Relapsing Evans syndrome and systemic lupus erythematosus with antiphospholipid syndrome treated with Bortezomib in combination with plasma exchange

Tkachenko, Olga; Лапин, С. В.; Maslyansky, Alexey; Myachikova, Valentina; Михайлова, Л. Р.; Gilburd, Boris

doi:10.1016/j.clim.2018.12.010

Cited by 11 publications

(9 citation statements)

References 9 publications

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“…10,[49][50][51][52][53]55 In particular, 17 patients suffered from lupus nephritis, and 2 had underlying MM. Bortezomib was administered from one to four cycles, mostly in combination with steroids, and all patients had a Tkachenko et al 48 AIN, autoimmune neutropenia; AH, acquired hemophilia; CR, complete remission; ES, Evans syndrome; HLA, human leukocyte antigen; HSCT, hematopoietic stem cell transplant; ITP, immune thrombocytopenia; IV, intravenous; NR, non-response; PEX, plasma exchange; PR, partial remission; SC, subcutaneous; TTP, thrombotic thrombocytopenic purpura.…”

Section: Bortezomib In Systemic Connective Tissue Diseasesmentioning

confidence: 99%

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Bortezomib in autoimmune hemolytic anemia and beyond

Pasquale

Giannotta

Barcellini

et al. 2021

Therapeutic Advances in Hematology

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Bortezomib is a first-in-class, potent, selective and reversible proteasome inhibitor approved for the treatment of multiple myeloma (MM) and relapsed/refractory mantle cell lymphoma. In these diseases, bortezomib targets plasma cells and lymphocytes reducing tumor burden. Recently, preclinical evidence highlighted its efficacy in reducing long-lived plasma cells responsible of autoantibodies production in several models of autoimmune conditions. These findings paved the way to a number of experiences of bortezomib use in patients with various autoimmune conditions, including autoimmune hemolytic anemia (AIHA). The latter is a nice model of autoimmunity in hematology and is caused by the production of autoantibodies against erythrocytes resulting in various degrees of hemolytic anemia. AIHA is classified in warm and cold forms according to the thermal characteristics of the autoantibody, and first-line treatment mainly relies on steroids for warm cases and the anti-CD20 rituximab for cold ones. Relapsed/refractory cases are still an unmet need, and bortezomib has been proposed in this setting with intriguing efficacy. In this review, we collected available literature on bortezomib use in AIHA and in other immune-mediated hematologic and non-hematologic diseases. Overall, most experiences highlight bortezomib efficacy even in multi-relapsed/refractory patients and suggest to consider its use in AIHA after rituximab failure.

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Section: Bortezomib In Systemic Connective Tissue Diseasesmentioning

confidence: 99%

“…Eight cases of ITP have been described, six pediatrics and two adults. 22,26,48 They were treated with bortezomib from less than one to two cycles, and five patients responded (four pediatric and one adult). The non-responding adult patient died for uncontrollable bleeding.…”

Section: Bortezomib In Autoimmune Cytopenias Other Than Aihamentioning

confidence: 99%

Bortezomib in autoimmune hemolytic anemia and beyond

Pasquale

Giannotta

Barcellini

et al. 2021

Therapeutic Advances in Hematology

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“…After 6 cycles of IV bortezomib (1.3 mg/m 2 twice weekly) in addition to 3 cycles of plasma exchange and cyclosporine, anemia and thrombocytopenia improved in 6 weeks, and skin lesions regressed with no relapse of hemolysis or thrombosis at 1 year follow-up while still on bortezomib every 3 weeks. 22 In summary, despite limited basic science and clinical experience, B-cell inhibition is an option for aPL-positive patients with prominent hematologic and microvascular manifestations. Given that B cells have a role in disease pathogenesis not limited to antibody production, it is highly likely that the clinical response seen in some aPL-positive patients is independent of a substantial change in aPL profile.…”

Section: Caps Managementmentioning

confidence: 99%

Is there a role for immunosuppression in antiphospholipid syndrome?

2019

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Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition (eculizumab), mechanistic target of rapamycin inhibition (sirolimus), vascular endothelial cell modulation (defibrotide), statins, and traditional rheumatologic disease–modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide).

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“…[4] In recent years, due to its ability to induce plasma cell apoptosis, [5][6][7] proteasome inhibitor bortezomib has been used alone or in combination with dexamethasone, rituximab, plasma exchange and other drugs to treat autoimmune diseases such as AIHA, Evans syndrome and immune thrombocytopenia. [8][9][10][11][12][13] The standard dose of rituximab is 375 mg/m 2 per week for 4 weeks. Still, it has been reported that low-dose rituximab 100 mg/d has a similar effect, [14] reducing the financial burden of patients and also the adverse reactions.…”

Section: Introductionmentioning

confidence: 99%

Combination of low-dose rituximab, bortezomib and dexamethasone for the treatment of autoimmune hemolytic anemia

Yao

Zhang

et al. 2022

Medicine

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Autoimmune hemolytic anemia (AIHA) therapy may be associated with severe complications such as diabetes, hypertension, obesity, osteoporosis, peptic ulcers, infection, and some other diseases. To reduce those effects, we used low-dose rituximab, bortezomib and dexamethasone (LowR-BD regimen) to treat AIHA. The purpose of this study was to evaluate the efficacy and safety of this regimen. Seven patients with warm AIHA (wAIHA) admitted from March 2020 to October 2020 were treated with LowR-BD regimen: Rituximab 100 mg by intravenous infusion on day 1 combined with bortezomib 1.3 mg/m 2 by subcutaneous injection on day 2 plus dexamethasone 20 mg by intravenous infusion on days 2, 3. Clinical efficacy and safety were assessed at the regular reexamination of relevant indicators and follow-up. After 4 cycles of the LowR-BD regimen, the overall response rate (ORR) was 85.71% with a complete response (CR) of 28.57% and a partial response (PR) of 57.14%. After a median follow-up of 12 (range 7–13) months, 5 patients achieved CR and 2 patients had PR status, including 1 patient who did not respond to LowR-BD treatment and reached CR after using methylprednisolone combined with cyclophosphamide. One patient relapsed and achieved PR after retreatment of 2 cycles LowR-BD regimen. The patients tolerated the treatment well and did not complain of apparently adverse reactions except a patient with Sjogren's syndrome and bronchiectasis who developed a severe infection during treatment. Low-dose rituximab combined with bortezomib and dexamethasone is effective and relatively safe in patients with wAIHA.

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Relapsing Evans syndrome and systemic lupus erythematosus with antiphospholipid syndrome treated with Bortezomib in combination with plasma exchange

Cited by 11 publications

References 9 publications

Bortezomib in autoimmune hemolytic anemia and beyond

Bortezomib in autoimmune hemolytic anemia and beyond

Is there a role for immunosuppression in antiphospholipid syndrome?

Combination of low-dose rituximab, bortezomib and dexamethasone for the treatment of autoimmune hemolytic anemia

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