Abstract:A 42-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital for evaluation of severe thrombocytopenia. She was treated with steroids, intravenous cyclophosphamide, intravenous immunoglobulin, and plasma exchange, but her thrombocytopenia did not improve. Renal biopsy showed class IV-S(C) + V lupus nephritis, according to the classification of the International Society of Nephrology/Renal Pathology Society. The PA-IgG and serum thrombopoietin (TPO) levels were elevated. Her thromboc… Show more
“…TPO-RAs, especially eltrombopag, have shown satisfactory efficacy and well-tolerability in other off-label indications, including SARS-CoV-2 related [ 50 ] or vaccine-induced [ 51 ] thrombocytopenia, and refractory thrombocytopenia in systemic lupus erythematosus [ 52 ] or pregnant women [ 53 , 54 ]. Other common OLDUs include chemotherapy-induced [ 55 ] or inherited thrombocytopenia [ [56] , [57] , [58] ], and myelodysplastic syndromes [ 59 , 60 ].…”
“…TPO-RAs, especially eltrombopag, have shown satisfactory efficacy and well-tolerability in other off-label indications, including SARS-CoV-2 related [ 50 ] or vaccine-induced [ 51 ] thrombocytopenia, and refractory thrombocytopenia in systemic lupus erythematosus [ 52 ] or pregnant women [ 53 , 54 ]. Other common OLDUs include chemotherapy-induced [ 55 ] or inherited thrombocytopenia [ [56] , [57] , [58] ], and myelodysplastic syndromes [ 59 , 60 ].…”
“… 7 Today, it is believed that conventional therapies are effective in autoantibody-mediated peripheral destruction of platelets whereas new agent eltrombopag is effective in impaired platelet production due to autoantibodies related to bone marrow-derived-megakaryocytes. 8 …”
Thrombocytopenia is one of the common haematological manifestations, occurring in 7% to 30% of systemic lupus erythematosus (SLE) patients. Immune thrombocytopenia (ITP) may occur in variable pathways as a result of cross-reacting antibodies or immune complexes that bind to platelet receptors, or infection of progenitor megakaryocytes, and decreased production of thrombopoietin (TPO). It was shown that the vast majority of SLE patients with thrombocytopenia had increased levels of anti-glycoprotein IIb/IIIa (anti-GPIIb/IIIa) or anti-thrombopoietin receptor (anti-TPOR). Eltrombopag is a thrombopoietin receptor agonist that binds to the transmembrane portion of the surface receptor and induces maturation of megakaryocytes and production of platelets. Herein, we report two SLE patients with severe thrombocytopenia who are also refractory to both intravenous immunoglobulin (IVIG), rituximab, and splenectomy. Ultimately, they successfully treated with eltrombopag without any complication. Eltrombopag worked well and their platelet counts increased above 200,000/mm3 only two weeks later.
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