Management of acquired haemophilia A

Tiede, Andreas; Scharf, Rüdiger E.; Dobbelstein, Christiane; Werwitzke, Sonja

doi:10.5482/hamo-14-11-0064

Cited by 28 publications

(7 citation statements)

References 30 publications

(23 reference statements)

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“…[12] Most factor VIII allo antibodies are directed against epitopes in the A2 and A3-C1 domain of factor VIII, or auto antibodies, those that suddenly appear in person with normal factor VIII gene and previously normal plasma level of plasma factor VIII, causing acquired hemophilia. [131415] Factor affecting development of inhibitor classified as non-modifiable and modifiable. Non-modifiable risk factors include high risk hemophilia genotype, co-stimulatory genotype-immuno genotype interaction, ethnicity and positive family history.…”

Section: Discussionmentioning

confidence: 99%

A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

Kumar

Sinha

Bharti

et al. 2019

J Family Med Prim Care

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Introduction: Deficiency of factor VIII (Hemophilia A), factor IX (Hemophilia B) and Von Willebrand's factor are the most frequent coagulation defects. The incidence of inhibitors in patients of factor VIII deficiency is varies in different regions of India. Aim: To determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of Hemophilia in north eastern part of India. Methods: Selected patients were under went for complete Blood Count (CBC), General Blood Picture (GBP), Prothrombin time (PT), Activated partial thromboplastin time (APTT), Thrombin time, Correction experiment to know the specific factor deficiency or inhibitors present by Normal Plasma, Normal aged serum, Al(OH) 3 adsorbed plasma. Results: 92 patients diagnosed as suffering with Hemophilia A or B were included in study. The age of patients ranged from 2.5 month to 53 years. Out of 92, seventy nine (85.87%) were Haemophilia A and thirteen were (14.13%) Hemophilia B patients. 3.50% (2/55) cases of treated Hemophilia A patient develop inhibitor. Conclusion: The prevalence of hemophilia and incidence of inhibitors in these patients is varies in different regions of India. This variation may be due to the type of product used as treatment, intensity of treatment or the genetic characteristics of the patients.

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Section: Discussionmentioning

confidence: 99%

A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

Kumar

Sinha

Bharti

et al. 2019

J Family Med Prim Care

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“…Other immunosuppressive regimens include azathioprine, bortezomib, and mycophenolate mofetil, among others [ 6 , 18 , 60 , 61 ]. Recently, rituximab has gained increasing acceptance because of the low rate of adverse events, although it is used off label in this indication [ 18 , 62 – 64 ]. Data from the EACH2 registry showed a 58% remission rate with steroids alone, 80% with steroids and cyclophosphamide, and 61% with rituximab-containing combinations [ 18 ].…”

Section: Clinical Managementmentioning

confidence: 99%

Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A

Knöbl

2018

Drugs

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Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies inhibiting the function of coagulation factor VIII. It is characterized by spontaneous bleeding in patients with no previous family or personal history of bleeding. Although several large registries have collected clinical data on AHA, limited information is available on the optimal management of AHA because controlled clinical trials are lacking. AHA can easily be diagnosed if the problem (prolonged activated partial thromboplastin time in a bleeding patient) is recognized. After the effects of anticoagulants are excluded, low factor VIII activity and the detection of circulating inhibitors confirms the diagnosis. However, lack of familiarity with this rare condition may delay diagnosis and adequate therapy. Treatment of AHA is based on measures for prompt hemostatic control to stop (and prevent) bleeding, immunosuppression to eradicate the autoantibodies, and supportive care for the adverse effects of that treatment and patients’ often complex comorbidities. This article gives a comprehensive overview of the current knowledge about the pathophysiology, diagnosis, and treatment of AHA.

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“…It is usually recommended to administer immunosuppressive therapy in patients with AHA to induce remission of the disease. Glucocorticoids, cyclophosphamide, and rituximab, or combinations thereof, are frequently used for immunosuppression in AHA [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

2020

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Immunosuppressive therapy (IST) is administered to patients with acquired hemophilia A (AHA) to eradicate autoantibodies against coagulation factor VIII (FVIII). Data from registries previously demonstrated that IST is often complicated by adverse events, in particular infections. This pilot study was set out to assess the feasibility of reduced-intensity, risk factor–stratified IST. We followed a single-center consecutive cohort of twenty-five patients with AHA receiving IST according to a new institutional treatment standard. Based on results from a previous study, GTH-AH 01/2020, patients were stratified into “poor risk” (FVIII < 1 IU/dl or inhibitor ≥ 20 Bethesda units (BU)/ml) or “good risk” (FVIII ≥ 1 IU/dl and inhibitor < 20 BU/ml). Outcomes were compared between the current cohort and the GTH registry as a historic control (n = 102). Baseline characteristics of the cohort were not different from the historic control. Partial remission, defined as FVIII recovered to > 50 IU/dl, was achieved by 68% of patients after a median time of 112 days, which was lower and significantly later than in the historic control (hazard ratio: 1.8, 95% confidence interval 1.2–2.8). Complete remission, overall survival, and frequency of fatal infections were not different. Grade 3 and 4 infections were more frequent. The impact of risk factors that was observed in the historic cohort was no longer apparent, as partial and complete remission and overall survival were similar in “good risk” and “poor risk” patients. In conclusion, reduced-intensity, risk factor–stratified IST is feasible in AHA but did not decrease the risk of infections and mortality in this cohort.

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Management of acquired haemophilia A

Cited by 28 publications

References 30 publications

A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

A study to determine the prevalence, clinical profile and incidence of formation of inhibitors in patients of hemophilia in north eastern part of India

Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A

Reduced-intensity, risk factor–stratified immunosuppression for acquired hemophilia A: single-center observational study

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