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Published in: Journal of Medical Case Reports 1/2021

Open Access 01-12-2021 | Anemia | Case report

Pure red cell aplasia secondary to rheumatoid arthritis: a case report

Authors: Suneth Weerasinghe, Parackrama Karunathilake, Udaya Ralapanawa, Thilak Jayalath, Shamali Abeygunawardena, Manel Rathnayaka

Published in: Journal of Medical Case Reports | Issue 1/2021

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Abstract

Background

Rheumatoid arthritis is a common autoimmune disease with many extra-articular manifestations. Pure red cell aplasia is a rare manifestation of rheumatoid arthritis and is sparsely documented in the literature, with a variable clinical outcome following immunosuppressive therapy.

Case presentation

A 63-year-old Sinhalese female presented with transfusion-dependent anemia associated with deforming inflammatory arthritis. She also had leukopenia, right subclavian venous thrombosis, and generalized lymphadenopathy. The diagnosis of rheumatoid arthritis following initial clinical workup and additional blood and bone marrow investigations revealed pure red cell aplasia as a secondary manifestation of rheumatoid arthritis after excluding other secondary causes, such as infections, thymoma, thrombophilic conditions, and hematological malignancy. She responded well to oral prednisolone, cyclosporine A, and hydroxychloroquine, and she attained complete recovery in 2 months.

