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Neurological Complications Associated with Hereditary Bleeding Disorders
Muhammad Qasim Bhatti1, Ezekiel Gonzalez-Fernandez1, Kunal Bhatia1
1Department of Neurology, University of Mississippi Medical Center, Jackson, MS, USA.
Current Neurology and Neuroscience Reports
|October 21, 2023
Summary
Hereditary bleeding disorders like hemophilia and von Willebrand disease can cause severe central nervous system (CNS) bleeding. Effective management involves factor replacement, risk factor identification, and optimal coagulant levels to prevent intracranial hemorrhage (ICH).
Area of Science:
- Neurology
- Hematology
- Genetics
Background:
- Hereditary bleeding disorders present with diverse clinical symptoms, from mild bleeding to severe central nervous system (CNS) complications.
- Intracranial hemorrhage (ICH) is the most feared complication, significantly increasing morbidity and mortality in patients with hemophilia.
Purpose of the Study:
- To review the pathophysiology and clinical manifestations of hemophilia A, hemophilia B, and von Willebrand disease (vWD).
- To emphasize neurological complications, including ICH, and discuss current management strategies.
- To review literature guiding treatment for CNS bleeding in these disorders.
Main Methods:
- Literature review of hereditary bleeding disorders, focusing on neurological complications.
- Analysis of management strategies for CNS bleeding in hemophilia and vWD.
- Evaluation of recent advancements, including Emicizumab therapy.
Main Results:
- ICH is a major cause of mortality in hemophilia patients; optimal factor management is crucial.
- Emicizumab has transformed prophylactic therapies for hemophilia.
- ICH management in vWD requires individualized treatment with factor concentrates and antifibrinolytics.
Conclusions:
- Hemophilia and vWD predispose patients to life-threatening CNS bleeds.
- Prophylactic regimens and early hematologist consultation are vital for prevention.
- Further research is needed on Emicizumab for on-demand ICH treatment in hemophilia.
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