Low-dose rituximab in the treatment of acquired haemophilia

Yao, Qingmin; Zhu, Xiaojuan; Liu, Yanxia; Yuan, Ting; Xu, Jian; Wang, Xin

doi:10.1179/1607845414y.0000000152

Cited by 13 publications

(19 citation statements)

References 16 publications

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“…1 Rituximab is often used at the licensed dose for the treatment of non-Hodgkin lymphoma (i.e., 375 mg/m 2 /week), but has also been used in lower doses of 100 mg/week in some reported cases. [62][63][64][65] The drug is licensed for the treatment of rheumatoid arthritis at a fixed dose of 1,000 mg on days 1 and 15. 66 This regimen has also been used in immune thrombocytopenia.…”

Section: Rituximabmentioning

confidence: 99%

International recommendations on the diagnosis and treatment of acquired hemophilia A

et al. 2020

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A cquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged activated partial thromboplastin time should never be ignored prior to invasive procedures. Control of acute bleeding and prevention of injuries that may provoke bleeding are top priorities in patients with AHA. We recommend treatment with bypassing agents, including recombinant activated factor VII, activated prothrombin complex concentrate, or recombinant porcine FVIII in bleeding patients. Autoantibody eradication can be achieved with immunosuppressive therapy, including corticosteroids, cyclophosphamide and rituximab, or combinations thereof. The median time to remission is 5 weeks, with considerable interindividual variation. FVIII activity at presentation, inhibitor titer and autoantibody isotype are prognostic markers for remission and survival. Comparative clinical studies to support treatment recommendations for AHA do not exist; therefore, we provide practical consensus guidance based on recent registry findings and the authors' clinical experience in treating patients with AHA.

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Section: Rituximabmentioning

confidence: 99%

International recommendations on the diagnosis and treatment of acquired hemophilia A

et al. 2020

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“…This approach has previously only been described in case reports. [12][13][14][15] Within this cohort, some of the patients received 100 mg weekly for 4 weeks (in conjunction with prednisolone). Efficacy of low-dose rituximab has been well-described in other autoimmune conditions including immune thrombocytopenia, autoimmune haemolytic anaemia and ANCA vasculitis.…”

Section: Discussionmentioning

confidence: 99%

“…Over the past decade, several case reports have described the use of a fixed low-dose rituximab schedule (100mg weekly IV for 4 doses) in AHA, typically with rapid immunologic response, few side effects and clinical outcomes comparable to more intensive regimens. [12][13][14][15] The primary aim of this study was to define the incidence, clinical characteristics and outcomes of AHA patients managed through the Queensland Haemophilia Treatment Centre (HTC) over a 4 years period, highlighting the heterogeneity of haemostatic and inhibitor eradication treatment that occurs in the absence of an established protocol. This study highlights early experience using a low-dose rituximab-based regimen in a subset of patients, demonstrating a simplified treatment with reduced toxicity and improved outcomes.…”

Section: Introductionmentioning

confidence: 99%

A low‐dose rituximab regimen for first‐line treatment of acquired haemophilia A

Hunt

Robertson

Casey

et al. 2021

European J of Haematology

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Novelty statement:1. What is the new aspect of your work?• Acquired haemophilia A (AHA) typically occurs in elderly patients with multiple comorbidities. Immunosuppression has demonstrated improved overall survival and remission rates; however, treatment-related complications in this cohort often occur. Efficacious but dose-reduced immunosuppression for this rare condition will hopefully reduce morbidity and is an important area for further research. What is the central finding of your work?• Universal immunosuppression in AHA patients improves overall survival, and 87.5% of patients achieved a complete remission in a median of 48 days. The use of upfront rituximab with corticosteroids is increasing and is associated with shorter times to complete remission. Low-dose rituximab (100 mg/m 2 weekly IV for 4 doses) is emerging as an alternative and appears non-inferior in this case series.

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“…A monthly scheme was preferred in our patient, since the hematological condition imposed a more aggressive treatment than those used in RA. Rituximab therapy is also reported in other cases of AH not related to autoimmune diseases [53][54][55][56][57][58][59][60][61][62][63].…”

Section: Case Reportmentioning

confidence: 99%

Acquired Hemophilia in a Patient With Rheumatoid Arthritis: Case Report and Literature Review

Jaber

Bortolotti

Martinelli

et al. 2019

CMI

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Acquired hemophilia (AH) is a rare bleeding disorder caused by the spontaneous development of autoantibodies against coagulation factors, most commonly factor (F) VIII (acquired hemophilia A, AHA). The clinical manifestation of AHA includes mostly spontaneous hemorrhages into skin, mucous membranes, muscles, soft tissues, or joints. AHA should be suspected when a patient with no history of hemorrhages presents with bleeding and an unexplained prolonged activated partial thromboplastin time. The diagnosis is based on the clinical picture, the presence of low FVIII activity and evidence of FVIII inhibitor. In around half of patients, an underlying disorder (rheumatic diseases, malignancy, infections) or taking some drugs are associated with AHA; the remaining cases are idiopathic. Rheumatoid arthritis is a chronic inflammatory condition, marked by swelling and tenderness of small joints; it is usually treated with steroid and immunosuppressive drugs such as methotrexate, TNF-alpha inhibitors, and other biologic therapies (abatacept, tocilizumab, rituximab).We presented a patient with rheumatoid arthritis who developed acquired hemophilia A with hemarthroses; starting from this case, we focused on the literature about AHA in rheumatic diseases. We found 35 cases, 15 in systemic lupus erythematosus and 12 in rheumatoid arthritis, while the remaining cases were reported in Sjögren’s syndrome, polymyalgia rheumatica, systemic sclerosis, and psoriatic arthritis. Ecchymosis and cutaneous hematomas were the main clinical features while hemarthroses was quite a rare condition, shown in just three patients.

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Low-dose rituximab in the treatment of acquired haemophilia

Abstract: At a follow-up of 34 months, the patient remains in remission without further treatment, suggesting low-dose rituximab seems to be a safe and effective regimen for the elderly patients with acquired haemophilia.

Cited by 13 publications

References 16 publications

International recommendations on the diagnosis and treatment of acquired hemophilia A

International recommendations on the diagnosis and treatment of acquired hemophilia A

A low‐dose rituximab regimen for first‐line treatment of acquired haemophilia A

Acquired Hemophilia in a Patient With Rheumatoid Arthritis: Case Report and Literature Review

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