Abstract
Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good’s syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good’s syndrome. Lethal opportunistic infections are a feature of Good’s syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good’s syndrome. We now describe a 70 year old man with Good’s syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
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 (Baltimore). 2001;80(2):123–33.
Crawford WW, Yusin JS, Klaustermeyer WB. Nonsurgical regression of thymoma following corticosteroid/azathioprine therapy. Ann Allergy Asthma Immunol. 1999;83(1):13–6. doi:10.1016/S1081-1206(10)63505-3.
Degos L, Faille A, Housset M, Boumsell L, Rabian C, Parames T. Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma. Blood. 1982;60(4):968–72.
Federico P, Imbimbo M, Buonerba C, Damiano V, Marciano R, Serpico D, et al. Is hypogammaglobulinemia a constant feature in Good’s syndrome? Int J Immunopathol Pharmacol. 2010;23(4):1275–9.
Ohuchi M, Inoue S, Hanaoka J, Igarashi T, Tezuka N, Ozaki Y, et al. Good syndrome coexisting with leukopenia. Ann Thorac Surg. 2007;84(6):2095–7. doi:10.1016/j.athoracsur.2007.06.070.
Jethava Y, Alamelu J, Rangarajan S, Lang-Lazdunski L, van der Walt J, Fields P. Acquired agranulocytosis and factor XI deficiency in association with thymoma. J Clin Oncol. 2011;29(20):e604–6. doi:10.1200/JCO.2010.34.3707.
Jeandel C, Gastin I, Blain H, Jouanny P, Laurain MC, Penin F, et al. Thymoma with immunodeficiency (Good’s syndrome) associated with selective cobalamin malabsorption and benign IgM-kappa gammopathy. J Intern Med. 1994;235(2):179–82.
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. doi:10.1016/j.clim.2010.01.006.
Rogers BH, Manligod JR, Blazek WV. Thymoma associated with pancytopenia and hypogammaglobulinemia. Report of a case and review of the literature. Am J Med. 1968;44(1):154–64.
Yip D, Rasko JE, Lee C, Kronenberg H, O’Neill B. Thymoma and agranulocytosis: two case reports and literature review. Br J Haematol. 1996;95(1):52–6.
Kelleher P, Misbah SA. What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003;56(1):12–6.
Postiglione K, Ferris R, Jaffe JP, Stroncek D. Immune mediated agranulocytosis and anemia associated with thymoma. Am J Hematol. 1995;49(4):336–40.
Thiele HG, Frenzel H. Immunoglobulin deficiency syndrome and agranulocytosis in thymoma [in German]. Schweiz Med Wochenschr. 1967;97:1606–11.
Jacobson BM, Castleman B. Anaemia, granulocytopenia, and terminal sepsis in a 70-year-old woman. N Engl J Med. 1971;284:39–47.
Young RH. Thymoma with agranulocytosis and hypogammaglobulinaemia. Ir J Med Sci. 1977;146(8):266–8.
Sugiura Y, Itoh K, Hakumei O, Asai T, Fujioka S, Kumagaya A. A case of pure red cell aplasia associated with thymoma, granulocytopenia, hypogammaglobulinemia, and hypothyroidism. Rinsho Ketsueki. 1982;23(5):700–7.
Ackland SP, Bur ME, Adler SS, Robertson M, Baron JM. White blood cell aplasia associated with thymoma. Am J Clin Pathol. 1988;89(2):260–3.
Weir 3rd AB, Dow LW. Response of agranulocytosis to thymectomy in a patient with thymoma and chronic lymphocytic leukemia. Med Pediatr Oncol. 1989;17(1):58–61.
El-Galaly TC, Gregersen H, Bukh A. Good’s syndrome with lymphopenia and neutropenia. Ugeskr Laeger. 2011;173(4):280–1.
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.
Tumours of the thymus in Travis W, Brambilla E, Muller-Hermelink HK, Harris CC. Pathology genetics: Tumours of the lung, pleura, thymus and heart. World Health Organization Classification of tumours. Lyon: IARC Pres; 2004.
Courville P, Favennec L, Viacroze C, Barrel A, Young P, Abboud P, et al. Co-existent cutaneous cryptococcosis of the forearm and cutaneous alternariosis of the leg in patient with metastatic thymoma. J Cutan Pathol. 2002;29(1):55–8.
Kishi K, Homma S, Nakatani T, Nakata K. A clinical study of cryptococcal meningitis—sequential changes of cryptococcal antigen titers. Kansenshogaku Zasshi. 2003;77(3):150–7.
Rowland LP, Griffiths CO, Kabat EA. Myasthenia gravis, thymoma and cryptococcal meningitis. N Engl J Med. 1965;273(12):620–7. doi:10.1056/NEJM196509162731202.
Schmidt S, Padberg F. Late onset immunodeficiency in a patient with recurrent thymic carcinoma and myasthenia gravis. J Neurol Sci. 1998;157(2):201–5.
Park BJ, Wannemuehler KA, Marston BJ, Govender N, Pappas PG, Chiller TM. Estimation of the current global burden of cryptococcal meningitis among persons living with HIV/AIDS. AIDS. 2009;23(4):525–30. doi:10.1097/QAD.0b013e328322ffac.
Centers, for, Disease, Control, and, Prevention. C. neoformans cryptococcosis. http://www.cdc.gov/fungal/cryptococcosis-neoformans/. Accessed 27 Jan 2013.
Bodem CR, Hamory BH, Taylor HM, Kleopfer L. Granulomatous bone marrow disease. A review of the literature and clinicopathologic analysis of 58 cases. Medicine (Baltimore). 1983;62(6):372–83.
Pantanowitz L, Omar T, Sonnendecker H, Karstaedt AS. Bone marrow cryptococcal infection in the acquired immunodeficiency syndrome. J Infect. 2000;41(1):92–4. doi:10.1053/jinf.2000.0667.
Rosai J. In: Ackerman’s Surgical Pathology, editor. Bone marrow. Singapore: Harcourt Brace & Co Asia Pte Ltd; 1996. p. 1806–14.
Kontoyiannis DP, Peitsch WK, Reddy BT, Whimbey EE, Han XY, Bodey GP, et al. Cryptococcosis in patients with cancer. Clin Infect Dis. 2001;32(11):E145–50. doi:10.1086/320524.
Feldmesser M, Harris C, Reichberg S, Khan S, Casadevall A. Serum cryptococcal antigen in patients with AIDS. Clin Infect Dis. 1996;23(4):827–30.
Shelly S, Agmon-Levin N, Altman A, Shoenfeld Y. Thymoma and autoimmunity. Cell Mol Immunol. 2011;8(3):199–202. doi:10.1038/cmi.2010.74.
Acknowledgments
The authors would like to thank Dr David Webster for critical appraisal of our manuscript and fruitful feedback.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Akinosoglou, K., Melachrinou, M., Siagris, D. et al. Good’s Syndrome and Pure White Cell Aplasia Complicated by Cryptococcus Infection: A Case Report and Review of the Literature. J Clin Immunol 34, 283–288 (2014). https://doi.org/10.1007/s10875-014-0014-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10875-014-0014-7