Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report.
BACKGROUND
Thrombotic microangiopathies constitute a heterogeneous group of diseases characterised by microangiopathic haemolytic anaemia and thrombocytopaenia associated with platelet aggregation in the microcirculation responsible for ischaemic manifestations. Classically, thrombotic microangiopathies are described as encompassing two main syndromes: thrombotic thrombocytopaenic purpura and the haemolytic-uraemic syndrome Many cases of idiopathic thrombotic thrombocytopaenic purpura have, to date, been associated with severe ADAMTS13 metalloprotease deficiency while haemolytic uraemic syndrome usually occurs in the context of normal protease activity. Oestrogens and factor V Leiden have rarely been implicated in the pathogenesis of thrombotic microangiopathy.


METHODS
We describe the case of a 17-year-old female with refractory thrombotic thrombocytopaenic purpura. The patient was receiving a new generation of oral contraceptives for dysmenorrhoea and had factor V Leiden. After undergoing prolonged and intense plasma exchange therapy for 40 days and high dose oral corticosteroids therapy for 90 days, our patient recovered fully.


CONCLUSIONS
Patients with refractory thrombotic thrombocytopaenic purpura should likely be evaluated for congenital thrombophilic disorders and for ingestion of drugs that have been associated with this rare form of thrombotic microangiopathy. Identification of these and as yet other unknown genetic and/or acquired risk factors may lead to more judicious treatment approaches.
Stylianou
Kostas
K
Department of Nephrology, University Hospital of Heraklion, Heraklion, Crete, Greece. kstylianu@gmail.com
Tsirakis
George
G
Mantadakis
Elpis
E
Xylouri
Irini
I
Foudoulakis
Andreas
A
Vardaki
Eleftheria
E
Katsipi
Irene
I
Daphnis
Eugene
E
Samonis
George
G