REVIEW PAPER
Hematologic features of systemic lupus erythematosus
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Online publication date: 2013-03-05
Reumatologia 2013;51(1):31-35
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ABSTRACT
Changes in the hematopoietic system are characteristic features of systemic lupus erythematosus (SLE). Anemia of chronic disease is the most common, whereas autoimmune hemolytic anemia, anemia associated with iron deficiency or renal failure, and iatrogenic anemia (associated with the use of cytotoxic drugs) occur less frequently. Leucopenia, lymphopenia and thrombocytopenia are the other common symptoms of SLE. Causes of hematological disorders in SLE are complex and include autoimmune damage to the bone marrow and peripheral blood cells, erythropoietin deficiency, infections, drug-induced disorders, bone marrow fibrosis and hypersplenism. In the treatment of SLE-related cytopenias glucocorticoids and cytotoxic drugs (azathioprine, cyclosporine A, and mycophenolatemofetil) are used. High doses of intravenous immunoglobulins and danazol are effective in the treatment of thrombocytopenia. Cyclophosphamide is beneficial in severe and refractory thrombocytopenia. Splenectomy and bone marrow transplant may also be considered.
REFERENCES (28)
1.
Sultan SM, Begum S, Isenberg DA. Prevalence, patterns of disease and outcomein patients with systemic lupus erythematosus who develop severe haematological problems. Rheumatology (Oxford) 2003; 42: 230-234.
2.
To CH, Mok CC, Tang SS, et al. Prognostically distinct clinical patterns of systemic lupus erythematosus identified by cluster analysis. Lupus 2009; 18: 1267-1275.
3.
Giannouli S, Voulgarelis M, Ziakas PD, et al. Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment. Ann Rheum Dis 2006; 65: 144-148.
4.
Schett G, Firbas U, Füreder W, et al. Decreased serum erythropoietin and its relation to anti-erythropoietin antibodies in anaemia of systemic lupus erythematosus. Rheumatology 2001; 40: 424-431.
5.
Hara A, Wada T, Kitajima S, et al. Combined pure red cell aplasia and autoimmune hemolytic anemia in systemic lupus erythematosus with anti-erythropoietin autoantibodies. Am J Hematol 2008; 83: 750-752.
6.
Fietta P, Delsante G, Quaini F. Hematologic manifestations of connective autoimmune diseases. Clin Exp Rheumatol 2009; 27:140-154.
7.
Junca J, Cuxart A, Olive A, et al. Anti-intrinsic factor antibodies in systemic lupus erythematosus. Lupus 1993; 2: 111-114.
8.
Korbet SM, Corwin HL. Pernicious anemia associated with systemic lupus erythematosus. J Rheumatol 1986; 13: 193-194.
9.
Hunt BJ, Tueger S, Pattison J, et al. Microangiopathichaemolytica naemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura. Lupus 2007; 16: 358-362.
10.
Durupt S, David G, Durieu I, et al. Myelofibrosis in systemic lupus erythematosus: a new case. Eur J Intern Med 2000; 11: 98-100.
11.
Bass RD, Pullarkat V, Feinstein DI, et al. Pathology of autoimmune myelofibrosis. A report of three cases and a review of the literature. Am J Clin Pathol 2001; 116: 211-216.
12.
Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology 2010; 49: 2243-2254.
13.
Levine AB, Erkan D. Clinical assessment and management of cytopenia in lupus patients. Curr Rheumatol Rep 2011; 13: 291-299.
14.
McDonald EJ, Butler A. Immune thrombocytopaenia in adults: a single-centre retrospective review of patients presenting over 7 years. N Z Med J 2010; 123: 18-25.
15.
Pereira RM, Velloso ER, Menezes Y, et al. Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias. Clin Rheumatol 1998; 17: 219-222.
16.
Liu H, Ozaki K, Matsuzaki Y, et al. Suppression of haematopoiesis by IgG autoantibodies from patients with systemic lupus erythematosus (SLE). Clin Exp Immunol 1995; 100: 480-485.
17.
Yamasaki K, Niho Y, Yanase T. Erythroid colony forming cells in systemic lupus erythematosus. J Rheumatol 1984; 11: 167-171.
18.
Papadaki HA, Boumpas DT, Gibson FM, et al. Increased apoptosis of bone marrow CD34(+) cells and impaired function of bone marrow stromal cells in patients with systemic lupus erythematosus. Br J Haematol 2001; 115: 167-174.
19.
Abu-Shakra M, Shoenfeld Y. Azathioprine therapy for patients with systemic lupus erythematosus. Lupus 2001; 10: 152-153.
20.
Singh NP, Prakash A, Garg D, et al. Aplastic anemia complicating systemic lupus erythematosus: successful management with cyclosporine. Rheumatol Int 2004; 24: 40-42.
21.
Zandman-Goddard G, Levy Y, Shoenfeld Y. Intravenous immunoglobulin therapy and systemic lupus erythematosus. Clin Rev Allergy Immunol 2005; 29: 219-228.
22.
Roldan R, Roman J, Lopez D, et al. Treatment of hemolytic anemia and severe thrombocytopenia with high-dose methylprednisolone and intravenous immunoglobulins in SLE. Scand J Rheumatol 1994; 23: 218-219.
23.
Letchumanan P, Thumboo J. Danazol in the treatment of systemic lupus erythematosus: a qualitative systematic review. Semin Arthritis Rheum 2011; 40: 298-306.
24.
Alba P, Karim MY, Hunt BJ. Mycophenolate mofetil as a treatment for autoimmune haemolytic anaemia in patients with systemic lupus erythematosus and antiphospholipid syndrome. Lupus 2003; 12: 633-635.
25.
Gupta RK, Ezeonyeji AN, Thomas AS, et al. A case of pure red cell aplasia and immune thrombocytopenia complicating systemic lupus erythematosus: response to rituximab and cyclophosphamide. Lupus 2011; 20: 1547-1550.
26.
Song XN, Lv HY, Sun LX, et al. Autologous stem cell transplantation for systemic lupus erythematosus: report of efficacy andsafety at 7 years of follow-up in 17 patients. Transplant Proc 2011; 43: 1924-1927.
27.
Cela I, Miller IJ, Katz RS, et al. Successful treatment of amegakaryocytic thrombocytopenia with eltrombopag in a patient with systemic lupus erythematosus (SLE). Clin Adv Hematol Oncol 2010; 8: 806-809.
28.
Gonzalez-Nieto JA, Martin-Suarez I, Quattrino S, et al. The efficacy of romiplostim in the treatment of severe thrombocytopenia associated to Evans syndrome refractory to rituximab. Lupus 2011; 20: 1321-1323.
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