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Deficit del factor XIII

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Deficit del factor XIII

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  1. 2 3 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text Deficit del factor XIII 4 5 6 7 8 Dr Andrés Borda

  2. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Generalidades. Michel M et al. Br J Haematol. 2008;123(1):142-146.

  3. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text • Deficienciacongenita del Factor XIII-A. • Autosomicarecesiva. • Rara. ( 1 a 2 o 3 millones de personas) • Todaslasrazas y genero. • Más de 500 casosdescritos. 3 4 6 5 7 8 Generalidades. SeminThrombHemost 2009;35(4):426–438

  4. 2 1 • Deficit congénitodel Factor XIII-B. • Pocoscasosdescritos. • Subunidad residual del FXIII-A ( < 10% VN) • Sintomashemorragicos más levesque en déficit del FXIII-A. • Ocasionalmentemanifestacioneshemorragicasseveras. • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Generalidades. SeminThrombHemost 2009;35(4):426–438

  5. A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 Generalidades.

  6. Generalidades.

  7. Generalidades.

  8. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 J ThrombHaemost2013;11(2):234–244) panel Estructura y función Factor XIII. Listado

  9. 2 1 • Clinica – según severidad del deficit. • Neonatal: • Sangrado del cordon umbilical. • Hematomas subcutaneos, intramuscular y articular, • Hemorragía intracraneal. • Cicatrización alterada.* • Abortos.* • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Manifestaciones clinicas. SeminThrombHemost 2013;39:632–641. panel Listado

  10. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Genética del deficit FXIII. Int J Hematol 2012;96(6):781–785 Panel Listado

  11. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Genética del deficit FXIII. Int J Hematol 2012;96(6):781–785 Panel Listado

  12. 2 1 • Síntomas clínicos sugestivos. • Pruebas cuantitativas / cualitativas. • Interpretación. • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Diagnostico de laboratorio Thromb Res 2007;119(3):311–318 Panel Listado

  13. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Diagnostico de laboratorio Thromb Res 2007;119(3):311–318 Panel Listado

  14. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Diagnostico de laboratorio Thromb Res 2007;119(3):311–318 Panel Listado

  15. 2 1 • Activación de la coagulación. • Consumo del FXIII. • Alteración de la sintesis del FXIII. • Auto anticuerpos contra FXIII. • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Deficit del factor XIII adquirido. (etiología) Panel principal Listado principal

  16. 2 1 • Reducciones del FXIII • Cirugia mayor. • Embolismo pulmonar. • Ictus. • Enfermedad de Crohn. • Colitis ulcerative. • Sepsis. • Medicamentos ( tocilizumab) • Recuentos entre 20% a 50%. • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Consumo del Factor XIII KohlerHP et al . Br J Haematol 2002;118 (3):825–832 KucherN, et al. Thromb Haemost 2003;90 (3):434–438 Panel principal Listado principal.

  17. 2 1 • Auto anticuerpos contra el FXIIIA. • Inhiben la activación del FXIIIA. • Actividad del FXIIIA. • Inhiben la unión del FXIII al fibrinógeno. • Enfermedades autoinmunes asociadas ( ej. LES) • Autoanticuerpos anti FXIIIB ( LES) • Diagnóstico: test de mezclas. • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Auto anticuerpos contra FXIII. Blood Rev2011;25(5):193–204 Panel Listado

  18. 2 1 • A content placeholder. Use for text, graphics, tables and graphs. You can change this text or delete it. • Here is a placeholder for more text. You may delete this text • Here is a placeholder for more text. You may delete this text 3 4 6 5 7 8 Algoritmo diagnostico. SeminThrombHemost 2011;37(4):382–388 panel Listado

  19. panel Tratamiento del deficit del Factor XIII Terapia de sustitución del FXIII Concentrado del FXIII derivado del plasma FVIII recombinante Monitorización de pacientes en TRP con FXIII Alerta global para sospecha del déficit del factor XIII Conclusiones

  20. Panel Terapia de sustitución del FXIII Listado 1 • Terapia de remplazo profilactico. • Plasma fresco congelado. • Concentrado del factor XIII derivado del plasma. 2 3 4 5 6 7 8 SeminThrombHemost 2011;37(4):382–388

  21. panel Concentrado del FXIII derivado del plasma. Listado 1 • Fibrogammin P o Corifact, CSL Behring, King of Prussia, PA). ( vial de 250 UI) • Profilaxis hemorragia 8 – 12 ml / 4 semanas. • Hemorragia grave 8-20 ml hasta cese de hemorragia. • Deficit adquirido: 20 ml/dia. • T1/2 larga • Buen pronóstico. • Riesgo de sangrado persiste sin tratamiento. 2 3 4 5 6 7 8 SeminThrombHemost 2013;39:632–641.

  22. Panel FVIII recombinante Listado 1 • Sub unidad FXIII-A asociada con subunidadendogena FXIII-B para formar un tetrameroestable del FXIII. • Desarrollo de anticuerpos ( no neutralizantes, bajafrecuencia ). • Tratamiento nuevo y seguro. 2 3 4 5 6 7 8 Arch Pathol Lab Med. 2014;138:278–281

  23. Panel Monitorización de pacientes en TRP con FXIII. Listado 1 • En terapia profilactica cada 4 a 6 semanas, la determinación de actividad del FXIII pre y post , no es necesaria. • Determinar nivel de actividad en: • Embarazo. ( >20% gestación y > 50% parto) • Intervenciones quirurgicas. (>50%) • Episodios de sangradoa pesar de terapia. • No guías disponibles. 2 3 4 5 6 7 8 Arch Pathol Lab Med. 2014;138:278–281

  24. Panel Alerta global para sospecha del déficit del factor XIII. Listado 1 • “ Todo paciente durante el primer episodio de sangrado , debe ser correctamente estudiado, con un indice de sospecha adecuado al deficit del factor XIII”. • “ Deficit de la coagulación más subdiagnosticado en el mundo”. 2 3 4 5 6 7 8 Arch Pathol Lab Med. 2014;138:278–281

  25. Panel Conclusiones Listado 1 • El diagnóstico actualmente no es difícil, pero require conocimiento de la función y limitaciones de las pruebas. • Se require aumentar el nivel de sospechadx. • Se requieren registros internacionales. 2 3 4 5 6 7 8 ThrombHaemost. 2011;105(5):925–927.

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