Eltrombopag improves refractory thrombocytopenia in patients with Sjögren's syndrome

Abstract

Introduction: Eltrombopag, a kind of thrombopoietin (TPO) receptor agonist, plays the role on the megakaryocyte to activate the platelet production and rapidly increase the number of circulating platelets in patients with primary immune thrombocytopenia (ITP). Eltrombopag provides an opportunity for rapid tapering and/or cessation of corticosteroid therapy. However, it is not clear about the platelet response to Eltrombopag in ITP associated with Sjögren's Syndrome(SS). Methods: A retrospective research was conducted on the clinical course of three patients, each with ITP secondary to SS, and initially received therapy of corticosteroids or other immunomodulatory. They took this drug for bleeding diseases. Referring to the description, Eltrombopag was prescribed and adjusted with an initial dose of 25 mg daily, then weekly, then monthly according to the monitoring of platelet counts. Results: All patients maintained a satisfactory level of platelet counts (>100,000/mm3 for >2 years) following corticosteroid withdrawal. Meanwhile, Eltrombopag was well-tolerated, and there were no adverse effects, such as thrombotic events. Conclusions: Eltrombopag is effective and safe for patients with ITP associated with SS during corticosteroid withdrawal. Thus it may be a crucial therapeutic strategy for reducing corticosteroid-related side effects in SS patients with ITP.

Comprehensive analysis of the clinical manifestations and hematological parameters associated with secondary immune thrombocytopenia in patients with primary Sjögren syndrome: An observational study

“…[ 16 , 17 ] This study delves into the complex interplay between pSS and secondary ITP, highlighting key differences in clinical presentations, demographic profiles, and both hematological and immunological parameters between pSS patients with and without secondary ITP. [ 18 , 19 ] Our findings reveal a unique pattern in pSS patients with secondary ITP, characterized by increased fatigue, a higher incidence of lymphadenopathy and arthritis, and distinct hematological and immunological markers. These differences underscore the need for a nuanced understanding of pSS, emphasizing the importance of comprehensive clinical evaluation in patients with this condition.…”

Nomogram Based on Inflammatory Factor to Predict Therapeutic Response of Thrombocytopenia in Patients with Primary Sjögren’s Syndrome

“…All patients underwent routine laboratory assessments 1 month after the end of treatment. The response criteria were as follows: 15 (1) complete remission: platelet count ≥100×10 9 /L and absence of bleeding; (2) partial remission: platelet count ≥ 30×10 9 /L or increased above baseline by at least 2 times and no bleeding episodes; (3) no remission: platelet count <30×10 9 /L or bleeding, or increase lower than 2 times of baseline. Patients with complete remission and partial remission were considered in the remission group and no remission was the non-remission group.…”

“…7,8 The current treatment options for such diseases include glucocorticoids, immunosuppressants, and intravenous immunoglobulin. 9 However, a proportion of patients may develop resistance to this therapy or exhibit dependency on corticosteroids and require the application of secondline treatments. Even after receiving treatment with glucocorticoids and more than one immunosuppressant, some patients remain refractory or encounter unacceptable toxicity.…”

Treatment with Erythromycin and Tacrolimus for Primary Sjogren’s Syndrome-Related Immune Thrombocytopenia: A Case Report

“…The mechanism of ITP in SjS is due to anti-platelet (PLT) autoantibody-induced peripheral PLT destruction and splenic sequestration, as well as reduced PLT production by impaired megakaryocytes caused by anti-PLT autoantibodies. 1 Corticosteroids supplemented with immunoglobulin (Ig) are the first-line treatments for SjS-related ITP, while second-line treatments including danazol, cyclosporin A, azathioprine, eltrombopag (ELT), and rituximab have been proposed. However, clinical trials evaluating their efficacy and safety specifically in patients with SjS-related ITP are not documented.…”

“…However, clinical trials evaluating their efficacy and safety specifically in patients with SjS-related ITP are not documented. 1 Erythromycin (EM), a macrolide (MAC), provides not only anti-bacterial activities but also immunomodulatory effects. Similarly, tacrolimus (TAC), a calcineurin inhibitor and MAC, also provides immunomodulatory effects.…”