Eltrombopag as steroid sparing therapy for immune thrombocytopenic purpura in systemic lupus erythematosus

Maroun, Marie Claire; Ososki, Rosanne; Andersen, Judith C.; Dhar, J. Patricia

doi:10.1177/0961203314559632

Cited by 28 publications

(20 citation statements)

References 14 publications

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“…There are very few reported cases of ITP secondary to autoimmune disorders successfully treated with THPO-RAs. (Gonzalez-Nieto et al, 2011;Alkaabi et al, 2012;Ruiz-Arguelles et al, 2013;Magnano et al, 2014;Scheinberg et al, 2014;Chang & Shih, 2015;Maroun et al, 2015) Here we report an excellent response in ITP secondary to autoimmune disorders. Similarly, the response rate in ITP secondary to infectious disorders was excellent.…”

Section: Discussionsupporting

confidence: 58%

“…Also, eltrombopag is currently approved by the US Food and Drug Administration and the European Medicines Agency for the treatment of thrombocytopenia secondary to aplastic anaemia (Olnes et al, 2012) and thrombocytopenia secondary to hepatitis C infection (McHutchison et al, 2007;Afdhal et al, 2012). Eltrombopag has also been successfully used in a very small number of patients with immune thrombocytopenia secondary to chronic lymphatic leukaemia (Koehrer et al, 2010;Sinisalo et al, 2011;Gudbrandsdottir et al, 2012;Jolliffe & Romeril, 2014;Chang & Shih, 2015), systemic lupus erythematosus (SLE: Chang & Shih, 2015;Gonzalez-Nieto et al, 2011;Alkaabi et al, 2012;Scheinberg et al, 2014;Magnano et al, 2014;Maroun et al, 2015), Evans syndrome (Gonzalez-Nieto et al, 2011;Alkaabi et al, 2012;Ruiz-Arguelles et al, 2013;Magnano et al, 2014;Scheinberg et al, 2014;Chang & Shih, 2015;Maroun et al, 2015), human immunodeficiency virus (Quach et al, 2012;Aslam et al, 2014;Chang & Shih, 2015;Kowalczyk et al, 2015) and coeliac disease (Chang & Shih, 2015) who had a loss of response to standard treatment for ITP. The communication of such cases is essential to support the potential utility of eltrombopag as salvage therapy in the treatment of secondary immune thrombocytopenia.…”

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confidence: 99%

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Use of eltrombopag for secondary immune thrombocytopenia in clinical practice

et al. 2017

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Eltrombopag is a second-line treatment in primary immune thrombocytopenia (ITP). However, its role in secondary ITP is unknown. We evaluated the efficacy and safety of eltrombopag in secondary ITP in daily clinical practice. Eighty-seven secondary ITP patients (46 with ITP secondary to autoimmune syndromes, 23 with ITP secondary to a neoplastic disease subtype: lymphoproliferative disorders [LPDs] and 18 with ITP secondary to viral infections) who had been treated with eltrombopag were retrospectively evaluated. Forty-four patients (38%) had a platelet response, including 40 (35%) with complete responses. Median time to platelet response was 15 days (95% confidence interval, 7-28 days), and was longer in the LPD-ITP group. Platelet response rate was significantly lower in the LPD-ITP than in other groups. However, having achieved response, there were no significant differences between the durable response of the groups. Forty-three patients (49·4%) experienced adverse events (mainly grade 1-2), the commonest being hepatobiliary laboratory abnormalities. There were 10 deaths in this case series, all of which were related to pre-existing medical conditions. In routine clinical practice, eltrombopag is effective and well-tolerated in unselected patients with ITP secondary to both immune and infectious disorders. However, the response rate in LPD-ITP is low.

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Section: Discussionsupporting

confidence: 58%

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Use of eltrombopag for secondary immune thrombocytopenia in clinical practice

et al. 2017

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“…Many patients with ITP or secondary ITP due to lymphoproliferative disorders responding to eltrombopag have already been reported, but the efficacy of this drug for thrombocytopenia associated with SLE remains unclear, and only three reports have been published [ 3 – 5 ]. Maroun et al reported 3 SLE patients with thrombocytopenia refractory to glucocorticoids, in whom elevation of the platelet count was achieved by administration of eltrombopag.…”

Section: Discussionmentioning

confidence: 99%

Eltrombopag Improves Refractory Thrombocytopenia in a Patient with Systemic Lupus Erythematosus

Shima

Sumida

Kawada

et al. 2018

Case Reports in Rheumatology

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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 thrombocytopenia responded to off-label administration of eltrombopag, which was discontinued after 42 months. At 18 months after stopping eltrombopag, the platelet count was 19.3 × 104/μL. Eltrombopag may be a therapeutic option for SLE patients with severe thrombocytopenia refractory to conventional therapy.

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“…76 Contrasting to these results a young SLE patient developed thrombotic microangiopathy with renal failure after romiplostim therapy. 77 Maroun et al 78 reported on three patients with SLE thrombocytopenia in which administration of eltrombopag was successful as steroid-sparing treatment. Another patient with refractory thrombocytopenia achieved a complete response after eltrombopag treatment.…”

Section: Thrombopoietin Receptor Agonistsmentioning

confidence: 99%

Lupus thrombocytopenia: pathogenesis and therapeutic implications

Galanopoulos¹,

Christoforidou²,

Bezirgiannidou³

2017

MJR

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disorder that is frequently complicated by hematological manifestations such as hemolytic anemia, leukopenia and thrombocytopenia. 1,2 These disorders are included in the diagnostic criteria of SLE both in the earlier (1982), as well as in the revised criteria of the American College of Rheumatology (2003), and the 2012 Systemic Lupus International Collaborating Clinics (SLCC) criteria. 3-5 Thrombocytopenia (<100x10 9 /L) has been reported in 20% to 40% of patients with SLE 2,6-8 and is usually attributed to an autoimmune mechanism similar to that of idiopathic immune thrombocytopenia (ITP). It may be the first manifestation of lupus in up to 16% of patients, presenting months or as early as 10 years before diagnosis. 9,10 It can also be a complication of therapeutic agents such as azathioprine, methotrexate and rarely hydroxychloroquine. Severe thrombocytopenia (<20 to 50x10 9 /L according to study definition) is relatively rare, occurring in about 3-10% of patients. 11,12 Occasionally, thrombo-Cite this article as: Galanopoulos N, Christoforidou A, Bezirgiannidou Z. Lupus thrombocytopenia: pathogenesis and therapeutic implications.

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Eltrombopag as steroid sparing therapy for immune thrombocytopenic purpura in systemic lupus erythematosus

Cited by 28 publications

References 14 publications

Use of eltrombopag for secondary immune thrombocytopenia in clinical practice

Use of eltrombopag for secondary immune thrombocytopenia in clinical practice

Eltrombopag Improves Refractory Thrombocytopenia in a Patient with Systemic Lupus Erythematosus

Lupus thrombocytopenia: pathogenesis and therapeutic implications

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