• Thrombophilia: any disorder associated with increased risk of venous thromboembolic disease; more appropriately called "hypercoagulable state" if not genetic, although terms often used interchangeably
• Common risk factors are: antiphospholipid antibodies, chronic DIC, essential thrombocythemia, heparin induced thrombocytopenia, hyperhomocysteinemia, immobility, increasing age, malignancy, nephrotic syndrome, obesity, oral contraceptives, paroxysmal nocturnal hemoglobinuria, polycythemia vera, post-operative state, pregnancy, prior thromboembolism, systemic lupus erythematosus, trauma
• Presence of more than one risk factor further increases risk (Arch Pathol Lab Med 2002;126:295)

Initiation and propagation of blood coagulation

Activation of protein C by thrombin-thrombomodulin

Degradation of FVa and FVIIIa by APC

Blood coagulation and protein C anticoagulant system

Activation and degradation of normal FV and FV Leiden

20210G>A mutation in the prothrombin gene

Regulation of hemostasis

Balance of hemostasis

Genetic polymorphisms of various procoagulant proteins

Activated protein C (APC)

Transsulfuration pathway and metabolism of homocysteine

• 33 year old man with fatal pulmonary emboli associated with hypereosinophilia (J Clin Path 2004;57:541)
• 52 year old woman with portal vein thrombosis associated with myeloproliferative disorder (Arch Pathol Lab Med 2003;127:e385)

Thrombi in major trunks of pulmonary artery and large thrombus near left iliac vein.

Fig. 1/2: old, recanalized thrombi in portal veins; Fig. 3: recanalization of portal vein webs with intimal tags; Fig. 4: bone marrow is hypercellular with large, dysplastic, abnormally clustered megakaryocytes

• Hematology Am Soc Hematol Educ Program 2007:127-35, Blood 2008;112:19

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