Hematologic Manifestations in Systemic Lupus Erythematosus:A Systematic Review of Clinical Patterns, Prognostic Implications,and Epidemiology

Main Article Content

Abdullah Al-Sheef
Luluh Khalid
Raneem Almutairi
Renad Alhajlah
Nora Alotaibi
Shaden Almutairi
Abdullah Hamdan
Khalid Al-Moqader
Amjad Al-Moqader
Maryam Alfehaid
Shahad Alelaiw
Maha Alfayad
Sarah Jabbari
Wasan Alenazi
Abdullah Shaher

Keywords

Systemic Lupus Erythematosus, SLE, Hematologic manifestations, Cytopenia, Anemia, Thrombocytopenia, Leukopenia, Pediatric SLE, Splenomegaly, Autoimmune cytopenias, Prognosis

Abstract

Systemic lupus erythematosus (SLE) is a long-term autoimmune disease that has various clinical manifestations, the hematologic abnormalities among which are frequent and vary in clinical severity. These may cause simple cytopenias up to extreme autoimmune cytopenias, and significantly affect the activity of the disease, prognosis, and treatment. The proposed systematic review is expected to synthesise existing data on the prevalence, clinical features, and prognostic consequences of hematologic involvement in patients with SLE in children and adults. The studied data were searched in PubMed, PMC, WHO IMSEAR, and other databases to find publications published in 2025. They included observational studies, cohort studies, cross-sectional studies and reviews that were relevant. Information was coming out on the hematologic parameters, the occurrence of cytopenias, disease-related activity, autoantibody profiles, and the presence of splenomegaly. The inclusion studies were evaluated by the Newcastle-Ottawa Scale to assess quality, and the PRISMA was used to report them. Among the studies that were included, hematologic abnormalities that were most common were anaemia, leukopenia, and thrombocytopenia, though autoimmune hemolytic anaemia and immune thrombocytopenia occurred in a subset of patients. Guidelines showed that the incidence of cytopenias was high in pediatric populations, in contrast with adults. Hematologic aberrations were often related to augmented disease activity, involvement of the kidneys, and the presence of autoantibodies. Splenomegaly, being less prevalent, showed an association with bad cytopenias and poor prognosis across various cohorts. Hematologic presentations are inherent parts of SLE and have both diagnostic, prognostic, and treatment ramifications. Early detection and surveillance of the hematologic involvement can promote person-specific management approaches, enhance the outcomes, and guide future research topics related to the pathogenesis of the disease and targeted therapies.

