Rituximab for eradicating inhibitors in people with acquired haemophilia A

Remmington, Tracey; Smith, Sherie

doi:10.1002/14651858.cd011907.pub3

Cited by 6 publications

(7 citation statements)

References 102 publications

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“…The combined treatment of steroids with cyclophosphamide is able to eradicate inhibitors in approximately 70% of patients with AHA. The monoclonal antibody rituximab is a chimeric antibody targeting CD20 antigen on B-cell surface [ 42 ]. It has been reported to be effective in eradicating the inhibitors in AHA and 172 patients with this condition have been treated so far, including the present cases [ 43 ].…”

Section: Discussionmentioning

confidence: 99%

A Diagnostic of Acquired Hemophilia Following PD1/PDL1 Inhibitors in Advanced Melanoma: The Experience of Two Patients and a Literature Review

et al. 2022

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Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the development of specific autoantibodies against factor VIII (FVIII). Immunotherapy is a recent therapeutic option that targets the patient’s self-tolerance against tumor cells. Because therapeutic effects of the immune checkpoint inhibitors (ICIs) are mediated by enhancing the immune response to restore antitumor immunity, autoimmune-related adverse effects can be seen in up to 80% of patients during treatment and after treatment. A rare hematologic ICIs-related adverse event is AHA. Hereafter we report two cases of AHA developed during anti-PD-1 immunotherapy for advanced melanoma: one secondary to treatment with nivolumab and one secondary to pembrolizumab. Both patients were treated with activated FVII (Novoseven®, Novo Nordisk, Bagsværd, Denmark) as hemostatic treatment combined with the eradication of antibodies anti-FVIII obtained with rituximab. In the last few years these drugs have significantly improved the therapeutic armamentarium for the management of AHA. Indeed, while FVIIa has proven to be an effective and safe tool for the treatment of acute bleeding related to FVIII autoantibodies, rituximab is a promising alternative for the autoantibodies’ elimination and the restoration of normal hemostasis. Our finding supports the use of this combination even in AHA secondary to ICIs treatment.

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Section: Discussionmentioning

confidence: 99%

A Diagnostic of Acquired Hemophilia Following PD1/PDL1 Inhibitors in Advanced Melanoma: The Experience of Two Patients and a Literature Review

et al. 2022

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“…Zbog brzog uklanjanja inhibitora iz cirkulacije mogu se primijeniti plazmafereza i imunoadsorpcija. u refraktornim slučajevima ili pri kontraindiciranoj primjeni drugih imunospresivnih lijekova primjenjuje se liječenje rituksimabom, anti-Cd20 monoklonalnim protutijelom (375 mg/m 2 ), čime se postiže kontrola krvarenja i uklanjanje stečenih protutijela, većinom uz istodobnu primjenu kortikosteroida ili drugih imunosupresivnih lijekova (1,9,18,19).…”

Section: Raspravaunclassified

Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

Malić Tudor,

Armanda

2024

Paediatr Croat

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Acquired coagulopathy is a rare, potentially life-threatening disorder which should be suspected if acute bleeding in patients with a negative previous hemorrhagic diathesis is encountered. This disease arises due to the formation of antibodies against clotting factors in a previously healthy child or adult, which specifically partially or completely neutralize the procoagulant activity of clotting factors or accelerate their removal from the circulating blood, reducing their plasma concentrations and increasing the bleeding tendency. The aforementioned disorder should be confirmed by determining global coagulation times, concentrations of individual clotting factors, and determining the presence of specific or non-specific antibodies. Timely treatment aimed at prompt stopping of bleeding and eradicating the resulting inhibitors will prevent the unfavorable outcome. It is recommended to continue monitoring patients in the outpatient setting with determination of PT and APTT for a year, because in 20% of cases a relapse of the disease occurs, although more often in adults. In this paper, we presented a three-year-old boy in whom we determined prolonged global coagulation times and the presence of non-specific inhibitors of the coagulation factors F II, F IX, F XI and F XII through the diagnostic workup of macrohematuria. The treatment with fresh frozen plasma and corticosteroids resulted in clinical improvement and disease remission. Acquired coagulopathy is a very rare disorder in childhood with scarce literature data. The aim of this paper is to demonstrate the possible causes, clinical course and treatment of this life-threatening disorder in children

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“… 4 , 113 , 115–117 However, there are published experiences with rituximab in AHA at doses of 100mg/m 2 /weekly with good results. 118 , 119 …”

Section: Immunosuppressive Treatment To Eradicate Neutralizing Autoan...mentioning

confidence: 99%

“…4,113,[115][116][117] However, there are published experiences with rituximab in AHA at doses of 100mg/m 2 /weekly with good results. 118,119 The results of IST from EACH2 113 show that the association of steroids and cyclophosphamide achieved a CR rate of 77%, steroids in monotherapy 48%, rituximab and other immunosuppressive drug 59% and rituximab in monotherapy scheme dropped to 42%. In addition, to avoid bias derived from the different clinical characteristics of the patients, they perform a statistical analysis using the "propensity score", to control age, gender, factor VIII levels, inhibitor titer and underlying pathologies, concluding that the combination of steroids with cyclophosphamide achieves a significantly higher CR rate (p<0.003).…”

Section: Immunosuppressive Treatment To Eradicate Neutralizing Autoan...mentioning

confidence: 99%

Acquired Haemophilia A: A Review of What We Know

Mingot‐Castellano¹,

Rodríguez-Martorell²,

Nuñez-Vázquez³

et al. 2022

JBM

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Autoantibodies against plasma coagulation factors could be developed by some individuals inducing severe and sometimes fatal bleedings. This clinical entity is called acquired haemophilia. It should be suspected in subjects with acute abnormal bleedings, without personal or familiar history of congenital bleeding disorders with an unexplained prolonged aPTT. It is rare disease, although its incidence may be underestimated due to the low knowledge about it by many specialists, the frequent use of anticoagulant or antiplatelet therapies in the affected population that can mask the diagnosis and, sometimes, a so withering effect that avoid its confirmation. Mortality ranges between 9% and 33% depending on the series in the first 2 months after diagnosis. This mortality is attributed in up to 40% of the cases to infections in the context of immunosuppressive treatments used to eliminate the inhibitor. Factor VIII levels below 1% and high inhibitor titers are conditions of worse response rates. Advanced age, patient’s ECOG, and underlying conditions are key prognostic factors for response to treatment and patient survival. To reduce morbidity and mortality in these patients, it is important to have clinical knowledge and access to guidelines to achieve an early diagnosis and to optimize the haemostatic and immunosuppressive treatment. This review aims to contribute to the dissemination of basic concepts on the epidemiology etiopathogenesis, diagnosis, treatment and management of these patients, as well as risk factors to get remission and the longest overall survival to allow individualized care. Especial awareness will be proposed in patients with some underlying conditions like cancer, autoimmune diseases, children, pregnancy or drugs.

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Rituximab for eradicating inhibitors in people with acquired haemophilia A

Cited by 6 publications

References 102 publications

A Diagnostic of Acquired Hemophilia Following PD1/PDL1 Inhibitors in Advanced Melanoma: The Experience of Two Patients and a Literature Review

A Diagnostic of Acquired Hemophilia Following PD1/PDL1 Inhibitors in Advanced Melanoma: The Experience of Two Patients and a Literature Review

Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

Acquired Haemophilia A: A Review of What We Know

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