Haemophilia in South Asia: A perspective from Bangladesh

Hossain, Mohammad Sorowar; Mosabbir, Abdullah Al

doi:10.1111/hae.14445

Cited by 2 publications

(2 citation statements)

References 10 publications

(13 reference statements)

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“…Additionally, despite majority of the participants presented with target joints at baseline, 100% of patients reported zero treated target joint bleeds after receiving emicizumab prophylaxis, which was higher than the published studies of both standard and extended half-life FVIII prophylaxis regimens [3,22,23], indicating that emicizumab prophylaxis given subcutaneously can provide effective bleed prevention. This is the first study to investigate the efficacy and safety of emicizumab prophylaxis in adults and adolescents with hemophilia A, both with and without FVIII inhibitors in Bangladesh, a lower middleincome country where only 5.5% of hemophilia A patients receive prophylactic treatment and most of the patients cannot afford standard treatment and are receiving inadequate on-demand therapy for hemophilia A [24]. Majority of the participants included in the study were above the age of 18 years, had spontaneous bleeding episodes of more than 20, were mostly moderate cases of hemophilia A, and did not have FVIII inhibitors or a record of ITI therapy or prior use of bypassing agents, which was anticipated and consistent with demographic information on the hemophilia A population in previous studies [15,25].…”

Section: Discussionmentioning

confidence: 99%

“…This is the first study to investigate the efficacy and safety of emicizumab prophylaxis in adults and adolescents with hemophilia A, both with and without FVIII inhibitors in Bangladesh, a lower middle‐income country where only 5.5% of hemophilia A patients receive prophylactic treatment and most of the patients cannot afford standard treatment and are receiving inadequate on‐demand therapy for hemophilia A [24]. Majority of the participants included in the study were above the age of 18 years, had spontaneous bleeding episodes of more than 20, were mostly moderate cases of hemophilia A, and did not have FVIII inhibitors or a record of ITI therapy or prior use of bypassing agents, which was anticipated and consistent with demographic information on the hemophilia A population in previous studies [15, 25].…”

Section: Discussionmentioning

confidence: 99%

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Effectiveness of emicizumab in preventing bleeding events in severe and moderate hemophilia A: A single‐center experience in Bangladesh

Tory,

Ghosh,

Nazneen

et al. 2024

eJHaem

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Emicizumab is a monoclonal antibody that bridges activated factor IX (FIX) and factor X (FX) to replace the function of missing activated factor VIII (FVIII) in hemophilia A patients irrespective of FVIII inhibitor status. This study assessed the effectiveness of emicizumab in preventing bleeding episodes in patients with hemophilia A. This observational study included patients with moderate to severe hemophilia A who were undergoing episodic FVIII replacement therapy. The primary endpoint was the difference in annualized bleeding rates (ABR) and the secondary endpoint was the difference in Hemophilia Joint Health Score (HJHS) before and after emicizumab prophylaxis. A total of 30 male hemophilia patients were included, the mean age was 16.7 (SD: ±8.1) years, and most of them had moderate hemophilia A [63.3%]. Before prophylaxis, the median ABR was 48 (interquartile range [IQR]: 35–60), and 93.3% of patients had ABR greater than eight, whereas after prophylaxis the median ABR decreased significantly (median [IQR]: 0 [0.0–0.4], p < 0.001), and 56.7% had zero bleeds. ABR was not significantly different in patient with and without FVIII inhibitors. The HJHS scores significantly improved after prophylaxis (10 vs. 2.5, p < 0.001). The bleeding events were reduced significantly (23 vs. 0.0, p < 0.001), and zero new target joints were reported after prophylaxis. Most of the patients [93.3%] did not face any serious adverse events after prophylaxis. Emicizumab prophylaxis was associated with a significantly lower rate of bleeding events among participants with hemophilia A, regardless of inhibitor status.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Effectiveness of emicizumab in preventing bleeding events in severe and moderate hemophilia A: A single‐center experience in Bangladesh

Tory,

Ghosh,

Nazneen

et al. 2024

eJHaem

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Functional evaluation of joint in moderate to severe hemophilia patients treated with on-demand factor replacement: insights from a single hemophilia treatment center in Bangladesh

Ghosh,

Tory,

Nazneen

et al. 2024

Egypt J Intern Med

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Background Hemophilia patients are prone to spontaneous or post-traumatic bleeding, particularly in joints and various other body sites. The high incidence of joint hemorrhages in hemophilia patients underscores the importance of routine joint assessments in clinical care to monitor the condition and evaluate the efficacy of hemophilia treatments. Aim This study aimed to assess the joint health of hemophilia patients receiving FVIII or FIX on-demand replacement therapy treatment at a tertiary care hospital in Bangladesh. Methods This single-center study involved 93 patients diagnosed with moderate (n = 72;77.4%) and severe (n = 21;22.6%) hemophilia. The assessment of joint health was carried out on six key joints—both elbows, knees, and ankles—using the Hemophilia Joint Health Score (HJHS) version 2.1. Clinical and laboratory data were meticulously documented in a structured questionnaire. Results Among the 93 hemophilic patients, the majority had hemophilia A (96.8%). The median HJHS of the patients was 15 (IQR:9.5–20) with the knee joint being mostly affected (88.2%). A significant positive correlation was observed between HJHS and age (r = 0.385, p < 0.001) and total number of joints involved (r = 0.405, p < 0.001). No significant association was found between factor level, education status, and socio-economic status with HJHS score. Conclusion Early assessment and monitoring of joint health are essential in clinical practice to prevent severe complications in hemophilia patients.

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Haemophilia in South Asia: A perspective from Bangladesh

Cited by 2 publications

References 10 publications

Effectiveness of emicizumab in preventing bleeding events in severe and moderate hemophilia A: A single‐center experience in Bangladesh

Effectiveness of emicizumab in preventing bleeding events in severe and moderate hemophilia A: A single‐center experience in Bangladesh

Functional evaluation of joint in moderate to severe hemophilia patients treated with on-demand factor replacement: insights from a single hemophilia treatment center in Bangladesh

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