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Which tests can help confirm SCPCD?

Which Test Can Help Desktop 2

SCPCD diagnosis can be confirmed by a combination of:

PROTEIN C (PC) ACTIVITY ASSAY1,3

Protein S assay1 (low PC levels but normal Protein S levels are suggestive of SCPCD)4

PC antigen5

Full blood count (Hemoglobin, White blood cells, Platelets)5,6

Fibrinogen2,5

D-dimer5

Prothrombin time (PT)2,5

Activated partial prothrombin time2,5

Genetic analysis2

PC level testing in parents1

PC activity assay and PC antigen

MRI (to check for cerebral venous thrombosis)1

Given possible confounding factors, PC deficiency should be confirmed using multiple methods.2

PC testing in family members is recommended to determine if the deficiency is genetic and whether it is a homozygous or heterozygous mutation.2 A partial reduction in protein C levels in both parents is very suggestive of SCPCD.1

 

PC activity levels are mostly undetectable in PC deficient-homozygote neonates, therefore the age-specific references below CAN BE USED as a guide for interpreting results.

Premature infantsa
AGE
PROTEIN C MEANS (95%CI)
Day 1
Antigen: 28% (12-44)
Day 5
Antigen: 31% (11-51)
Full-term infantsb
AGE
PROTEIN C MEANS (95%CI)
Day 1
Chromogenic: 36% (24-44)
Clotting: 32% (24-40)
Day 3
Chromogenic: 44% (28-54)
Clotting: 33% (24-51)
aData obtained using ELISA (Protein C) method

bData obtained using Stachrom Protein C and Staclot Protein C

Adapted from Price 20112

References:

  1. Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.

  2. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

  3. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  4. Libourel, EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. British Journal of Haematology. 2002;118:615–618.

  5. Khor, B, et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440–442.

  6. Tairaku S, et al. Prenatal genetic testing for familial severe congenital protein C deficiency. Hum Genome Var. 2015;2:15017.

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