Autoimmune Hemolytic Anemia in Systemic Lupus Erythematosus: Clinical Presentation, Diagnostic Challenges, and Management Strategies
Ramesh T V1, Gopi Ayyaswamy2, Zioni Sangeetha3 and Vinod Kumar P 4*
Journal of Angiotherapy 6(1) 1-5 https://doi.org/10.25163/angiotherapy.6162187290707122
Submitted: 29 November 2021 Revised: 14 December 2021 Published: 07 January 2022
This case determines the importance of early diagnosis and treatment of rare cold agglutinin-induced hemolytic anemia in systemic lupus erythematosus.
Abstract
Background: Cold agglutinin-induced hemolytic anemia is an extremely rare presentation in systemic lupus erythematosus (SLE). This case report discusses a 32-year-old male who presented with severe anemia and was later diagnosed with SLE. The patient exhibited symptoms of fatigue, weight loss, and shortness of breath, and laboratory findings revealed a hemoglobin level of 7 g/dL, positive cold agglutinins, and autoimmune markers consistent with SLE. Methods: A detailed clinical and laboratory investigation was performed, including blood tests, autoimmune profiles, bone marrow aspiration, and positron emission tomography-computed tomography (PET-CT) to assess the extent of systemic involvement. Nerve conduction studies were conducted to evaluate the patient's neurological symptoms. Results: The patient was diagnosed with cold agglutinin-induced hemolytic anemia secondary to SLE. Treatment included corticosteroids, hydroxychloroquine, and antibiotics for an accompanying infection. The patient responded well to treatment, with stabilization of hemoglobin levels and improvement in neurological symptoms. Conclusion: Cold agglutinin-induced hemolytic anemia is a rare initial manifestation of SLE. Early diagnosis and treatment are critical to preventing complications. This case underscores the importance of considering autoimmune hemolytic anemia in patients presenting with unexplained anemia, especially in the context of potential autoimmune diseases like SLE.
Keywords: Autoimmune hemolytic anemia, systemic lupus erythematosus, cold agglutinin disease, corticosteroids.
References
Alarcon-Segovia, D., Deleze, M., Oria, C. V., ... (1989). Antiphospholipid antibodies and the antiphospholipid syndrome in systemic lupus erythematosus: A prospective analysis of 500 consecutive patients. Medicine, 68(6), 353–365. https://doi.org/10.1097/00005792-198968060-00001
Alger, M., Alargon-Segovia, D., & Riveroa, S. J. (1977). Hemolytic anemia and thrombocytopenic purpura: Two related subsets of systemic lupus erythematosus. Journal of Rheumatology, 4(4), 351–357.
Bijaya Mohanty, D., Ansari, M. Z., Kumari, P., & Sunder, A. (2019). Autoimmune hemolytic anemia in systemic lupus erythematosus. Journal of Family Medicine and Primary Care, 8(5), 1807-1808. https://doi.org/10.4103/jfmpc.jfmpc_298_19
Budman, D. R., & Steinberg, A. D. (1977). Hematologic aspects of systemic lupus erythematosus: Current concepts. Annals of Internal Medicine, 86(2), 220–229. https://doi.org/10.7326/0003-4819-86-2-220
Drenkard, C., Villa, A. R., Alargon-Segovia, D., ... (1994). Influence of the antiphospholipid syndrome on the survival of patients with systemic lupus erythematosus. Journal of Rheumatology, 21(6), 1067–1072.
Gormezano, N. W., Kern, D., Pereira, O. L., Esteves, G. C., Sallum, A. M., Aikawa, N. E., et al. (2017). Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: Differences between pediatric and adult patients. Lupus, 26(4), 426-430. https://doi.org/10.1177/0961203316676379
Harris, N. E., Phil, M., Chan, J. K. H., ... (1986). Thrombosis, recurrent fetal loss, and thrombocytopenia. Archives of Internal Medicine, 146(12), 2153–2156. https://doi.org/10.1001/archinte.146.12.2153
Harvey, A. M., Shulman, L. E., Tumulty, P. A., ... (1954). Systemic lupus erythematosus: Review of the literature and clinical analysis of 138 cases. Medicine, 33(4), 291–437. https://doi.org/10.1097/00005792-195409000-00001
Keeling, D. M., & Isenberg, D. A. (1993). Haematological manifestations of systemic lupus erythematosus. Blood Reviews, 7(3), 199–207. https://doi.org/10.1016/S0268-960X(05)80003-2
Michel, M. (2011). Classification and therapeutic approaches in autoimmune hemolytic anemia: An update. Expert Review of Hematology, 4(6), 607-618. https://doi.org/10.1586/ehm.11.60
Nossent, J. C., & Swaak, A. J. G. (1991). Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus. QJM: An International Journal of Medicine, 80(6), 605–612. https://doi.org/10.1093/qjmed/80.6.605
Tan, E. M., Cohen, A. S., Fries, J. F., et al. (1982). The revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatology, 25(11), 1271–1277. https://doi.org/10.1002/art.1780251101
Tzioufas, A. G., Kokori, S. I., Petrovas, C. I., ... (1997). Autoantibodies to human recombinant erythropoietin in patients with systemic lupus erythematosus: Correlation with anemia. Arthritis & Rheumatology, 40(12), 2212–2216. https://doi.org/10.1002/art.1780401223
Tzioufas, A. G., Manoussakis, M. N., Drosos, A. A., ... (1987). Enzyme immunoassays for the detection of IgG and IgM anti-ds DNA antibodies: Clinical significance and specificity. Clinical and Experimental Rheumatology, 5(3), 247–253.
Vlachoyiannopoulos, P. G., Karassa, F. B., Karakostas, K. X., ... (1993). Systemic lupus erythematosus in Greece: Clinical features, evolution and outcome—a descriptive analysis of 292 patients. Lupus, 2(5), 303–312. https://doi.org/10.1177/096120339300200504
Ward, M. M., Pyun, E., & Studenski, S. (1996). Mortality risks associated with specific clinical manifestations of systemic lupus erythematosus. Archives of Internal Medicine, 156(12), 1337–1344. https://doi.org/10.1001/archinte.156.12.1337
Worlledge, S. (1982). Immune haemolytic anaemias. In R. M. Hardisty & D. J. Weatherall (Eds.), Blood and its disorders (pp. 479–513). Oxford: Blackwell Scientific Publications.
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