Conclusion

Pure red cell aplasia is a disabling illness that may lead to transfusion-dependent anemia, which may occur due to rare extrapulmonary manifestation of rheumatoid arthritis. The diagnosis of pure red cell aplasia secondary to rheumatoid arthritis may be challenging where hematological investigations, including bone marrow biopsy, will aid in the diagnosis, and early diagnosis and treatment will bring about a better outcome.
Literature
1.
go back to reference Bullock J, Rizvi SAA, Saleh AM, et al. Rheumatoid arthritis: a brief overview of the treatment. Med Princ Pract. 2019;27:501–7.CrossRef Bullock J, Rizvi SAA, Saleh AM, et al. Rheumatoid arthritis: a brief overview of the treatment. Med Princ Pract. 2019;27:501–7.CrossRef
2.
go back to reference Koch AE. The pathogenesis of rheumatoid arthritis. Am J Orthop (Belle Mead NJ). 2007;36(7 Suppl):5–8. Koch AE. The pathogenesis of rheumatoid arthritis. Am J Orthop (Belle Mead NJ). 2007;36(7 Suppl):5–8.
3.
go back to reference Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142(4):505–14.CrossRef Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142(4):505–14.CrossRef
4.
go back to reference Parida PK, Shukla SN, Talati SS, et al. Acquired pure red cell aplasia in a patient of rheumatoid arthritis. Indian J Hematol Blood Transfus. 2014;30:255–7.CrossRef Parida PK, Shukla SN, Talati SS, et al. Acquired pure red cell aplasia in a patient of rheumatoid arthritis. Indian J Hematol Blood Transfus. 2014;30:255–7.CrossRef
5.
go back to reference Dessypris EN, Baer MR, Sergent JS, et al. Rheumatoid arthritis and pure red cell aplasia. Ann Intern Med. 1984;100:202–6.CrossRef Dessypris EN, Baer MR, Sergent JS, et al. Rheumatoid arthritis and pure red cell aplasia. Ann Intern Med. 1984;100:202–6.CrossRef
7.
go back to reference Aletaha D, Neogi T, Silman AJ, et al. 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2010;62:2569–81.CrossRef Aletaha D, Neogi T, Silman AJ, et al. 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2010;62:2569–81.CrossRef
8.
go back to reference Rajesh K. A case report on acquired pure red cell aplasia in a patient of rheumatoid arthritis. J Med Sci Clin Res. 2016;04:9160–2. Rajesh K. A case report on acquired pure red cell aplasia in a patient of rheumatoid arthritis. J Med Sci Clin Res. 2016;04:9160–2.
9.
go back to reference Rodrigues JF, Harth M, Barr RM. Pure red cell aplasia in rheumatoid arthritis. J Rheumatol. 1988;15:1159–61.PubMed Rodrigues JF, Harth M, Barr RM. Pure red cell aplasia in rheumatoid arthritis. J Rheumatol. 1988;15:1159–61.PubMed
11.
go back to reference Markusse HM, Breedveld FC. Rheumatoid arthritis with eosinophilic fasciitis and pure red cell aplasia. J Rheumatol. 1989;16:1383–4.PubMed Markusse HM, Breedveld FC. Rheumatoid arthritis with eosinophilic fasciitis and pure red cell aplasia. J Rheumatol. 1989;16:1383–4.PubMed
12.
go back to reference Harper K, McIlwaine L, Leach M, et al. Giant proerythroblasts in pure red cell aplasia due to parvovirus B19 infection in a patient with rheumatoid arthritis. Am J Hematol. 2020;95:990–1.CrossRef Harper K, McIlwaine L, Leach M, et al. Giant proerythroblasts in pure red cell aplasia due to parvovirus B19 infection in a patient with rheumatoid arthritis. Am J Hematol. 2020;95:990–1.CrossRef
13.
go back to reference Sinha AK, Agarwal A, Lakhey M, et al. Pure red cell aplasia—report of 11 cases from eastern Nepal. Indian J Pathol Microbiol. 2003;46:405–8.PubMed Sinha AK, Agarwal A, Lakhey M, et al. Pure red cell aplasia—report of 11 cases from eastern Nepal. Indian J Pathol Microbiol. 2003;46:405–8.PubMed
14.
go back to reference Singh RR, Malviya AN, Kapoor SK, et al. Pure red cell aplasia with rheumatoid arthritis. A case report and review of literature. J Assoc Physicians India. 1990;38:233–5.PubMed Singh RR, Malviya AN, Kapoor SK, et al. Pure red cell aplasia with rheumatoid arthritis. A case report and review of literature. J Assoc Physicians India. 1990;38:233–5.PubMed
15.
go back to reference Banavali SD, Parikh PM, Charak BS, et al. Corticosteroid-responsive pure red cell aplasia in rheumatoid arthritis and its association with pregnancy: a case report. Am J Hematol. 1989;31:58–61.CrossRef Banavali SD, Parikh PM, Charak BS, et al. Corticosteroid-responsive pure red cell aplasia in rheumatoid arthritis and its association with pregnancy: a case report. Am J Hematol. 1989;31:58–61.CrossRef
16.
go back to reference Tsai CY, Yu CL, Tsai YY, et al. Pure red cell aplasia in a man with RA. Scand J Rheumatol. 1997;26:329–31.CrossRef Tsai CY, Yu CL, Tsai YY, et al. Pure red cell aplasia in a man with RA. Scand J Rheumatol. 1997;26:329–31.CrossRef
17.
go back to reference Tishler M, Kahn Y, Yaron M. Pure red cell aplasia caused by d-penicillamine treatment of rheumatoid arthritis. Ann Rheum Dis. 1991;50:255–6.CrossRef Tishler M, Kahn Y, Yaron M. Pure red cell aplasia caused by d-penicillamine treatment of rheumatoid arthritis. Ann Rheum Dis. 1991;50:255–6.CrossRef
