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Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura

S L Allford¹, S J Machin¹

¹ Haemostasis Research Unit, University College Hospital, 98 Chenies Mews, London WC1E 6HX, UK

Correspondence to:
Dr Allford email: mach263@msn.com

Accepted December 14, 1999

Introduction

Thrombotic thrombocytopenic purpura (TTP) was first described by Moschowitz in 1924.¹ Although it is now well recognised, being characterised by the classic pentad of fever, thrombocytopenia, microangiopathic haemolytic anaemia (MAHA), renal impairment, and fluctuating neurological signs, its precise pathophysiology remains elusive. Several other syndromes including haemolytic uraemic syndrome (HUS), eclampsia and HELLP (haemolysis, elevated liver enzymes, and low platelets) are also characterised by MAHA and this has given rise to the concept that they might represent a spectrum of disease. However, recent independent work by Tsai and Furlan indicates that TTP and HUS appear to be distinct pathophysiological entities, which might explain in part the wide variation in response to treatment that is seen in clinical practice.

Clinical variants

Several variants of TTP are recognised. Most cases are defined as single episode TTP: in these patients there is no identifiable precipitant and no subsequent recurrence. Because survival has improved with advances in . . . [Full text of this article]

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