Emicizumab prophylaxis in a Korean child with severe hemophilia A and high titer inhibitor: a case report

Ahn, Ji Hyun; Jung, Nani; Shim, Ye Jee; Kim, Heung Sik

doi:10.5045/br.2021.2020184

Cited by 4 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…After 6 months of therapy, the chemoport was removed without the use of other haemostatic agents, and no complications were observed. During emicizumab prophylaxis, no bleeding was observed compared to treatment with bypassing agents (BPA) -46 bleeding episodes occurred in the previous year before emicizumab was initiated [33].…”

Section: Discussionmentioning

confidence: 99%

A 9-year-old patient with severe haemophilia A complicated by factor VIII inhibitor treated with emicizumab – case report and literature review

Tomasik,

Szuba,

Chrostowski

et al. 2024

J Pre Clin Clin Res.

View full text Add to dashboard Cite

Introduction.The case is presented of a 10-year-old boy with severe Haemophilia A, in whom standard therapy led to the development of an inhibitor to factor VIII. Immunological tolerance induction (ITI) did not yield the expected result, and inhibitor titers were not reduced. Due to numerous recurrent bleeds, prophylaxis with activated prothrombin complex concentrate (aPCC) was initiated, with recombinant factor VIIa administered in the case of bleeding episodes. Despite second and third-line ITI treatments, desired outcomes were not achieved, leading to therapy discontinuation and initiation of emicizumab treatment. The new therapy significantly reduced bleeding occurrences. Results. This case clearly illustrates the greater efficacy of emicizumab compared to other drugs, confirming its application in patients with developed factor VIII inhibitors where ITI therapy was ineffective. Prophylaxis with this antibody significantly reduces bleeding frequency and improves the patient's quality of life.

show abstract

Section: Discussionmentioning

confidence: 99%

A 9-year-old patient with severe haemophilia A complicated by factor VIII inhibitor treated with emicizumab – case report and literature review

Tomasik,

Szuba,

Chrostowski

et al. 2024

J Pre Clin Clin Res.

View full text Add to dashboard Cite

show abstract

“… 15 In Korea, the FVIII concentrate is mainly used in managing patients with HA without inhibitors because of the Korean National Health Insurance Service (NHIS) and the opinions of Korean experts. 16 Considering the history of the management of HA in Korea, we can expect that older patients with HA who received pdFVIIIc, mainly on-demand therapy, are more likely to have complications. Further, newly diagnosed neonates or infants with HA who will receive non-factor agents in the future are expected to have different distribution of complications compared to older patients.…”

Section: Introductionmentioning

confidence: 99%

Complication Analysis in Korean Patients With Hemophilia A From 2007 to 2019: A Nationwide Study by the Health Insurance Review and Assessment Service Database

et al. 2023

Self Cite

View full text Add to dashboard Cite

Background There has been remarkable progress in hemophilia A (HA) treatment in Korea. Viral inactivation products were developed in 1989, use of recombinant factor VIII (FVIII) concentrates started in 2002, and prophylaxis expanded thereafter. This study was conducted to identify the changes in complications in HA before and after 1989 or 2002. Methods The study was performed using the 2007–2019 Healthcare Big Data Hub of the Health Insurance Review and Assessment Service. Results Among 2,557 patients, 1,084 had ≥ 1 complication; 829 had joint problems, 328 had viral infections, 146 had neurologic sequelae, and 87 underwent 113 surgeries or procedures due to complications. Patients born after 1989 had a significantly lower risk of viral infections than those born before 1989; 27.1% vs. 1.4% ( P < 0.001, odds ratio [OR], 0.037). Patients born after 2002 had a significantly lower risk of joint problems than those born before 2002; 36.8% vs. 24.7% ( P < 0.001, OR, 0.538). Patients born after 2002 had a higher incidence of neurologic sequelae than those born before 2002; 3.7% vs. 11.1% ( P < 0.001, OR, 3.210). Medical expenses for complication-associated surgeries or procedures were ₩2,957,557,005. Conclusion Viral infections have significantly decreased in Korean patients with HA. The degree of reduction of joint problems was lower than we expected, because it took > 10 years to expand prophylaxis widely. Neurologic sequelae have not decreased; thus, additional efforts to decrease intracranial hemorrhage are needed. We suggest personalized dosing of FVIII and more meticulous care during childbirth to further reduce the complications.

show abstract

Eptacog-alfa/factor-VIII-inhibitor-bypassing-fraction

2021

Reactions Weekly

View full text Add to dashboard Cite

Emicizumab prophylaxis in a Korean child with severe hemophilia A and high titer inhibitor: a case report

Cited by 4 publications

References 10 publications

A 9-year-old patient with severe haemophilia A complicated by factor VIII inhibitor treated with emicizumab – case report and literature review

A 9-year-old patient with severe haemophilia A complicated by factor VIII inhibitor treated with emicizumab – case report and literature review

Complication Analysis in Korean Patients With Hemophilia A From 2007 to 2019: A Nationwide Study by the Health Insurance Review and Assessment Service Database

Eptacog-alfa/factor-VIII-inhibitor-bypassing-fraction

Contact Info

Product

Resources

About