Abstract 227 | PDF Downloads 60

References

1. Sasidharan, P. K., Bindya, M., & Sajeeth Kumar, K. G. (2012). Hematological Manifestations of SLE at Initial Presentation: Is It Underestimated?. ISRN hematology, 2012, 961872.
2. Nossent, J. C., & Swaak, A. J. (1991). Prevalence and significance of haematological abnormalities in patients with systemic lupus rythematosus. The Quarterly journal of medicine, 80(291), 605–612.
3. Dhir, V., Kumar, A., Kumar, S., et al. (2010). Haematological profile in North Indian SLE patients: A retrospective study. Indian Journal of Hematology and Blood Transfusion, 26, 42–47.
4. Aringer, M., Costenbader, K., Daikh, D., et al. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for SLE. Arthritis & Rheumatology, 71, 1400–1412.
5. Pons-Estel, G. J., Alarcón, G. S., Scofield, L., Reinlib, L., & Cooper, G. S. (2010). Understanding the epidemiology and progression of systemic lupus erythematosus. Seminars in arthritis and rheumatism, 39(4), 257–268.
6. Yuce Inel, T., Uslu, S., Demirci Yildirim, T., Gulle, S., & Sen, G. (2025). Hematologic Involvement in Systemic Lupus Erythematosus: Clinical Features and Prognostic Implications in a Hematology-Referred Cohort. Journal of clinical medicine, 14(20), 7304.
7. Mina, R., & Brunner, H. I. (2021). Pediatric SLE: Clinical and hematologic features. Lupus, 30, 1732–1742.
8. Keeling, D. M., & Isenberg, D. A. (1993). Haematological manifestations of systemic lupus erythematosus. Blood reviews, 7(4), 199–207.
9. Shin, J. I., Lee, K. H., Park, S., Yang, J. W., Kim, H. J., Song, K., Lee, S., Na, H., Jang, Y. J., Nam, J. Y., Kim, S., Lee, C., Hong, C., Kim, C., Kim, M., Choi, U., Seo, J., Jin, H., Yi, B., Jeong, S. J., … Kronbichler, A. (2022). Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review. Journal of clinical medicine, 11(22), 6714.
10. Moher, D., Liberati, A., Tetzlaff, J., & Altman, D. G.; PRISMA Group. (2009). Preferred reporting items for systematic reviews and meta-analyses: The PRISMA statement. PLoS Medicine, 6(7), e1000097.
11. Aringer, M., Costenbader, K., Daikh, D., et al. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for SLE. Arthritis & Rheumatology, 71, 1400–1412.
12. Petri, M., Orbai, A. M., Alarcón, G. S., et al. (2012). Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for SLE. Arthritis & Rheumatism, 64, 2677–2686.
13. Higgins, J. P. T., Thomas, J., Chandler, J., et al. (Eds.). (2021). Cochrane Handbook for Systematic Reviews of Interventions (2nd ed.). London: Cochrane.
14. Liberati, A., Altman, D. G., Tetzlaff, J., et al. (2009). The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: Explanation and elaboration. BMJ, 339, b2700.
15. Wells, G. A., Shea, B., O’Connell, D., et al. (2014). The Newcastle–Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses. Ottawa Hospital Research Institute.
16. Stroup, D. F., Berlin, J. A., Morton, S. C., et al. (2000). Meta-analysis of observational studies in epidemiology: A proposal for reporting. JAMA, 283, 2008–2012.
17. Booth, A., Sutton, A., & Papaioannou, D. (2016). Systematic Approaches to a Successful Literature Review (2nd ed.). London:
SAGE.
18. Higgins, J. P. T., & Green, S. (Eds.). (2011). Cochrane Handbook for Systematic Reviews of Interventions (Version 5.1.0). The
Cochrane Collaboration.
19. Egger, M., Davey Smith, G., Schneider, M., & Minder, C. (1997). Bias in meta-analysis detected by a simple, graphical test. BMJ,
315, 629–634.
20. Deeks, J. J., Higgins, J. P. T., & Altman, D. G. (2011). Analysing data and undertaking meta-analyses. In J. P. T. Higgins & S. Green
(Eds.), Cochrane Handbook for Systematic Reviews of Interventions (5.1.0).
21. Stang, A. (2010). Critical evaluation of the Newcastle–Ottawa scale for the assessment of the quality of nonrandomized studies
in meta-analyses. European Journal of Epidemiology, 25, 603–605.
22. Aleem, A., Al Arfaj, A. S., khalil, N., & Alarfaj, H. (2014). Haematological abnormalities in systemic lupus erythematosus. Acta
reumatologica portuguesa, 39(3), 236–241.
23. Nam, S. H., Park, H. S., Ahn, S. M., Oh, J. S., Kim, Y. G., Lee, C. K., Yoo, B., & Hong, S. (2023). Clinical features of systemic lupus
