4/29/2018 Protein C/S Deficiency - Hematology - Medbullets Step 1

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

Thrombocytopenic Purpura
(TTP)  


Hemolytic Uremic
Protein  0  0  0  0  1
 Hematology C/S

Syndrome (HUS)

MIXED
PLATELET/COAGULATION

Deficiency
DISORDERS Lucy
Liu
Von Willebrand Disease

Disseminated Intravascular Snapshot

Coagulation (DIC)

Heparin-Induced A 40-year-old woman is bridged

Thrombocytopenia (HIT) from heparin to warfarin for a
recent provoked DVT. Soon
HYPERCOAGULABLE
after receiving warfarin, she


STATES
develops dark lesions on her
Factor V Leiden arms. They are large, dark, and
Prothrombin Gene necrotic. Warfarin is stopped
Mutation and vitamin K and heparin are
Antithrombin III Deficiency administered. Suspicious, her physician orders a specific
assay that measures the activity of protein C.
Protein C/S Deficiency

CANCER (SEE  Overview

ONCOLOGY)

HEME DISORDERS


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

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)
 Hematology Pathogenesis
review of anticoagulation pathway
protein C (with cofactor protein S)
inactivates factors V and VIII
remember, both are vitamin K-
dependent
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
administration of warfarin
remember, 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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4/29/2018 Protein C/S Deficiency - Hematology - Medbullets Step 1

protein C concentrate
anticoagulate with heparin
For warfarin-induced necrosis
stop warfarin
administer IV vitamin K
  administer heparin
 Hematology
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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