Laboratory Monitoring of Factor VIII and Factor IX in Hemophilia Patients

Michael A. Nardi

doi:10.29074/ascls.2018001305

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Figure 1.
Schematics of FVIII and FIX activity assays. (A) One-stage clotting assay. (B) Chromogenic substrate assay. CaCl₂, calcium chloride; CSA, chromogenic substrate assay; FII, prothrombin (factor II); FIX, factor IX; FIXa, activated factor IX; FVIII, factor VIII; FVIIIa, activated factor VIII; FX, factor X; FXa, activated factor X; FXIa, activated factor XI; PL, phospholipid; pNA, para-nitroanaline.

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Table 1.

List of available chromogenic substrate assay kits

Factor VIII Kits
Name	Manufacturer	FDA Status	Detection Limit^c,e
Biophen FVIII:C^a	Hyphen-Biomed	None (RUO)	2%
Coamatic Factor VIII^a	Chromogenix	Approved	0.5%
Coatest SP FVIII^b	Chromogenix	Approved	1%
Coatest SP4 FVIII^b	Chromogenix	Approved	1%
FVIII Chromogenic Assay^b	Siemens Healthcare	Approved	NR
Hemosil Electrachrome FVIII^a	Insturmentation Laboratory	None (RUO)	0.5%
Technoclone FVIII:C^ab	Technoclone	None (RUO)	0%
Factor IX Kits
Name	Manufacturer	FDA Status	Detection Limit
Biophen Factor IX^a	Hyphen-Biomed	None (RUO)	0.5%
ROX Factor IX^a	Rossix	None (RUO)	0.5%

FDA, U.S. Food and Drug Administration; N.R., not reported; RUO, research use only; ^a High range and low range calibration curves suggested; ^b Single calibration curve;^c Value obtained from reagent package insert; ^d Value for low range calibration curve listed.

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Table 2.

List of FDA-approved EHL products

Variant Name	Manufacturer	Year Approved	Half-life
EHL-FVIII Products
Adynovate (amino-group PEGylation of full-length FVIII)	Shire	2015	14.7 hours
Eloctate (Fc-fusion with BDD FVIII)	Bioverativ	2014	19.0 hours
EHL-FIX Products
Alprolix (Fc-FIX fusion)	Bioverativ	2014	82.1 hours
Idelvion (Albumin-FIX fusion)	CSL Behring	2015	90.0 hours
N9-GP, Rebinyn, Refixia, (GlycoPEGylated-FIX)	Novo-Nordisk	2017	92.7 hours

EHL, extended half-life; FDA, U.S. Food and Drug Administration; FIX, factor IX; FVIII, factor VIII; N9-GP, glycopegylated rFIX; PEG, polyethylene glycol.