To the Editor:
Thrombocytopenia is frequent in patients with systemic lupus erythematosus (SLE), occurring in 7% to 30% of patients. Less than 5% present a platelet count of < 50,000/mm3 and they usually respond to first- or second-line therapy [corticosteroids, immunosuppressive agents, intravenous immunoglobulin (IVIG), rituximab, or splenectomy]. However, a significant number of patients will not respond to these treatments or will relapse afterward1.
Romiplostim and eltrombopag are 2 thrombopoietin-receptor agonist drugs that were approved in 2008 by the US Food and Drug Administration (FDA) to treat patients with chronic idiopathic thrombocytopenic purpura who have an insufficient response to conventional therapy2,3,4,5. To date, there are only 4 published cases of patients with SLE and immune thrombocytopenia successfully treated with these agents6,7,8,9 and 1 case in whom this treatment was not effective10. We describe 2 additional cases of patients with SLE and refractory immune thrombocytopenia who responded to thrombopoietin-receptor agonists.
Case 1
A 69-year-old woman was diagnosed with SLE in 1992 because of anemia, thrombocytopenia, positive antinuclear antibody (ANA), anti-dsDNA, anti-Sm antibodies, lupus anticoagulant, and hypocomplementemia. In 1999, a laparoscopic splenectomy was performed because of severe immune thrombocytopenia (< 10,000/mm3) that was refractory to high-dose prednisone and IVIG. She maintained a normal platelet count until March 2013 when she presented with mild epistaxis, ecchymosis, and thrombocytopenia of 22,000/mm3 without …
Address correspondence to Dr. G. Espinosa, Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036 Barcelona, Catalonia, Spain. E-mail: gespino{at}clinic.ub.es
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