출혈성 질환의 진단적 접근

• Superficial: 피부, 점막, 코 등
• 외상 후 즉시

• Deep: 관절, 후복막, 근육 등
• 지연성 출혈

• 점상 출혈(petechia)
• 반상출혈(ecchymosis)

• 혈종(hematoma)
• 관절강 내 출혈(hemoarthrosis)

• 다량의 지속적 출혈

• 소량의 출혈

• 국소압박에 의해 쉽게 지혈

• 지속적 전신치료 필요

• 드묾

• 흔함(AR 혹은 XR 유전)

혈소판의 수와 기능

혈소판 수 이상

• Thrombocytopenia (ITP)

혈소판 기능 이상

• 선천적:vWD, afibrinogenemia, Bernard Soulier Syndrome, Glanzmann’s disease

• 후천적:Uremia, paraproteinemia, platelet inhibitory drug

Extrinsic & common pathway (II, VII, IX, X)

• Liver disease
• Vitamin K deficiency (factor II, VII, IX, X)
• Warfarin therapy
• Factor VII deficiency
• DIC

Intrinsic pathway (VIII, IX, XI, XII)

• Factor VIII (Hemophilia A, vWD)
• Factor IX deficiency (Hemophilia B)
• Congenital factor deficiencies
• Heparin therapy
• Lupus
• DIC (PT, aPTT 모두 증가)

Common pathway

• Fibrinogen deficiency (DIC, liver disease등)

• Factor VII deficiency

• Mild vitamin K deficiency
• Liver disease
• Warfarin administration
• Acquired inhibitor of factor VII
• Lupus anticoagulant(more commonly causes isolated prolonged aPTT; may be associated with thrombosis rather than bleeding)

• Deficiency of factors VIII, IX, or XI
• Deficiency of factor XII, prekallikrein, or HMW kininogen(not associated with a bleeding diathesis)
• von Willebrand disease (variable)

• Heparin administration
• Inhibitor of factors VIII, IX, XI, or XII
• Acquired von Willebrand disease
• Lupus anticoagulant(may be associated with thrombosis rather than bleeding)

• Dificiency of prothrombin, fibrinogen, or factors V or X
• Combined factor deficiency

• Liver disease
• Disseminated intravascular coagulation
• Supratherapeutic doses of anticoagulants
• Severe vitamin K deficiency
• Combined heparin and warfarin administration
• Direct thrombin inhibitor administration ( argatroban, dabigatran)
• Direct factorXa inhibitor administration ( rivaroxaban, apixaban, edoxaban)
• Fondaparinux administration (slight prolongation)
• Inhibitor or prothrombin, fibrinogen, or factors V or X
• Primary amyloidosis-associated factor X deficiency
• Anticoagulant rodenticide poisoning