Abstract

IntroductionChronic refractory immune thrombocytopenic purpura can be a challenging condition to treat. By definition, the standard first and second line treatments have failed in these patients and modalities such as thrombopoiesis-stimulating agents and more intensive immunosuppressive drugs are therefore used. However, there still remains a subset of patients who continue to be refractory to treatment.Case presentationWe present the case of a 30-year-old Hispanic woman with recurrent intracranial bleeds, in whom multiple lines of treatment had failed. She was treated with a combination of bortezomib and rituximab based on previously published data that suggested this therapy effectively blocks all antibody-producing cells. Our patient’s platelet counts rapidly improved and subsequently normalized following this treatment.ConclusionTo the best of our knowledge, this case represents the first report of the effective use of bortezomib and rituximab in highly refractory immune thrombocytopenic purpura. We believe further study of this therapy is warranted in this setting.

Highlights

  • Chronic refractory immune thrombocytopenic purpura can be a challenging condition to treat

  • To the best of our knowledge, this case represents the first report of the effective use of bortezomib and rituximab in highly refractory immune thrombocytopenic purpura

  • We report the case of a patient with refractory and symptomatic Immune thrombocytopenic purpura (ITP) in whom multiple lines

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Summary

Introduction

Immune thrombocytopenic purpura (ITP) is characterized by immune-mediated platelet destruction and is often a chronic disease in adults. Case presentation A 30-year-old Hispanic woman with a history of ITP and autoimmune hemolytic anemia for almost 12 years presented with recurrent intracranial bleeds She had developed chronic refractory disease following the failure of a splenectomy and rituximab therapy shortly after her diagnosis. Her hemoglobin level stayed between 8 and 10g/dL but she had severe thrombocytopenia with platelet counts typically in the range of 5000 to 10,000 cells/mm. Over the three months, after some fluctuations, her platelet count normalized despite cessation of all therapy after six doses of rituximab By this time, she had received a total of 14 subcutaneous injections of bortezomib (Figure 1).

Conclusion
Fierabracci A
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