Outcome of CARE: a 6‐year national registry of acquired haemophilia A in China

Sun, Boyang; Xue, Feng; Feng, Ying; Sun, Jing; Yu, Ziqiang; Hou, Ming; Zhang, Jingyu; Zeng, Xiaojing; Zhao, Yongqiang; Lian, Shimei; Huang, Meijuan; Niu, Ting; Cui, Zhongguang; Wu, Jingsheng; Yang, Renchi

doi:10.1111/bjh.16128

Cited by 29 publications

(48 citation statements)

References 26 publications

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“…FVIII:C levels were not predictive of bleeding risk. 9,15,20 The bleeding pattern did not differ from that previously reported, with a large prevalence of bleeding of mucocutaneous origin followed by muscular and, to a lesser extent, urinary, digestive, or retroperitoneal bleeds. 2,3,20 One remarkable finding, not described before in AHA, was the high proportion of patients on antithrombotic therapy in the days before AHA diagnosis, namely one-third of the whole series.…”

Section: Discussionsupporting

confidence: 44%

“…[2][3][4][5] AHA shows a high mortality rate, which ranges between 9% and 33%. 3,[6][7][8][9] Although roughly half of all cases are idiopathic, AHA is often associated with underlying diseases and conditions such as autoimmune disorders, neoplasia, the puerperal period, or drug intake. Bleeding manifestations may occur at varied localizations, spontaneously or induced by trauma or invasive procedures, and differ greatly in severity, being sometimes life-threatening.…”

Section: Introductionmentioning

confidence: 99%

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Management of acquired hemophilia A: results from the Spanish registry

et al. 2021

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The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed-up for a median time of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only one patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy (IST). Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), while no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within two months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of non-survivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other non-survivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.

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Section: Discussionsupporting

confidence: 44%

Section: Introductionmentioning

confidence: 99%

Management of acquired hemophilia A: results from the Spanish registry

et al. 2021

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“…As stated above, this study demonstrated the superior efficacy of steroid/cyclophosphamide combination therapy compared to steroid monotherapy, which is in line with the EACH2 and CARE registries. 15,16 We additionally showed that steroids combined with rituximab results in significantly higher remission rates as well.…”

Section: Discussionmentioning

confidence: 83%

“…These results are comparable with most of the AHA cohort studies. 3,15,16,20 In the GTH-AH 01/2020 study some additional potential negative predictors for survival were described, which include a FVIII activity <1 IU/dL, a poor WHO-performance status and the presence of aFVIII IgA. 5,8 In this cohort many patients died of an AHA unrelated cause or were already in CR, which could explain the lack of identified" AHA-specific" markers and the identification of general markers of a poor survival instead.…”

Section: Discussionmentioning

confidence: 98%

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

et al. 2020

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Acquired hemophilia A (AHA) is a severe auto‐immune bleeding disorder. Treatment of AHA is burdensome and optimal management is still unresolved. Therefore a retrospective nationwide multi‐center cohort study (1992‐2018) was performed to evaluate clinical presentation and treatment efficacy and safety of AHA in the Netherlands. Multivariate logistic and Cox regression analysis was used to study independent associations between patient characteristics and clinical outcomes. A total of 143 patients (median age 73 years; 52.4% male) were included with a median follow‐up of 16.8 months (IQR 3.6‐41.5 months). First‐line immunosuppressive treatment was mostly steroid monotherapy (67.6%), steroids/cyclophosphamide (11.9%) and steroids/rituximab (11.9%), with success rates of 35.2%, 80.0% and 66.7% respectively, P < .05. Eventually 75% of patients achieved complete remission (CR). A high anti‐FVIII antibody titer, severe bleeding and steroid monotherapy were associated with lower CR rates. Infections, the most important adverse event, occurred significantly more often with steroid combination therapy compared to steroids alone (38.7% vs 10.6%; P = .001). Overall mortality was 38.2%, mostly due to infections (19.2%) compared to 7.7% fatal bleeds. Advanced age, underlying malignancy and ICU admission were predictors for mortality. This study showed that AHA is characterized by significant disease‐related and treatment‐related morbidity and mortality. A high anti‐FVIII titer, severe bleeding and steroid monotherapy were associated with a lower CR rate. The efficacy of steroid combination therapies however, was overshadowed by higher infection rates and infections represented the most important cause of death. The challenging and delicate balance between treatment effectivity and safety requires ongoing monitoring of AHA and further identification of prognostic markers.

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“…disease prevalence, clinical and treatments scenarios) to medical experts and healthcare providers on acquired bleeding coagulopathies. Some countries [62][63][64][65] have already national registries for inherited bleeding disorders, allowing easy and permanent access to patients' data which favors prompt treatment, improves quality of care and facilitates administrative, statistical, economic and research activities. It could be beneficial to develop an informative tool implemented at a nationwide level to guide better healthcare decisions for the management of acquired hemorrhagic coagulopathies.…”

Section: Discussionmentioning

confidence: 99%

Portuguese Consensus and Recommendations for Acquired Coagulopathic Bleeding Management (CCBM)

Gomes¹,

Rodrigues

Carrilho

et al. 2021

Clin Appl Thromb Hemost

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We aimed to determine how Portuguese physicians handle major bleeding. We also aim to establish global diagnostic and therapeutic recommendations to be followed in clinical practice by using a step-wise approach of evidence generation. This study followed a three-step process: a steering committee desk review, a Delphi technique, an expert panel meeting. A modified 3-round Delphi including 31 statements was performed. Questions were answered in a five-point Likert-type scale. Consensus threshold was established as a percentage of agreement among participants ≥90% in the first round, and ≥85% in the second and third rounds. The level of consensus achieved by panelists was discussed with the scientific committee (January-2020). Fifty-one physicians participated in the study (compliance rate >90%). Analyzing the three rounds, consensus was reached on 20 items (64.5%) in the first, 4/11 items (36.4%) in the second and 6/7 items (85.7%) in the third. One statement about administration of clotting factor concentrates for bleeding control did not reach consensus. A high level of consensus was reached toward the need for implementing Patient Blood Management strategies in Portuguese hospitals, reduce exposure to allogeneic blood components, to use goal directed therapies for acquired bleeding management, and the need for evaluating blood transfusion indirect costs. A final version with 12 recommendations was built, according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Our results provide critically appraised and updated evidence on bleeding coagulopathies management in Portugal. Additional studies, mainly about indirect costs of blood transfusion, are needed.

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Outcome of CARE: a 6‐year national registry of acquired haemophilia A in China

Cited by 29 publications

References 26 publications

Management of acquired hemophilia A: results from the Spanish registry

Management of acquired hemophilia A: results from the Spanish registry

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

Portuguese Consensus and Recommendations for Acquired Coagulopathic Bleeding Management (CCBM)

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