Hematological manifestations in patients with systemic lupus erythematosus

Authors: M. Schubertová;  P. Horák;  M. Skácelová;  J. Zadražil
Authors‘ workplace: III. interní klinika – nefrologická, revmatologická a endokrinologická ;  Fakultní nemocnice, Olomouc
Published in: Čes. Revmatol., 24, 2016, No. 1, p. 6-14.
Category: Original article


Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease characterized by hyperactivity of B cells and overproduction of organ nonspecific autoantibodies. The most common organ manifestations include impaired skin, kidneys, joints, cardiovascular system, central nervous system or hematologic manifestations. The aim of this study was to assess the incidence of hematological symptoms and their frequency in patients with SLE.

Patients and methods:
Single-center, retrospective study analyzed the incidence of hematologic manifestations in 160 patients who met the ACR diagnostic criteria for SLE. The presence and representation of hematologic manifestations was compared at the onset of the disease and at remission induction. The obtained data were statistically analyzed.

Hematologic manifestations were present in 67% of patients with SLE. The most common one, anemia, occurred early in the disease in 45% of patients with hemoglobin (Hb) levels of 101 ± 13 g / l and an average number of erythrocytes 3.50 ± 0.53 x 1012 / l. After stabilization of the disease the incidence of anemia decreased to 20% with a mean Hb value of 123 ± 18 g / l and the number of erythrocytes 3.9 ± 0.53 x 1012 / l. Hemolytic anemia was found in 21% of patients. Leucopenia was present in 24% of patients, the average number of leukocytes in these patients was 2.71 ± 0.71 x 109 / l. Twenty-four % of patients had thrombocytopenia and antiphospholipid syndrome was found in 16% of patients.

Hematological abnormalities are among the most common manifestations of SLE. They represent one of the ACR and SLICC diagnostic criteria for SLE. Anemia, which affects almost half of the patients, was the most frequent abnormality.

Key words:
Systemic lupus erythematosus, hematopoiesis, anemia, antiphospholipid syndrome


1. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus May 2006; 15: 308–318.

2. Wallace DJ, Hahn BH. Dubois´ lupus erythematosus. Section IV: autoantibodies, Lea and Febinger. Philadelphia 1993; 181–276.

3. Hochberg MC. 1997 Update of the 1982 American College of Rheumatology Revised Criteria for Classification of Systemic Lupus Erythematosus. Arthritis Rheum 1997; 40; 1725.

4. Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012; 64: 2677–2686.

5. Sasidharan PK, Bindiya M, Sajeeth Kumar KG. Systemic lupus erythematosus–A Hematological Problem. J Blood Disorder Transf 2013; 4: 168.

6. Al Afraj AS, Khalil N. Clinical and imunological manifestatins in 624 SLE patients in Saudi Arabia. Lupus 2009; 18(5): 465–73.

7. Marchand A, Galen RS, Van Lente F. The predictive value of serum haptoglobin in hemolytic disease. JAMA 1980; 243: 1909–1911.

8. Galen RS. Application of the predictive value model in the analysis of test effectiveness. Clin Lab Med 1982; 2: 685–699.

9. Voulgarelis M, Kokori SL, Loannidis JP, et al. Anemia in systemic lupus erythematosus: aetiological profileand the role of erythopoetin. Ann Rheum Dis 2000 59(3): 217–222.

10. Valent P, Lechner K. Diagnosis and treatment of autoimunne haemolytic anemia in adults: a clinical review. Wien Klin Wochenschr 2008 120(5-6):136–151.

11. Hux M, Fan ZR, Zhou SY et al. Hematological abnormality and clinical characteristics in systemic lupus erythematosus. Zhongguo shi Yan 2004;12(2): 170–173.

12. Rouf A, Uddin R, Hoque E. Pattern of Hematological Manifestations in Patients with Systemic Lupus Erythematosus Attending in a Tertiary Care Hospital. Chattagram Maa-O-Shishu Hospital. Medical College Journal 2014; 13(1): 49–53.

13. Aleem A, Al Arfaj AS, Khalil N, et al. Haematological abnormalities in systemic lupus erythematosus. Acta Reumatol Port 2014; 39(3): 236–241.

14. Simantov R, Laurence J, Nachman RL. The cellular hematology of systemic lupus erythematosus. Systemic lupus erythematosus, Fourth edition, 2004; Elsevier

15. Pisetski, DS, Gilkeson G, St Clair EW. Systemic lupus erythematosus, Diagnosis and treatment. Med Clin North Am 1997; 81: 113–128.

