Abstract
Thymomas are often associated with a variety of autoimmune diseases, mostly myasthenia gravis. The association of thymomas with both pure red cell aplasia (PRCA) and Good’s syndrome is exceedingly rare. To the best of our knowledge, the combination of a thymoma with manifestations of myasthenia gravis, PRCA, and Good’s syndrome, as in our case herein, has not been described before in the medical literature. We present a 90-year-old man initially diagnosed with an asymptomatic thymoma. Later, he developed generalized muscle weakness and was found to have severe anemia. He was diagnosed with PRCA, myasthenia gravis and Good’s syndrome. He responded to rituximab with restoration of bone marrow erythroid maturation and stabilization of red blood cell counts.
This is a preview of subscription content, log in via an institution to check access.
References
Rosai J, Sobin LH (1999) Histological typing of tumours of the thymus. Springer, New York, p 1–16
Kondo K, Yoshizawa K, Tsuyuguchi M, Kimura S, Sumitomo M, Morita J, Miyoshi T, Sakiyama S, Mukai K, Monden Y (2004) WHO histologic classification is a prognostic indicator in thymoma. Ann Thorac Surg 77:1183–1188
Muller-Hermelink HK, Marx A (2000) Thymoma. Curr Opin Oncol 12:426–433
Shelly S, Agmon-Levin N, Altman A, Shoenfeld Y (2011) Thymoma and autoimmunity. Cell Mol Immunol 8:199–202
Hoffacker V, Schultz A, Tiesinga JJ, Gold R, Schalke B, Nix W, Kiefer R, Müller-Hermelink HK, Marx A (2000) Thymomas alter the T-cell subset composition in the blood: a potential mechanism for thymoma-associated autoimmune disease. Blood 96:3872–3879
Lin CS, Yu YB, Hsu HS, Chou TY, Hsu WH, Huang BS (2009) Pure red cell aplasia and hypogammaglobulinemia in a patient with thymoma. J Chin Med Assoc 72:34–38
Chen J, Yang Y, Zhu D, Chen G, Wei S, Qiu X, Zhou Q (2011) Thymoma with pure red cell aplasia and Good’s syndrome. Ann Thorac Surg 91:1620–1622
van der Marel J, Pahlplatz PV, Steup WH, Hendriks ER (2007) Thymoma with paraneoplastic syndromes, Good’s syndrome, and pure red cell aplasia. J Thorac Oncol 2:325–326
Shiraishi J, Tsugata M, Masuda R, Mori Y, Suzuki K, Takemura T (2008) Type AB thymoma accompanied by pure red cell aplasia and Good syndrome with CMV infection of tumor cells. Pathol Int 58:489–493
Zeok JV, Todd EP, Dillon M, DeSimone P, Utley JR (1979) The role of thymectomy in red cell aplasia. Ann Thorac Surg 28:257–260
Kelleher P, Misbah S (2003) What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 56:12–16
Mamiya S, Itoh T, Miura AB (1997) Acquired pure red cell aplasia in Japan. Eur J Haematol 59:199–205
Thompson CA, Steensma DP (2006) Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institution experience. Br J Haematol 135:405–407
Garcia Vela JA, Monteserin MC, Ona F, Barea LM, Lastra A, Perez V (1993) Cyclosporine A used as a single drug in the treatment of pure red cell aplasia associated with thymoma. Am J Hematol 42:238–239
Pantelidou D, Tsatalas C, Margaritis D, Kaloutsi V, Spanoudakis E, Bourikas G (2004) Anti-CD20 monoclonal antibody rituximab for the treatment of B-cell chronic lymphocytic leukemia-associated pure red cell aplasia. Hematol J 5:546–547
Narra K, Borghaei H, Al-Saleem T, Hoglund M, Smith MR (2006) Pure red cell aplasia in B-cell lymphoproliferative disorder treated with rituximab: report of two cases and review of the literature. Leuk Res 30:109–114
Dungarwalla M, Marsh JC, Tooze JA, Lucas G, Ouwehand W, Pettengell R, Dearden CE, Gordon Smith EC, Elebute MO (2007) Lack of clinical efficacy of rituximab in the treatment of autoimmune neutropenia and pure red cell aplasia: implications for their pathophysiology. Ann Hematol 86:191–197
Disclosure
Institutional review board/Ethics committee approval for case report publication is not needed as per our hospital regulations.
Conflict of interest
All authors declare they do not have any competing interests to disclose.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Antar, A.I., Otrock, Z.K., Kharfan-Dabaja, M.A. et al. Thymoma with Concomitant Pure Red Cell Aplasia, Good’s Syndrome and Myasthenia Gravis Responding to Rituximab. Indian J Hematol Blood Transfus 32 (Suppl 1), 219–222 (2016). https://doi.org/10.1007/s12288-014-0478-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12288-014-0478-x