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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2021; 79(12): 1116-1112
DOI: 10.1590/0004-282X-ANP-2020-0467
Articles

Factor VIII prophylactic therapy reduces neurological complications in patients with Hemophilia A

Terapia profilática com Fator VIII reduz complicações neurológicas em paciente com Hemofilia A
José Marcelino Aragão FERNANDES 
1   Universidade Federal do Ceará, Programa de Pós-Graduação em Ciências da Saúde, Sobral CE, Brazil.
2   Centro de Hematologia e Hemoterapia do Ceará, Fortaleza CE, Brazil.
,
Francisco de Assis Aquino GONDIM 
3   Universidade Federal do Ceará, Departamento de Medicina Interna, Fortaleza CE, Brazil.
,
Antônia Maria Negreiro DIAS 
2   Centro de Hematologia e Hemoterapia do Ceará, Fortaleza CE, Brazil.
,
Rosângela de Albuquerque RIBEIRO 
2   Centro de Hematologia e Hemoterapia do Ceará, Fortaleza CE, Brazil.
,
Luiz Ivando Pires FERREIRA  FILHO
2   Centro de Hematologia e Hemoterapia do Ceará, Fortaleza CE, Brazil.
,
Vicente de Paulo Teixeira PINTO 
1   Universidade Federal do Ceará, Programa de Pós-Graduação em Ciências da Saúde, Sobral CE, Brazil.
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ABSTRACT

Background: Bleeding in hemophiliacs can cause complications in the central and peripheral nervous system (CNS and PNS). The incidence of intracranial hemorrhage has reduced after the introduction of prophylactic treatment with factor VIII or IX, but the benefits of this therapy have not yet been evaluated on PNS complications. Objective: The aim of this study was to determine the prevalence of neurological complications in hemophiliacs and verify the effect of prophylactic therapy in these patients, including PNS disorders. Methods: We retrospectively evaluated the prevalence of CNS and PNS disorders caused by bleeding in hemophiliacs seen at the Hemocentro Regional Norte, Ceará, Brazil, from 1992 to 2018, and we compared the incidence in different periods (before and after the introduction of prophylactic treatment in 2011). Results: Of 75 hemophilia A patients evaluated (4.61/100.000 population), 13.3% (n=10) had either CNS (n=5) or PNS (n=5) disorders secondary to bleeding. Patients submitted to factor VIII replacement prophylactic therapy were less likely to have CNS events: from 1992 to 2011, 5 of 63 patients had CNS disease, while from 2011 to 2018, there were no new cases (p=0.0181). From 2011 to 2018, 5 PNS events occurred in patients without prophylactic therapy, whereas none occurred in those covered by prophylactic therapy (5/20 versus 0/29, p=0.0081). Conclusions: The prevalence of neurological complications in hemophiliacs in our cohort is similar to other studies. Similar to CNS, prophylactic therapy also reduces the risk of PNS complications. This is the first report in the literature showing this benefit.

RESUMO

Antecedentes: O sangramento em hemofílicos causa complicações no sistema nervoso central e periférico (SNC e SNP). A incidência de hemorragia intracraniana diminuiu após a introdução da profilaxia com fator VIII ou IX, entretanto esse benefício ainda não foi avaliado no SNP. Objetivo: O objetivo deste estudo foi determinar a prevalência de complicações neurológicas em hemofílicos, verificando o efeito da terapia profilática também no SNP. Métodos: Avaliamos retrospectivamente a prevalência de complicações neurológicas causadas por sangramentos em hemofílicos atendidos no Hemocentro Regional Norte, Ceará, Brasil, de 1992 a 2018, comparando a incidência em diferentes períodos (antes e depois da introdução do tratamento profilático em 2011). Resultados: Foram avaliados 75 pacientes com hemofilia A (4,61/100 mil habitantes). Deles, 13,3% (n=10) tinham distúrbios do SNC (n=5) ou do SNP (n=5) secundários a hemorragias. Os pacientes submetidos à terapia profilática com fator VIII apresentaram menor probabilidade de eventos do SNC: de 1992 a 2011, cinco de 63 pacientes apresentaram hemorragia no SNC, enquanto de 2011 a 2018 não ocorreram novos casos (p=0,0181). De 2011 a 2018, cinco eventos no SNP ocorreram entre pacientes sem terapia profilática, e nenhum ocorreu entre aqueles cobertos pela profilaxia (5/20 × 0/29, p=0,0081). Conclusões: A prevalência de complicações neurológicas em hemofílicos em nossa coorte é similar à de outros estudos. Assim como no SNC, a terapia profilática também reduz o risco de complicações no SNP. Este é o primeiro relato na literatura a mostrar esse benefício.

Keywords:

Hemophilia A - Intracranial Hemorrhages - Peripheral Nervous System Diseases

Palavras-chave:

Hemofilia A - Hemorragias Intracranianas - Doenças do Sistema Nervoso Periférico

Authors’ contributions:

JMAF, FAAG, VPTP: conception and design of the study; JMAF, AMND: performed the research; JMAF, Dias AMND, RAD, LIPFF: patient management/data acquisition; JAMF, FAAG, VPTP: performed data analysis; JMAF, FAAG, VPTP: wrote the manuscript; JMAF, FAAG, VPTP, AMND, RAR, LIPFF: approved the final manuscript.




Publication History

Received: 15 October 2020

Accepted: 09 January 2021

Article published online:
07 June 2023

© 2021. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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