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Table 2 Characteristics of congenital and acquired Protein C deficits

From: Protein C in adult patients with sepsis: from pathophysiology to monitoring and supplementation

Type of deficiency

Mechanisms of deficiency

Antigen

Chromogenic activity

Coagulometric activity

Congenital deficiencies

 I

Quantitative or true deficiency

(75% of patients)

 II

Qualitative or dysfunctional

(IIa 23,75% and IIb 1.25% of patients)

N

↓ (IIa)

N (IIb)

 II

Quantitative and qualitative

↓↓

↓↓

Acquired deficiencies

Liver diseases

Vitamin K deficiency

AVK treatment

DIC

Sepsis

Chemotherapy with L-asparaginase

Nephrotic syndrome

Solid malignancies

Reduced synthesis/increased clearance

Antibodies against PC

Mainly inhibitors

N

Factors potentially interfering with results

 Pregnancy

 

(early)

 DOAC

 

-

False ↑

 Clotted/activated samples

 

False ↑

False ↓

 Lupus anticoagulant (aPTT-based, not dRVVt)

 

False ↑

 Factor V Leiden/Factor VIII > 200%

 

False ↓

(with aPTT-based assays)

 Increased FVIII activity > 200%

  

False ↓

(with aPTT-based assays)

  1. Modified from: Dinarvand P, Moser KA. Protein C deficiency. Arch Pathol Lab Med 2019; 143:1281- 1825; Cooper PC, Pavlova A, Moore GW, Hickey KP, Marlar RA. Recommendations for clinical laboratory testing for protein C deficiency, for the subcommittee on plasma coagulation inhibitors of the ISTH. J Thromb Haemost. 2020;18(2):271–277
  2. AVK anti-vitamin K, DIC disseminated intravascular coagulation, PC protein C, aPTT activated partial thromboplastin time