An acquired, calcium-dependent, factor X inhibitor

George J. Broze

doi:10.1016/j.bcmd.2013.09.001

An acquired, calcium-dependent, factor X inhibitor

George J. Broze

Division of Hematology

Research output: Contribution to journal › Article › peer-review

18 Scopus citations

Abstract

Acquired factor X (FX) deficiency unrelated to amyloidosis is a rare disorder in which an anti-FX antibody is infrequently detected. A patient with severe bleeding due to a calcium ion-dependent anti-FX IgG antibody is described. The FX affinity purified IgG bound the light chain of FX, but not FX lacking its γ-carboxyglutamic acid domain, and binding was enhanced > 1000-fold in the presence of calcium ions. The antibody also recognized prothrombin and factor VII with about 100-fold and 1000-fold lower affinity. Like a lupus anticoagulant, increasing concentrations of phospholipids in functional assays reduced the inhibitory activity of the antibody. The effect of these properties of the inhibitor on laboratory diagnostic studies is considered.

Original language	English
Pages (from-to)	116-120
Number of pages	5
Journal	Blood Cells, Molecules, and Diseases
Volume	52
Issue number	2-3
DOIs	https://doi.org/10.1016/j.bcmd.2013.09.001
State	Published - Feb 2014

Keywords

Acquired
Calcium-dependent
Factor X
Inhibitor

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10.1016/j.bcmd.2013.09.001

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title = "An acquired, calcium-dependent, factor X inhibitor",

abstract = "Acquired factor X (FX) deficiency unrelated to amyloidosis is a rare disorder in which an anti-FX antibody is infrequently detected. A patient with severe bleeding due to a calcium ion-dependent anti-FX IgG antibody is described. The FX affinity purified IgG bound the light chain of FX, but not FX lacking its γ-carboxyglutamic acid domain, and binding was enhanced > 1000-fold in the presence of calcium ions. The antibody also recognized prothrombin and factor VII with about 100-fold and 1000-fold lower affinity. Like a lupus anticoagulant, increasing concentrations of phospholipids in functional assays reduced the inhibitory activity of the antibody. The effect of these properties of the inhibitor on laboratory diagnostic studies is considered.",

keywords = "Acquired, Calcium-dependent, Factor X, Inhibitor",

author = "Broze, \{George J.\}",

note = "Funding Information: This work was supported by NIH grant R37HL077193 .",

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AU - Broze, George J.

N1 - Funding Information: This work was supported by NIH grant R37HL077193 .

PY - 2014/2

Y1 - 2014/2

N2 - Acquired factor X (FX) deficiency unrelated to amyloidosis is a rare disorder in which an anti-FX antibody is infrequently detected. A patient with severe bleeding due to a calcium ion-dependent anti-FX IgG antibody is described. The FX affinity purified IgG bound the light chain of FX, but not FX lacking its γ-carboxyglutamic acid domain, and binding was enhanced > 1000-fold in the presence of calcium ions. The antibody also recognized prothrombin and factor VII with about 100-fold and 1000-fold lower affinity. Like a lupus anticoagulant, increasing concentrations of phospholipids in functional assays reduced the inhibitory activity of the antibody. The effect of these properties of the inhibitor on laboratory diagnostic studies is considered.

AB - Acquired factor X (FX) deficiency unrelated to amyloidosis is a rare disorder in which an anti-FX antibody is infrequently detected. A patient with severe bleeding due to a calcium ion-dependent anti-FX IgG antibody is described. The FX affinity purified IgG bound the light chain of FX, but not FX lacking its γ-carboxyglutamic acid domain, and binding was enhanced > 1000-fold in the presence of calcium ions. The antibody also recognized prothrombin and factor VII with about 100-fold and 1000-fold lower affinity. Like a lupus anticoagulant, increasing concentrations of phospholipids in functional assays reduced the inhibitory activity of the antibody. The effect of these properties of the inhibitor on laboratory diagnostic studies is considered.

KW - Acquired

KW - Calcium-dependent

KW - Factor X

KW - Inhibitor

UR - https://www.scopus.com/pages/publications/84891901760

U2 - 10.1016/j.bcmd.2013.09.001

DO - 10.1016/j.bcmd.2013.09.001

M3 - Article

C2 - 24074948

AN - SCOPUS:84891901760

SN - 1079-9796

VL - 52

SP - 116

EP - 120

JO - Blood Cells, Molecules, and Diseases

JF - Blood Cells, Molecules, and Diseases

IS - 2-3

ER -

An acquired, calcium-dependent, factor X inhibitor

Abstract

Keywords

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