erythematosus patients with splenomegaly: focussed on the cytopenias. Internal medicine journal, 53(12), 2341–2345.
24. Yuce Inel, T., Uslu, S., Demirci Yildirim, T., Gulle, S., & Sen, G. (2025). Hematologic Involvement in Systemic Lupus Erythematosus:
Clinical Features and Prognostic Implications in a Hematology-Referred Cohort. Journal of Clinical Medicine, 14(20), 7304.
25. Characteristics of hematological manifestations in patients with SLE, HCTC 2025; 302 patients. (2025). https://doi.org/10.1016/j.
htct.2025.103931
26. Bathina, H., et al. (2024). Clinical and hematological manifestations of SLE at initial presentation in a tertiary healthcare center.
27. Pediatric SLE hematologic manifestations — Eastern/Northeast India, n=30. (2024).
28. Hematological disorders in systemic lupus erythematosus (review). (2014).
29. Santacruz, J. C., Mantilla, M. J., Rueda, I., Pulido, S., Rodriguez-Salas, G., & Londono, J. (2022). A Practical Perspective of the
Hematologic Manifestations of Systemic Lupus Erythematosus. Cureus, 14(3), e22938.
30. Adhikari, K., Karki, H., Shrestha, P., Sigdel, K. R., Sharma, L., & Paudel, B. P. (2025). Hematological manifestations in systemic
lupus erythematosus: a retrospective cross-sectional study from a tertiary care center in Nepal. Annals of medicine and
surgery (2012), 87(10), 6335–6339.
31. Irum, Saima. (2025). “CLINICOPATHOLOGICAL CORRELATION OF HEMATOLOGIC ALTERATIONS AND TISSUE HISTOPATHOLOGY
IN SYSTEMIC LUPUS ERYTHEMATOSUS. Journal of Population Therapeutics and Clinical Pharmacology. 809-815.
32. Essouma, M., & Noubiap, J. J. (2024). Lupus and other autoimmune diseases: Epidemiology in the population of African
ancestry and diagnostic and management challenges in Africa. The journal of allergy and clinical immunology. Global, 3(4),
100288.
33. Gulati, S., Kumar, V., Rawat, P., Chawla, K., Dessai, R., & Jain, S. Study of hematological …
34. Chen, J. L., Huang, X. M., Zeng, X. J., Wang, Y., Zhou, M. X., Ma, Y. H., Li, R. S., & Shen, T. (2007). Zhonghua yi xue za zhi, 87(19),
1330–1333.
35. Ramos-Casals, M., Nardi, N., Cuadrado, M. J., et al. (2015). Hematologic involvement in systemic lupus erythematosus: Clinical
patterns and immunologic associations. Autoimmunity Reviews, 14, 880–887.
36. Zhang, Y., Li, W., Zhang, P., Guo, J., Sun, J., Lu, J., & Liu, S. (2022). Hematological malignancies in systemic lupus erythematosus:
clinical characteristics, risk factors, and prognosis—a case-control study. Arthritis Research & Therapy, 24(1), 5.
37. Santacruz, J. C., Mantilla, M. J., Rueda, I., Pulido, S., Rodriguez-Salas, G., & Londono, J. A practical perspective of the hematologic
manifestations of systemic lupus erythematosus. Cureus 2022; 14 (3): e22938.
38. Sasidharan, P. K., Bindya, M., & Sajeeth Kumar, K. G. (2012). Hematological manifestations of SLE at initial presentation: is it
underestimated?. International Scholarly Research Notices, 2012(1), 961872.
39. Gokce, M., Bilginer, Y., Besbas, N., Ozaltın, F., Cetin, M., Gumruk, F., & Ozen, S. (2012). Hematological features of pediatric systemic lupus erythematosus: suggesting management strategies in children. Lupus, 21(8), 878-884.
40. Varghese Koshy, A. M., & Goel11, N. (2025). A Retrospective Study of Clinical Characteristics and Serological Profile of Male
Systemic Lupus Erythematosus Patients in India. Journal of The Association of Physicians of India, 73(4), 43.
41. Fayyaz, A., Igoe, A., Kurien, B. T., Danda, D., James, J. A., Stafford, H. A., & Scofield, R. H. (2015). Haematological manifestations
of lupus. Lupus science & medicine, 2(1), e000078.
42. Akca, Ü. K., Batu, E. D., Kısaarslan, A. P., Poyrazoğlu, H., Ayaz, N. A., Sözeri, B., Sağ, E., Atalay, E., Demir, S., Karadağ, Ş. G., Demir,
F., Bilginer, Y., Gümrük, F., & Özen, S. (2021). Hematological involvement in pediatric systemic lupus erythematosus: A multicenter
study. Lupus, 30(12), 1983–1990.
43. Newman, K., Owlia, M. B., El-Hemaidi, I., & Akhtari, M. (2013). Management of immune cytopenias in patients with systemic
lupus erythematosus—old and new. Autoimmunity reviews, 12(7), 784-791.