16. Sasidharan PK, Bindya M, Kumar KGS. Hematological manifestation of SLE at initial presentation: Is it underestimated? ISRN Hematol 2012; 961872–7.

17. Voulgarelis M, Kokoria SI, Ioannidisb JP, et al. Anaemia in systemic lupus erythematosus: aetiological profile and the role of erythropoetin. Ann Rheum Dis 2000; 59: 217–222.

18. Giannouli S, Voulgarelis M, Ziakas PD, et al. Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment. Annals of the Rheumatic Diseases 2006; 65(2): 144–148.

19. Nossent JC, Swaak AJ. Prevalence and Significance of Haematological Abnormalities in Patients with Systemic Lupus Erythematosus. Q J Med 1991; 80: 605–612.

20. Newman K, Owlia MB, El-Hemaidi I, et al. Management of immune cytopenias in patients with systemic lupus erythematosus–old and new. Autoimmun Rev 2013; 12: 784–791.

21. Chun Li, Rong Mu, Xiao-yan Lu. Antilymphocyte Antibodies in Systemic Lupus Erythematosus: Association with Disease Activity and Lymphopenia. J Immunology Research 2014; 2014; 309–326.

22. Budman DR, Steinberg AD. Hematologic aspect of systemic lupus erythematosus. Current concepts. Ann Int Med 1977; 86; 220–229.

23. Hepburn AL, Narat S, et al. The management of periperal blood cytopenias in systemic lupus erythematosus. Oxford Rheumatol J 2010; 49: 2243–2254.

24. Trench CM, McCurdie I, White PD, et al. The prevalence and association s of fatigue in systemic lupus erythetosus. Int J Clin Rheumatol 2012; 7(2): 217–227.

25. Jump RL, Robinson ME, Armstrong AE et al. Fatigue in systemic lupus: contributions of disease activity, pain, depression, and perceived socialsupport. J Rheumatol 2005; 32: 1699–1705.

26. Burgos PI, Alarcon GS, McGwin G, et al. Disease activity and damage are not associated with increased levels of fatigue in systemic lupus erythematosus patients from multiethnic cohort. Arthritis Rheum 2009; 61: 1179–1186.

27. Bazzan M, Vaccarino A, Marletto F. Systemic lupus erythematosus and thrombosis. Thrombosis J 2015; 13: 16–26.

28. Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46:1019–1027.

29. Stratta P, Canavese C, Ferrero S. Catastrophic antiphospholipid syndromes in systemic lupus erythematosus. Ren Fail 1999; 21(1): 49–61.

30. Zadrazil J, Horak P. Pathophysiology of anemia in chronic kidney diseases: A review. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2015; 159(2): 197–202.

31. Astor BC, Muntner P, Levin A, et al. Association of kidney function with anemia: the Third National Health and Nutrition Examination Survey (1988-1994). Arch Intern Med 2002; 162: 1401–1408.

32. Hsu CY, McCulloch CE, Curhan GC. Epidemiology of anemia associated with chronic renal insufficiency among adults in the United States: results from the Third National Health and Nutrition Examination Survey. J Am Soc Nephrol 2002; 13: 504–510.

33. El-Achkar TM, Ohmit SE, McCullough PA, et al. Higher prevalence of anemia with diabetes mellitus in moderate kidney insufficiency: The Kidney Early Evaluation Program. Kidney Int 2005; 67: 1483–1488.

34. Walle AJ, Wong GY, Clemons GK, et al. Erytropoetin–hematocrit feedback circuit in the anemia of end stage renal disease. Kidney Int 1987; 31: 1205–1259.

35. Nangaku M, Eckardt KU. Pathogenesis of renal anemia. Semin nephrol 2006; 26: 261–268.

36. Cao L, Tong H, Xu G, et al. Systemic Lupus Erythematous and Malignancy Risk: A Meta-Analysis. Scheurer M, ed. PLoS ONE 2015; 10(4).

37. Leandro MJ, Isenberg DA. Rheumatic diseases and malignancy–is there an association? Scand J Rheumatol 2001;30(4): 185–188.

38. Pistiner M, Wallace DJ, Nessim S, et al. Lupus erythematosus in 1980s: a survey of 570 patients. Semin Arthritis Rheum 1991; 21(1): 55–64.

39. Lansigan F, Isufi I, Tagoe CE. Microangiopatic haemolytic anémie resembling trombocytopenic purpura in SLE: the role of ADAMTS 13. Rheumatology (Oxford) 2011 50(5): 824–829.

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