Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy

Machado, Patricia; Raya, José‐María; Martin, Thomas; Morabito, Lucio; Brito, Miguel; Rodriguez-Martin, Juan

doi:10.1007/s12185-008-0077-4

Cited by 11 publications

(5 citation statements)

References 38 publications

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“…However, up to one third of patients are refractory to conventional strategies [12]. In non-responders, treatment with alternative drugs such as high-dose immunoglobulin, cyclosporine and, in particular, rituximab has been proposed [13]. …”

Section: Discussionmentioning

confidence: 99%

Efficacy of Low-Dose Rituximab in a Refractory Acquired Factor VIII Inhibitor Case Secondary to Pemphigus

Gao¹,

Liu²,

Ma³

et al. 2011

Acta Haematol

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Acquired factor VIII (FVIII) inhibitor induces a bleeding disorder caused by specific antibodies to FVIII. The cause of approximately one fifth of cases can be attributed to autoimmune disorders, such as pemphigus. Here, we describe a case of refractory acquired FVIII inhibitor in a patient with primary pemphigus and its successful treatment with low-dose rituximab. Coagulation studies revealed a prolonged activated partial thromboplastin time, which could not be corrected with the mixing test. At the same time, the FVIII activity level was significantly reduced, and the FVIII inhibitor titer was elevated. A treatment regimen with prednisolone/cyclophosphamide followed by prednisolone/cyclosporine was used. The patient temporarily responded but then became resistant to these medicines. However, subsequent treatment with low-dose rituximab achieved considerable clinical and laboratory improvement in the same patient. Follow-up at 6 months revealed a low level of residual FVIII inhibitor activity with normal coagulation functions. No drug-related side effects were detected. In conclusion, our results indicate that low-dose rituximab might be an effective and safe treatment for patients with acquired FVIII inhibitor.

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Section: Discussionmentioning

confidence: 99%

Efficacy of Low-Dose Rituximab in a Refractory Acquired Factor VIII Inhibitor Case Secondary to Pemphigus

Gao¹,

Liu²,

Ma³

et al. 2011

Acta Haematol

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“…Other approaches should be considered for refractory patients. In particular, rituximab may be suggested when first-line immunosuppressive treatment fails or is contraindicated [5, 10–14]. …”

Section: Discussionmentioning

confidence: 99%

“…[1–6]. In nonresponders, alternative approaches have been proposed, in particular, using rituximab [4, 10–14]. This chimeric human/mouse monoclonal antibody directed at the CD20 antigen expressed in mature-B and pre-B lymphocytes induces B-cell depletion rapidly and completely.…”

Section: Introductionmentioning

confidence: 99%

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Acquired Haemophilia A in the Elderly: Case Reports

Tufano

Coppola

Guida

et al. 2010

Current Gerontology and Geriatrics Research

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Acquired hemophilia A (AHA) is a very rare disease, caused by the development of autoantibodies, directed against circulating factor VIII of coagulation. Age distribution is bimodal, with a first peak occurring among young women in the postpartum period, and a second major peak of incidence among elderly patients in whom it is frequently associated with malignancy and drugs. This disease often represents a life-threatening bleeding condition, especially in the elderly, thus requiring a prompt therapeutic intervention, including control of acute bleeding and eradication of the inhibitor by immunosuppressive therapy. The diagnosis of AHA should be considered in any elderly patient who presents with bleeding and prolonged activated Partial Thromboplastin Time. Moreover, the coexistence of a series of underlying diseases associated with AHA should be always searched for. An early recognition and an adequate treatment of this coagulation disorder and of the possible associated diseases play a significant role for a favourable outcome, but concomitant morbidities in the elderly may limit aggressive therapy and may complicate the clinical scenario. We report 3 consecutive elderly patients successfully treated with recombinant activated factor VII and standard immunosuppressive regimens, with remission of the disease.

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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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Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy

Cited by 11 publications

References 38 publications

Efficacy of Low-Dose Rituximab in a Refractory Acquired Factor VIII Inhibitor Case Secondary to Pemphigus

Efficacy of Low-Dose Rituximab in a Refractory Acquired Factor VIII Inhibitor Case Secondary to Pemphigus

Acquired Haemophilia A in the Elderly: Case Reports

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

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