Subscribe to RSS

DOI: 10.4103/0971-5851.195729
Thymoma masquerading as transfusion dependent anemia
Financial support and sponsorship Nil.
Abstract
Pure red cell aplasia (PRCA) is a known entity in clinical medicine. Patients are often transfusion dependent for their whole life. Ascertaining its etiology is always a herculean task. We received a similar transfusion-dependent patient, who on evaluation was found to have thymoma as an etiological factor. Thymoma presenting as PRCA is seen in 2%–5% patients and evaluating PRCA for thymoma is seen in 5%–13% patient. As per the WHO histopathological classification, thymoma has six types and Type A is associated with PRCA and Type B is associated with myasthenia gravis. This correlation was not seen in our patient, who had Type B thymoma. Surgical resection of thymus improves 30% of PRCA and rest needs immunosuppression. Our patient was not the surgical candidate, and hence he was put on chemotherapy.
Keywords
Compound mean action potential - large granular leukemia - myasthenia gravis - pure red cell aplasia - repeated nerve stimulationPublication History
Article published online:
12 July 2021
© 2016. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
- 1 Dessypris EN. The biology of pure red cell aplasia. Semin Hematol 1991;28:275-84.
- 2 Thompson CA, Steensma DP. Pure red cell aplasia associated with thymoma: Clinical insights from a 50-year single-institution experience. Br J Haematol 2006;135:405-7.
- 3 Suzuki S, Utsugisawa K, Suzuki N. Overlooked non-motor symptoms in myasthenia gravis. J Neurol Neurosurg Psychiatry 2013;84:989-94.
- 4 van der Marel J, Pahlplatz PV, Steup WH, Hendriks ER. Thymoma with paraneoplastic syndromes, Good's syndrome, and pure red cell aplasia. J Thorac Oncol 2007;2:325-6.
- 5 Bhargava R, Dolai TK, Singhal D, Mahapatra M, Mishra P, Rathod N, et al. Pure red cell aplasia associated with thymoma: Is thymectomy the cure? Leuk Res 2009;33:e17-8.
- 6 Kaznelson P. For genesis of the platelet. Ratio Dtsch Ges Inn Med 1922;34:557-8.
- 7 Dessypris EN. Pure Red Cell Aplasia. Baltimore, London: The Johns Hopkins University Press; 1988.
- 8 Dessypris EN, Lipton JM. Red cell aplasia. In: Greer JP, Foerster J, Lukens JN, Rogers GM, Paraskevas MD, Glader B, editors. Wintrobe's Clinical Hematology. 11th ed. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 1421-7.
- 9 Charles RJ, Sabo KM, Kidd PG, Abkowitz JL. The pathophysiology of pure red cell aplasia: Implications for therapy. Blood 1996;87:4831.
- 10 Lacy MQ, Kurtin PJ, Tefferi A. Pure red cell aplasia: Association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities. Blood 1996;87:3000-6.
- 11 Krantz SB. Acquired pure red cell aplasia. In: Hoffman R, Benz EJ Jr., editors. Hematology Basic Principles and Practice. New York: Churchill Livingstone; 1991. p. 172.
- 12 Oosterhuis HJ, Feltkamp TE, van Rossum AL, van den Berg-Loonen PM, Nijenhuis LE. HL-A antigens, autoantibody production, and associated diseases in thymoma patients, with and without myasthenia gravis. Ann N Y Acad Sci 1976;274:468-74.
- 13 Charles RJ, Sabo KM, Kidd PG, Abkowitz JL. The pathophysiology of pure red cell aplasia: Implications for therapy. Blood 1996;87:4831-8.
- 14 Lardinois D, Rechsteiner R, Läng RH, Gugger M, Betticher D, von Briel C, et al. Prognostic relevance of Masaoka and Müller-Hermelink classification in patients with thymic tumors. Ann Thorac Surg 2000;69:1550-5.
- 15 Schneider PM, Fellbaum C, Fink U, Bollschweiler E, Präuer HW. Prognostic importance of histomorphologic subclassification for epithelial thymic tumors. Ann Surg Oncol 1997;4:46-56.
- 16 Teramura M, Kimura A, Iwase S, Yonemura Y, Nakao S, Urabe A, et al. Treatment of severe aplastic anemia with antithymocyte globulin and cyclosporin A with or without G-CSF in adults: A multicenter randomized study in Japan. Blood 2007;110:1756-61.