Coagulation and thrombosis disorders
1. Factor VIII deficiency: hemophilia A
1) º´ÀÎ ¹× ÀÓ»ó¹ßÇö
2) Ä¡·á
Á¶±âÄ¡·á°¡ ´õ È¿°úÀûÀÌ¸ç ºñ¿ëÀÌ Àû°Ô µé°í lifesavingÇÒ¼ö ÀÖ´Ù.
aspirin or aspirin-containing drugsÀº Ç÷¼ÒÆÇ ±â´ÉÀ» ¼Õ»ó½ÃŰ°í ½ÉÇÑ ÃâÇ÷À» ¾ß±âÇÒ¼ö ÀÖ±â
¶§¹®¿¡ ÇÇÇØ¾ß ÇÑ´Ù. COX-2 inhibitors´Â Ç÷¼ÒÆÇ ±â´ÉÀ» ¼Õ»ó½ÃŰÁö ¾Ê±â ¶§¹®¿¡ »ç¿ëÇÒ ¼ö
ÀÖ´Ù.
factor VIII concentratesÀÇ ±¤¹üÀ§ÇÑ »ç¿ëÀº ¶ÇÇÑ ½ÉÇÑ ÇÕº´ÁõÀ» ¾ß±âÇϴµ¥ ÀÌ¿¡´Â viral
hepatitis,chronic liver disease, and AIDS¿Í °°Àº °ÍµéÀÌ ÀÖ´Ù. cryoprecipitate´Â original
volumeÀÇ 1/10¿¡
FFPÀÇ factor VIII acitivity 1/2À» Æ÷ÇÔÇÑ´Ù. ÀÌ´Â º´¿ø ¹× Ç÷¾×ÀºÇà¿¡¼ °£´ÜÇÏ°Ô ÁغñÇÒ ¼ö
ÀÖ´Ù.
¼ö¼úÀü ¸ðµç Ç÷¿ìº´ ȯÀÚ´Â factor VIII inhibitorÀÇ Á¸Àç¿¡ ´ëÇØ¼ screeningÇØ¾ß ÇÑ´Ù.
inhibitor°¡ ¾ø´Â Ç÷¿ìº´È¯ÀÚ´Â ¼ö¼úÁ÷Àü¿¡ factor VIII infusionÀ» ¹Þ¾Æ¾ß ÇÏ¸ç ¼ö¼úÈÄ 10-14
Àϰ£ factor VIII levelÀÌ 50%ÀÌ»óµÇ°Ô ¸ÅÀÏ monitorÇØ¾ß ÇÑ´Ù. ȯÀÚ°¡ joint replacement or
major orthopedic surgery¸¦ ½ÃÇàÇÒ¶§´Â wound healing ¹× PT¿¡ ÇÊ¿äÇÑ 3ÁÖ°£ Ä¡·á¸¦ °è¼Ó
ÇÏ¿©¾ß ÇÑ´Ù.
dental procedureÀü¿¡µµ Ä¡·á°¡ ÇÊ¿äÇÏ´Ù. ÃæÄ¡Ä¡·á¶§´Â factor VIII concentrateÀÇ single
infusion°ú EACA¸¦ ÇÏ·ç 4ȸ ¡¿ 3-4Àϰ£ Åõ¿©ÇÑ´Ù. EACA(¥å-aminocaproic acid)´Â oral
secretion¿¡ Á¸ÀçÇÏ´Â plasminogen activators¸¦ ¾ïÁ¦ÇÏ´Â °·ÂÇÑ antifibrinolytic agentÀ̸ç
oral tissueÀÇ clot formationÀ» ¾ÈÁ¤È½ÃŲ´Ù. ´ë¿ëÀ¸·Î tranexamic acid(=longer-acting
antifibrinolytic)°¡ ÀÖ´Ù.
2. Fac»ö IX deficiency - Hemophilia B
3. factor XI deficiency
4. other factor deficiencies
contact activation pathway¿¡ °ü¿©ÇÏ´Â Hageman factor(factor XII),
HMWK(high-molecular-weight kininogen), and prekallikreinÀÇ °áÇÌÀº °Ë»ç»ó ÀÌ»óÀ» ³ªÅ¸
³»Áö¸¸ ÀÓ»óÀû ÃâÇ÷Àº ¾ß±âÇÏÁö ¾Ê´Â´Ù. 100ÃÊÀÌ»ó PTTÀÇ ½ÉÇÑ ¿¬Àå¿¡µµ ºÒ±¸Çϰí ÀÌ·¯ÇÑ
ÀÎÀÚÀÇ °áÇÌȯÀÚ´Â Á¤»ó hemostasis¸¦ º¸À̰í
5. Afibrinogenemia and dysfibrinogenemia
6. Factor XIII deficiency and defective fibrin cross-linking
7. vitamin K deficiency