Protein C/S Deficiency - Hematology - Medbullets Step 1
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4/29/2018
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Lucy Liu
Von Willebrand Disease Disseminated Intravascular Coagulation (DIC) Heparin-Induced Thrombocytopenia (HIT)
HYPERCOAGULABLE STATES Factor V Leiden Prothrombin Gene Mutation
Antithrombin III Deficiency Protein C/S Deficiency CANCER (SEE ONCOLOGY)
HEME DISORDERS
Snapshot A 40-year-old woman is bridged from heparin to warfarin for a recent provoked DVT. Soon after receiving warfarin, she develops dark lesions on her arms. They are large, dark, and necrotic. Warfarin is stopped and vitamin K and heparin are istered. Suspicious, her physician orders a specific assay that measures the activity of protein C. Overview
Heme Synthesis, Porphyrias, and Lead Poisoning Methemoglobinemia Acute Intermittent Porphyria
PHARMACOLOGY
RBC/WBC Transfusion
Filgrastim / Sargramostim Erythropoietin
COAGULATION Anticoagulants Thrombolytics Antiplatelet Drugs
Introduction
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4/29/2018
Protein C/S Deficiency - Hematology - Medbullets Step 1
Hematology
Hypercoagulable state/thrombophilia caused by deficiency in protein C or S Genetics protein C deficiency - autosomal recessive protein S deficiency - autosomal dominant (much less common) Pathogenesis review of anticoagulation pathway protein C (with cofactor protein S) inactivates factors V and VIII , both are vitamin Kdependent both are synthesized in the liver deficiency of either protein C or S → active factors V and VIII thrombosis Mnemonics skin or subcutaneous necrosis after istration of warfarin , warfarin affects protein C and S first protein C deficiency is more common than protein S deficiency “protein C Cancels Coagulation” Presentation Symptoms of thrombosis after warfarin initiation skin and subcutaneous necrosis DVT or PE at a young age increased risk of DIC Evaluation Protein C or S functional assay (preferred) ↓ protein C or S activity levels Genetic testing not routinely done Differential Diagnosis Factor V Leiden mutation Antithrombin deficiency Antiphospholipid syndrome HIT Treatment For thrombosis
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Protein C/S Deficiency - Hematology - Medbullets Step 1
Hematology
protein C concentrate anticoagulate with heparin For warfarin-induced necrosis stop warfarin ister IV vitamin K ister heparin
Prognosis, Prevention, and Complications Prognosis risk of thrombosis risk of warfarin-induced skin necrosis Prevention avoid warfarin avoid conditions that increase risk of thrombosis Complications miscarriage
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Protein C/S Deficiency - Hematology - Medbullets Step 1
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