Splenectomy for refractory thrombocytopenia in juvenile systemic lupus erythematosus
© Campos et al; licensee BioMed Central Ltd. 2008
Published: 15 September 2008
The prevalence of autoimmune thrombocytopenia in juvenile systemic lupus erythematosus (JSLE) ranges from 7–52% (mean 14,5%) but profound rates (<50,000/mL) are uncommon. Although the exact mechanism is unknown, the spleen has been implicated either as the source of antiplatelet antibodies or the site of sensitized platelets destruction. Splenectomy is rarely indicated due to the increased risk of severe infections and controversial effectiveness.
Materials and methods
From 1983 to 2007, 5079 patients were followed at the Pediatric Rheumatology Unit and JSLE occurred in 228 (4.5%). We report three female JSLE patients with refractory thrombocytopenia to whom splenectomy was indicated.
Clinical and laboratorial manifestations and treatment of refractory thrombocytopenia
Months between JSLE/splenectomy
Pd, MP, CYC
Pd, MP, HCQ, IVG, AZA, C, RTX
Pd, MP, HCQ, IVG, AZA, C, MMF, RTX
Indicated (cancelled due to bone marrow hypoplasia-toxicity?)
Platelet count (time after surgery)
107,000/mL (2 w)
469,000/mL (2 m)
140,000/mL (1 d)
396,000/mL (3 d)
1 month after
Splenectomy should be considered for the treatment of thrombocytopenia refractory cases. Results are variable. Bone marrow hypoplasia should be excluded.
This article is published under license to BioMed Central Ltd.