Your browser doesn't support javascript.
loading
Inhibitor development in patients with congenital factor VII deficiency, a study on 50 Iranian patients.
Shams, Mahmood; Dorgalaleh, Akbar; Safarian, Nader; Emami, Amir Hossein; Zaker, Farhad; Tabibian, Shadi; Managhchi, Mohammad Reza; Faranoush, Mohammad; Tabatabaei, Tahere; Satari, Sedighe; Rezvani, Mohammad Reza.
Afiliación
  • Shams M; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
  • Dorgalaleh A; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
  • Safarian N; Thrombosis and Hemostasis Research Center.
  • Emami AH; Hematology-Oncology Department, Imam Khomeini Hospital, Tehran University of Medical Sciences.
  • Zaker F; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
  • Tabibian S; Cellular and Molecular Research Center.
  • Managhchi MR; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
  • Faranoush M; Thrombosis and Hemostasis Research Center.
  • Tabatabaei T; Pediatric Growth and Development Research Center, Institute of Endocrinology and Metabolism, Iran University of Medical Sciences, Tehran, Iran.
  • Satari S; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
  • Rezvani MR; Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences.
Blood Coagul Fibrinolysis ; 30(1): 24-28, 2019 Jan.
Article en En | MEDLINE | ID: mdl-30585836
: Congenital factor VII (FVII) deficiency is a rare bleeding disorder with an estimated prevalence of 1 per 500 000 in the general population. On-demand replacement therapy is the main therapeutic choice in patients with congenital FVII deficiency. Inhibitor formation against exogenous FVII is very rare and can cause challenges in the management of the disorder. The present study was conducted to assess the prevalence of FVII inhibitor in 50 patients with congenital FVII deficiency under on-demand or prophylaxis treatment by recombinant activated FVII. All patients with confirmed congenital FVII deficiency were assessed for inhibitor development in regular intervals. Inhibitor titer was determined by a modified Nijmegen-Bethesda assay. The study results were analyzed by SPSS software. Among all cases, two patients (4%) developed an FVII inhibitor. Case 1 was a 14-year-old boy with severe FVII deficiency (FVII activity <1%) with regular prophylaxis. The patient was a high-responder with high-titer FVII inhibitor (170 Bethesda Unit). This patient, who had a history of intracranial hemorrhage, had undergone brain surgery three times. The second patient was a 70-years old man with on-demand therapy that also developed a high-titer inhibitor (10 Bethesda Unit). This patient had experienced easy bruising and endured a few surgeries for his brain tumor and, finally, succumbed to the disease. Although the inhibitor formation is a rare phenomenon, it may result in a significant challenge to manage the affected patients.
Asunto(s)
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Factor VII / Deficiencia del Factor VII / Formación de Anticuerpos Tipo de estudio: Etiology_studies / Risk_factors_studies Límite: Adolescent / Aged / Humans / Male País/Región como asunto: Asia Idioma: En Revista: Blood Coagul Fibrinolysis Asunto de la revista: ANGIOLOGIA / HEMATOLOGIA Año: 2019 Tipo del documento: Article Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Factor VII / Deficiencia del Factor VII / Formación de Anticuerpos Tipo de estudio: Etiology_studies / Risk_factors_studies Límite: Adolescent / Aged / Humans / Male País/Región como asunto: Asia Idioma: En Revista: Blood Coagul Fibrinolysis Asunto de la revista: ANGIOLOGIA / HEMATOLOGIA Año: 2019 Tipo del documento: Article Pais de publicación: Reino Unido