18.
go back to reference Fisch P, Handgretinger R, Schaefer HE. Pure red cell aplasia. Br J Haematol. 2000;111:1010–22.CrossRef Fisch P, Handgretinger R, Schaefer HE. Pure red cell aplasia. Br J Haematol. 2000;111:1010–22.CrossRef
19.
go back to reference Masuda M, Teramura M, Matsuda A, et al. Clonal T cells of pure red-cell aplasia. Am J Hematol. 2005;79:332–3.CrossRef Masuda M, Teramura M, Matsuda A, et al. Clonal T cells of pure red-cell aplasia. Am J Hematol. 2005;79:332–3.CrossRef
20.
go back to reference Teramura M. Pure red cell aplasia. Ryoikibetsu Shokogun Shirizu. Wiley-Blackwell: Hoboken; 2000. p. 178–80. Teramura M. Pure red cell aplasia. Ryoikibetsu Shokogun Shirizu. Wiley-Blackwell: Hoboken; 2000. p. 178–80.
24.
go back to reference Hirokawa M, Sawada K, Fujishima N, et al. Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group. Br J Haematol. 2015;169:879–86.CrossRef Hirokawa M, Sawada K, Fujishima N, et al. Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group. Br J Haematol. 2015;169:879–86.CrossRef
25.
go back to reference Saito E, Sato S, Nogi S, et al. A case of rheumatoid arthritis and limited systemic sclerosis overlap successfully treated with tocilizumab for arthritis and concomitant generalized lymphadenopathy and primary biliary cirrhosis. Case Rep Rheumatol. 2014;2014:1–5. Saito E, Sato S, Nogi S, et al. A case of rheumatoid arthritis and limited systemic sclerosis overlap successfully treated with tocilizumab for arthritis and concomitant generalized lymphadenopathy and primary biliary cirrhosis. Case Rep Rheumatol. 2014;2014:1–5.
26.
go back to reference Chung WS, Peng CL, Lin CL, et al. Rheumatoid arthritis increases the risk of deep vein thrombosis and pulmonary thromboembolism: a nationwide cohort study. Ann Rheum Dis. 2014;73:1774–80.CrossRef Chung WS, Peng CL, Lin CL, et al. Rheumatoid arthritis increases the risk of deep vein thrombosis and pulmonary thromboembolism: a nationwide cohort study. Ann Rheum Dis. 2014;73:1774–80.CrossRef
27.
go back to reference Choi HK, Rho YH, Zhu Y, et al. The risk of pulmonary embolism and deep vein thrombosis in rheumatoid arthritis: a UK population-based outpatient cohort study. Ann Rheum Dis. 2013;72:1182–7.CrossRef Choi HK, Rho YH, Zhu Y, et al. The risk of pulmonary embolism and deep vein thrombosis in rheumatoid arthritis: a UK population-based outpatient cohort study. Ann Rheum Dis. 2013;72:1182–7.CrossRef
28.
go back to reference Robertson MD, Hart FD, White WF, et al. Rheumatoid lymphadenopathy. Ann Rheum Dis. 1968;27:253–60.CrossRef Robertson MD, Hart FD, White WF, et al. Rheumatoid lymphadenopathy. Ann Rheum Dis. 1968;27:253–60.CrossRef
29.
go back to reference Çalgüneri M, Öztürk MA, Özbalkan Z, et al. Frequency of lymphadenopathy in rheumatoid arthritis and systemic lupus erythematosus. J Int Med Res. 2003;31:345–9.CrossRef Çalgüneri M, Öztürk MA, Özbalkan Z, et al. Frequency of lymphadenopathy in rheumatoid arthritis and systemic lupus erythematosus. J Int Med Res. 2003;31:345–9.CrossRef
30.
go back to reference Motulsky AG, Weinberg S, Saphir O, et al. Lymph nodes in rheumatoid arthritis. AMA Arch Intern Med. 1952;90:660–76.CrossRef Motulsky AG, Weinberg S, Saphir O, et al. Lymph nodes in rheumatoid arthritis. AMA Arch Intern Med. 1952;90:660–76.CrossRef
31.
go back to reference Petri M, Orbai AM, Alarcõn GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677–86.CrossRef Petri M, Orbai AM, Alarcõn GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677–86.CrossRef
32.
go back to reference Charles RJ, Sabo KM, Kidd PG, et al. The pathophysiology of pure red cell aplasia: implications for therapy. Blood. 1996;87:4831–8.CrossRef Charles RJ, Sabo KM, Kidd PG, et al. The pathophysiology of pure red cell aplasia: implications for therapy. Blood. 1996;87:4831–8.CrossRef
33.
go back to reference Grigg AP, O’flaherty E. Cyclosporin a for the treatment of pure red cell aplasia associated with myelodysplasia. Leuk Lymphoma. 2001;42:1339–42.CrossRef Grigg AP, O’flaherty E. Cyclosporin a for the treatment of pure red cell aplasia associated with myelodysplasia. Leuk Lymphoma. 2001;42:1339–42.CrossRef
34.
go back to reference Wu X, Wang S, Shen W, et al. Adult patients with acquired pure red cell aplasia: treated by cyclosporine A and/or corticosteroids-single center experience. Blood. 2016;128:4818–4818.CrossRef Wu X, Wang S, Shen W, et al. Adult patients with acquired pure red cell aplasia: treated by cyclosporine A and/or corticosteroids-single center experience. Blood. 2016;128:4818–4818.CrossRef
Metadata
Title
Pure red cell aplasia secondary to rheumatoid arthritis: a case report
Authors
Suneth Weerasinghe
Parackrama Karunathilake
Udaya Ralapanawa
Thilak Jayalath
Shamali Abeygunawardena
Manel Rathnayaka
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2021
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-021-03141-5

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