Spinal epidural hematoma in a child with hemophilia A with high titer inhibitors and follow-up with prophylactic emicizumab: case report and literature review.

Autor: Villarreal-Martínez L; Hematology Service., Sepúlveda-Orozco MDC; Pediatric Department, Hospital Universitario 'Dr José Eleuterio González', Universidad Autónoma de Nuevo León, Monterrey, México., García-Viera DA; Hematology Service., Robles-Sáenz DA; Hematology Service., Bautista-Gómez AJ; Hematology Service., Ortiz-Castillo M; Hematology Service., González-Martínez G; Hematology Service., Mares-Gil JE; Hematology Service.
Jazyk: angličtina
Zdroj: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis [Blood Coagul Fibrinolysis] 2021 Sep 01; Vol. 32 (6), pp. 418-422.
DOI: 10.1097/MBC.0000000000001038
Abstrakt: Hemorrhage in the central nervous system is the most severe and debilitating manifestation affecting patients with hemophilia A. The spinal epidural space is the most unusual and clinically challenging site of central nervous system hemorrhage in hemophilia A. These patients often show insidious neurological signs and symptoms that delay diagnosis and treatment. We share our experience treating a 4-year-old male patient with severe hemophilia A and high titer inhibitors with a spontaneous spinal epidural hematoma. The patient presented initially with intense headache and neck pain. After blood tests and imaging studies, bypassing agent therapy with recombinant-activated factor VII was used until discharge; this was later replaced with emicizumab. After 18 months, the patient is without neurological sequelae and has not experienced subsequent bleeding episodes. We review the available literature and discuss the relevance of emicizumab compared with standard therapies in the context of spontaneous spinal epidural hematoma.
(Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)
Databáze: MEDLINE