Thymic hyperplasia as a rare etiology of pure red cell aplasia

  • Isidora Arsenović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Danijela Leković University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia; University of Belgrade, Faculty of Medicine, Belgrade, Serbia
  • Dijana Šefer University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Jelena Ivanović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Mihajlo Smiljanić University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Andrija Bogdanović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia; University of Belgrade, Faculty of Medicine, Belgrade, Serbia
Keywords: anemia, diagnosis, histological techniques, red-cell aplasia, pure, thoracic surgery, video-assisted, thymoma, thymus hyperplasia

Abstract


 

Introduction. Thymic hyperplasia is a rare condition caused by an increase in cellular thymic mass and, in some cases, is associated with autoimmune diseases, such as pure red cell aplasia (PRCA). Thymectomy is considered the most effective therapy for PRCA associated with thymoma, with a 31.5% complete remission rate. Other treatments may induce partial remissions, but complete remission remains elusive. A case of PRCA attributed to thymic hyperplasia is presented, highlighting the effectiveness of thymectomy. Case report. A previously healthy 18-year-old woman presented with severe anemia and after hematological evaluation, including bone marrow biopsy confirmation, a diagnosis of PRCA was made. Immunological and virological analyses were unremarkable. Given the history of thymoma in the family and the known association between thymoma and PRCA, a chest magnetic resonance imaging was performed, which proved the existence of thymic hyperplasia. The patient underwent the least invasive surgical procedure – total thymectomy using video-assisted thoracic surgery approach. Pathohistological examination of the operative material confirmed the presence of thymic hyperplasia with a simple intrathymic cyst. Following thymectomy, the patient’s hematological values significantly improved. Conclusion. The course and outcome of the patient’s treatment support the role of thymectomy in PRCA associated with thymic hyperplasia. However, further research and follow-up are needed to optimize management strategies for this rare condition.

References

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

Mangla A, Hamad H. Pure Red Cell Aplasia [updated 2022 Nov 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 July 13]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK549833/

Khan MA, Anjum F. Thymic Hyperplasia [updated 2023 Apr 27]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 July 15]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK5605

/

Shichkin VP, Antica M. Key Factors for Thymic Function and Development. Front Immunol 2022; 13: 926516.

Ackman JB, Wu CC. MRI of the thymus. AJR Am J Roent-genol. 2011; 197(1): W15–20.

Den Bakker MA, Oosterhuis JW. Tumours and tumour-like conditions of the thymus other than thymoma; a practical ap-proach. Histopathology 2009; 54(1): 69–89.

Le Panse R, Bismuth J, Cizeron-Clairac G, Weiss JM, Cufi P, Dartevelle P, et al. Thymic remodeling associated with hyperplasia in myasthenia gravis. Autoimmunity 2010; 43(5–6): 401–12.

Haider U, Richards P, Gianoukakis AG. Thymic Hyperplasia Associated with Graves' Disease: Pathophysiology and Proposed Management Algorithm. Thyroid 2017; 27(8): 994–1000.

de Castro MA, de Castro MA, Arantes Ade M, Roberti Mdo R. Thymoma followed by aplastic anemia – two different re-sponses to immunosuppressive therapy. Rev Bras Hematol Hemoter 2011; 33(6): 476–7.

Mohammad A, Dawson AG, Bajaj A, Rathinam S. True thymic hyperplasia causing pure red cell aplasia: a case report. Interact Cardiovasc Thorac Surg 2022; 34(4): 697–9. Erratum in: Interact Cardiovasc Thorac Surg 2022.

Konstantopoulos K, Androulaki A, Aessopos A, Patsouris E, Dosios TH, Psychogios A, et al. Pure red cell aplasia associated with true thymic hyperplasia. Hum Pathol 1995; 26(10): 1160–2.

Suto Y, Araga S, Sakuma K, Nakano T, Ishiga K, Tajima F, et al. Myasthenia gravis with thymus hyperplasia and pure red cell aplasia. J Neurol Sci 2004; 224(1–2): 93–5.

Wong KF, Chau KF, Chan JK, Chu YC, Li CS. Pure red cell aplasia associated with thymic lymphoid hyperplasia and secondary erythropoietin resistance. Am J Clin Pathol 1995; 103(3): 346–7.

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(3): 405–7.

Masuda M, Arai Y, Okamura T, Mizoguchi H. Pure red cell apla-sia with thymona: evidence of T-cell clonal disorder. Am J Hematol 1997; 54(4): 324–8.

Bakrac M, Jurisic V, Kostic T, Popovic V, Pekic S, Kraguljac N, et al. Pure red cell aplasia associated with type I autoimmune polyglandular syndrome-successful response to treatment with mycophenolate mofetil: case report and review of literature. J Clin Pathol 2007; 60(6): 717–20.

Masaoka A, Hashimoto T, Shibata K, Yamakawa Y, Nakamae K, Iizuka M. Thymomas associated with pure red cell aplasia. His-tologic and follow-up studies. Cancer 1989; 64(9): 1872–8.

Lesire B, Durieux V, Grigoriu B, Girard N, Berghmans T. Man-agement of thymoma associated autoimmune pure red cell aplasia: Case report and systematic review of the litera-ture. Lung Cancer 2021; 157: 131–46.

Infante M, Benato C, Giovannetti R, Bonadiman C, Canneto B, Fa-lezza, et al. VATS thymectomy for early stage thymoma and myasthenia gravis: combined right-sided uniportal and left-sided three-portal approach. J Vis Surg 2017; 3: 144.

Published
2024/01/30
Section
Case report