemia). Eur J Dermatol 2011; 21: 124–125.

9. Hayashi A, Shiono H, Okumura M. Thymoma accompanied by lichen planus. Interact Cardiovasc Thorac Surg 2008; 7: 347–347.

10. Bobbio A, Vescovi P, Ampollini L et al. Oral erosive lichen planus regression after thymoma resection. Ann Thorac Surg 2007; 83: 1197–1199.

11. Seneschal J, Orlandini V, Duffau P et al. Oral erosive lichen planus and Good’s syndrome: just a coincidence or a direct link between two diseases. J Eur Acad Dermatol Venerol 2008; 22: 506–507.

12. Moutasim KA, Poate TW, Setterfield JF et al. A case of vulvovaginal gingival lichen planus in association with Good’s syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 105: 57–60.

13. Lolis M, Levitt J. A case of oral lichen planus with Good syndrome. Dermatol Online J 2006; 12: 28.

14. Bajaj DR, Khoso NA, Devrajani BR et al. Oral lichen planus: a clinical study. J Coll Physicians Surg Pak 2010; 20: 154–157.

15. Habbab KM, Moles DR, Porter SR. Potential oral manifestations of cardiovascular drugs. Oral Dis 2010; 16: 769–773.

16. Leibovici V, Zlotogorski A, Heyman A et al. Polymorphous drug eruption due to nefedipine. Cutis 1988; 41: 367.

17. Travis WD, Brambilla E, Müller-Hermelink HK et al. Pathology and genetics of tumors of the lung, pleura, Thymus and heart. In: Travis WD, Brambilla E, Müller-Hermelink HK et al (eds). World Health Organization Classification of Tumours. Lyon: IARC Press 2004.

18. Masaoka A, Monden Y, Nakahara K et al. Follow-up study of thymomas with special reference to their clinical stages. Cancer 1981; 48: 2485–2492.

19. Ciernik IF, Meier U, Lütolf UM. Prognostic factors and outcome of incompletely resected invasive thymoma following radiation therapy. J Clin Oncol 1994; 12: 1484–1490.

20. Skeie GO, Romi F. Paraneoplastic myastenia gravis: immunological and clinical aspects. Eur J Neurol 2008; 15: 1029–1033.

21. Keesey JC. Clinical evaluation and management of myastenia gravis. Muscle Nerve 2004; 29: 484–505.

22. Evoli A, Minicuci GM, Vitaliani R et al. Paraneoplastic diseases associated with thymoma. J Neurol 2007; 254: 756–762.

23. Yamamoto AM, Gajdos P, Eymard B et al. Anti-tinin antibodies in myastenia gravis: tight association with thymoma and heterogenity of nonthymoma patients. Arch Neurol 2001; 58: 885–890.

24. Romi F, Skeie GO, Aarli JA et al. The sverity of myastenia gravis correlates with the serum concentration of tinin and ryanodine receptor antibodies. Arch Neurol 2000; 57: 1596–1600.

25. Okumura M, Inoue M, Kadota Y et al. Biological implications of thymectomy for myastenia gravis. Surg Today 2010; 40: 102–107.

26. Gold R, Schneider-Gold C. Current and future stabdards in treatment of myastenia gravis. Neurotherapeutics 2008; 5: 535–541.

27. Bril V, Kojic J, Dhanani A. The long-term clinical outcome of myastenia gravis in patients with thymoma. Neurology 1998; 51: 1198–1200.

28. Evoli A, Lo Monaco M, Marra R et al. Multiple paraneoplastic disease associated with thymoma. Neuromuscul Disord 1999; 9: 601–603.

29. Khella SL, Souyah N, Dalmau J. Thymoma, myastenia gravis, encephalitis, and a novel anticytoplasmic neuronal antibody. Neurology 2007; 69: 1302–1303.

30. Hammaoud K, Kandimala G, Warnack W et al. Multifocal paraneoplastic cortical encephalitis associated with myastenia gravis and thymoma. Arch Neurol 2009; 66: 1407–1409.

31. Antoine JC, Camdessanché JP, Absi L et al. Devic disease and thymoma with anti-central nervous system and antithymus antibodies. Neurology 2004; 62: 978–980.

32. Shiraishi J, Tsugata M, Masuda R et al. Type AB thymoma accompanied by pure red cell aplasia and Good syndrome with CMV infection of tumor cells. Pathol Int 2008; 58: 489–493.

33. Karras A, deMontpreville V, Fakhouri F et al. Groupe d‘Etudes des Néphropathies Associées aux Thymomes. Renal and thymic pathology in thymoma-associated nephropathy: report of 21 cases and review of the literature. Nephrol Dial Transplant 2005; 20: 1075–1082.

34. Wadhera A, Maverakis E, Mitsiades N et al. Thymoma-associated multiorgan autoimmunity: A graft-versus-host-like disease. J Am Acad Dermatol 2007; 57: 683–689.

35. Kelleher P, Misbah SA. What is Good’s syndome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003; 56: 12–16.

36. Koriyama N, Fukumoto O, Fukudome M et al. Successful treatment of Good syndrome with cytomegalovirus duaodenoenteritis using a combination of ganciclovir and immunoglobuin with high anti-cytomegaovirus antibody titer. Am J Med Sci 2004; 327: 49–54.

37. Sen HN, Robinson MR, Fisher SH. CMV retinitis in patient with Good syndrome. Ocul Immunol Inflamm 2005; 13: 475–478.

38. Striano P, Tortora F, Evoli A et al. Periodic myoclonus due to cytomegalovirus encephalitis in patient with good syndrome. Arch Neurol 2007; 64: 277–279.

39. Lai WH, Lu SY, Eng HL. Levamisole aid in treatment of refractory oral candidiasis in two patients with thymoma associated with myastenia gravis: report of two cases. Chang Gung Med J 2002; 25: 606–611.

40. Agarwal S, Cunningham-Rundles C. Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of literature. Ann Allergy Asthma Immunol 2007; 98: 185–190.

41. Tarr PE, Sneller MC, Mechanic LJ et al. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore) 2001; 80: 123–133.

42. Montela L, Masci AM, Merkabaoui G et al. B-cell lymphopenia and hypogammaglobulinaemia in thymoma patients. Ann Hematol 2003; 82: 343–347.

43. Krawczyk P, Adamcyk-Korbel M, Kieszko R et al. Immunological system status and the appearance of respiratory system disturbances in thymectomized patients. Arch Immunol Ther Exp 2007; 55: 49–56.

44. Oritani K, Medina KL, Tomiyama Y et al. Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors. Nat Med 2000; 6: 659–699.

45. Litwin SD, Zanjani ED. Lymphocyte suppressing both immunoglobulin and erythroid differentiation in hypogammaglobulinaemia. Nature 1977; 266: 57–58.

46. Shiono H, Wong YL, Matthews I et al. Spontaneous production of anti-IFN-alfa and anti-IL-12 autoantibodies by thymoma cells from myastenia gravis patients suggests autoimmunisation in the tumor. Inter Immunol 2003; 15: 903–913.

47. Raschal S, Siegel JN, Huml J et al. Hypogammaglobulinaemia and anemia 18 years after thymoma resection. J Allergy Clin Immunol 1997; 100: 846–848.

48. Vincent A, Willcox N. The role of T-cells in the initiation of autoantibody response in thymoma patients. Pathol Res Pract 1999; 195: 535–540.

49. Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survay of clinical manifestations and complications. Q J Med 1993; 86: 31–42.