The key complications of hemophilia and recent advancements in their management: an update

Afrisya Adlina Mohd Azhar; Alicia Ann Raj; Filzah Izam; Muhmammad Danial Mokhtar; Nur Asfarina Izzati Aziz Zakaria; Kishore Gopal Banerjee

doi:10.18203/2320-6012.ijrms20212258

Authors

Afrisya Adlina Mohd Azhar Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia
Alicia Ann Raj Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia
Filzah Izam Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia
Muhmammad Danial Mokhtar Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia
Nur Asfarina Izzati Aziz Zakaria Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia
Kishore Gopal Banerjee Department of Pathology, International Medical School, Management and Science University (MSU), Malaysia

DOI:

https://doi.org/10.18203/2320-6012.ijrms20212258

Keywords:

Hemophilia, Bleeding disorders, Factor VIII, Complications, Inhibitors, Prophylaxis, Management, Arthropathy, Psychosocial

Abstract

Hemophilia A is an X-linked recessive disorder which is due to factor VIII deficiency. It is a life-threatening coagulation disorder leading to complications including hemophilic-arthropathy, development of inhibitors, transfusion-related infections and profound psychosocial impact on the life of the patients. The aim of this study is to analyze the challenges in complications and the latest advancements in managing those complications. A thorough and systemic literature review was done to fulfill the objective of this study. The emergence of hemophilic arthropathy due to repeated symptoms of hemarthrosis is the most frequent complication found in haemophiliacs. Another critical complication, mainly seen in resource-poor settings is the development of lethal and severe bleeding episodes including episodes of intracranial hemorrhage. The development of inhibitors is another major challenge which often adds profoundly to the financial burden faced by hemophilia patients. Another major challenge is acquiring various transfusion-transmitted infections which was particularly more common few decades back. Studies also have revealed that there are myriads of significant psychosocial effects affecting the quality of life of hemophiliacs. Emerging treatments such as gene therapy, non-clotting factor concentrate products and extended half-life therapy can usher a new era in the management and the quality of life in hemophilia patients.

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