Differences in Major Bleeding Events Between Patients With Severe                     Hemophilia A and Hemophilia B: A Nationwide, Population-Based Cohort                     Study

Shih, Ming-Yang; Wang, Jiaan-Der; Yin, Jia-De; Tsan, Yu‐Tse; Chan, Wei-Cheng

doi:10.1177/1076029619888023

Cited by 7 publications

(3 citation statements)

References 25 publications

(30 reference statements)

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“…In another study by Pawan et al, the major bleeding symptoms were prolonged bleeding on trauma, hemarthrosis, and hematoma [21]. However, in a nationwide population-based cohort study from Taiwan which assessed 137 severe HB patients, there were 80.3% cases with GI bleeding, 52.6% with hemarthrosis, 40.9% with nontraumatic hematoma of soft tissue, and 29.2% with hematuria [23]. Finally, our study shows that the major clinical manifestations are hemarthrosis (especially in the elbows, knees, and ankles) bruising, and oral cavity bleeding.…”

Section: Discussionmentioning

confidence: 98%

Genotype-phenotype analyses of Iranian patients with and without hemophilia B Leyden: A single-center study

Moghadam,

Manafzadeh,

Dajliry

et al. 2024

Preprint

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Background There is a high prevalence of inherited bleeding disorders in Iran, such as hemophilia A (HA) and hemophilia B (HB). This study aimed to analyze the molecular and clinical profiles of patients with HB.Methods A single-center study was conducted among patients with severe HB between March 20, 2000, and June 31, 2023. The polymerase chain reaction (PCR) amplification was used for all of the major regions, such as the promoter, the exons, the adjacent intronic regions, and the untranslated regions of the F9 gene. Finally, Sanger sequencing was performed on the PCR products.Results A total of 111 HB patients (17 with HB Leyden and 94 without HB Leyden) were enrolled in this study. The median age of the patients at the time of diagnosis was 12 months (IQR: 6 months to 60 months). A family history of hemophilia was reported in 64 (57.7%) of patients. The most common bleeding manifestations were hemarthrosis, bruising, and oral cavity bleeding. Among 94 patients without HB Leyden, 59 (62.8%) had missense, 21 (22.3%) had nonsense, and 8 (8.5%) had frameshift mutations. Moreover, the most frequent mutation in HB Leyden was c.-17 A > G in this study. Finally, two novel mutations (c. -14 T > C and c. -56 T > A) were identified in the promotor region.Conclusion The results of this study confirm that HB is caused by a wide range of molecular defects in Iran. Thus, by knowing the genotypes and phenotypes, we would be able to stratify the patients which is important in terms of their management and outcome.

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Section: Discussionmentioning

confidence: 98%

Genotype-phenotype analyses of Iranian patients with and without hemophilia B Leyden: A single-center study

Moghadam,

Manafzadeh,

Dajliry

et al. 2024

Preprint

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“…The severity of hemophilia is classified by the level of coagulation factors: >5.0 IU/dL, mild; 1.0-5.0 IU/dL, moderate; and <1.0 IU/dL, severe hemophilia [2]. Hemophilia patients experience repeated joint bleeding episodes for their lifetime and arthropathy is the main complication of these repeated joint bleeding episodes, which often require orthopedic surgical management [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

Clinical Analysis of Hospitalized Patients with Hemophilia A: Single-hemophilia Treatment Center Experience in Korea over 10 years

2021

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Background: There is lack of data on admitted hemophilia patients in Korea. For this reason, this study was intended to analyze the hospitalization data of hemophilia patients in a regional Hemophilia Treatment Center (HTC) for the first time in Korea. Methods:In this retrospective study, we surveyed hospitalized patients with Hemophilia A (HA) in a HTC for 14 years. Medical records of these hemophiliacs were reviewed and data regarding demographic characteristics, cause of admissions and their outcomes in each patient were obtained. In addition, the data of admitted days, type and amount of Coagulation factor concentrate (CFC) used, treatments other than CFC infusion during the admission days were also obtained from the medical record of each patient.Results: A total 107 patients with hemophilia A were admitted during 14 years. Annual rate of admission of patients with HA was 8%. Mean age on admission was 29.63±19.51 years old and mean admission days were 11.28±5.46 days. Most admissions were occurred in severe and moderate hemophilia patients. The most common cause of admission was bleed control followed by surgery and other reasons. With modified WFH CFC supplementation guideline, all the bleeds were successfully controlled and all surgeries were also successfully conducted with less total CFC consumption compared to the consumed dose of other reports. Conclusion:These results suggest that it is necessary to develop more specified regimens different from WFH guidelines of CFC supplementation for admitted Korean patients with hemophilia A.

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“…Although milder arthropathy is reported in haemophilia B [3] , there appears to be little difference in health-related outcomes between the disorders. One survey of people with severe haemophilia A or B between 1998 and 2013, when treatment largely comprised on-demand factor replacement, found no significant difference in major bleeding events or resulting admissions [4] .…”

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confidence: 99%

Haemophilia specialist nurses’ perceptions of haemophilia B

Chaplin¹,

Birkedal

Crilly

et al. 2021

The Journal of Haemophilia Practice

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Introduction Some clinicians believe that haemophilia B is associated with less bleeding than haemophilia A, yet there appears to be little difference in health-related outcomes. Current clinical practice reduces the risk of bleeds, making differences difficult to measure. We surveyed specialist haemophilia nurses to discern their opinions about the impact of haemophilia B compared to haemophilia A. Methods Between July and September 2020, European and Canadian nurses were invited to complete an online survey (25 questions) about perceptions of management and treatment of haemophilia B. Results Fifty-nine nurses (46 European, 13 Canadian) completed the survey. Bleeding was reported as different in haemophilia B by 37% of respondents, and treatment as different by over half. Opinions and experience around using extended half-life (EHL) products varied. Self-reported confidence in using EHL products was rated at a mean of 7.1 (range 3–10) with 47% believing these would remain the optimal treatment in 2025. Conclusion Some nurses believe haemophilia A and B are managed differently. Variations in experience and levels of confidence in the use of EHL products, combined with a belief that these products will remain an optimal treatment for haemophilia B for the next five years, indicates a need for education to promote confidence and competence.

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Differences in Major Bleeding Events Between Patients With Severe Hemophilia A and Hemophilia B: A Nationwide, Population-Based Cohort Study

Cited by 7 publications

References 25 publications

Genotype-phenotype analyses of Iranian patients with and without hemophilia B Leyden: A single-center study

Genotype-phenotype analyses of Iranian patients with and without hemophilia B Leyden: A single-center study

Clinical Analysis of Hospitalized Patients with Hemophilia A: Single-hemophilia Treatment Center Experience in Korea over 10 years

Haemophilia specialist nurses’ perceptions of haemophilia B

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