Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS13 deficiency?

Soraya Krieg; Jan-Dirk Studt; Irmela Sulzer; Bernhard Lämmle; Johanna A. Kremer Hovinga

doi:10.1160/TH05-04-0279

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2005; 94(06): 1186-1189
DOI: 10.1160/TH05-04-0279

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS13 deficiency?

Authors

Soraya Krieg

¹Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland
Jan-Dirk Studt

¹Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland
Irmela Sulzer

¹Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland
Bernhard Lämmle

¹Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland
Johanna A. Kremer Hovinga

¹Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland

Abstract

Summary

About 60% of patients diagnosed with acute thrombotic thrombocytopenic purpura (TTP) display a severe ADAMTS13 deficiency. Recently, Raife et al. concluded from a small case series, that factor V Leiden (FVL) might constitute a risk factor for acute thrombotic microangiopathy (TMA) without severe ADAMTS13 deficiency. Therefore, we determined ADAMTS13 activity and FVL carrier-ship in 256 consecutive patients presenting with various forms of acute TMA, including patients diagnosed with TTP or hemolytic-uremic syndrome (HUS). The overall prevalence of FVL was 8.2% (6.25% among patients diagnosed with TTP, and 9% among those with HUS) concordant with the FVL prevalence reported in Europe. FVL was present in 9.9% of patients with ADAMTS13 activity <10% and in 9.7% of those with normal ADAMTS13 activity (>50%). We conclude that FVL is not more prevalent inTMA patients without as compared to those with severe ADAMTS13 deficiency. The prevalence of FVL carriers in certain HUS subgroups (HUS with ADAMTS13 activity >50%) reaching 12.3% suggests that a contributory role of FVL in the pathogenesis of defined forms of HUS needs further study.

Keywords

ADAMTS13 activity - factor V Leiden - HUS - thromboticmicroangiopathy - TTP

Full Text

References

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