INTRODUCTION
Thrombotic thrombocytopenic purpura (TTP) is triad of severe
thrombocytopenia (usually less than 100x109/l),
macroangiopathic hemolytic anemia and varying end organ involvement (1).
Also called Moschcowitz disease, this occurs following the increase in
prothrombotic state with the deficiency in the disintegrin and
metalloproteinase with thrombospondin motifs 13 (ADAMTS 13), the
molecule that cleaves and thereby activates the von Williebrand factor
(vWF) (2). Large, uncleaved multimers of vWF forms a substrate for the
binding of platelets within the blood vessels with subsequent mechanical
fragmentation of red blood cell, occlusion of the microcirculatory bed
and organ ischemia. The more prevalent form of this disease is acquired,
with the formation of autoantibodies against ADAMTS 13 however, compound
heterogeneous or homogeneous mutations may also occur: Upshaw-Schulman
syndrome (3, 4). TTP usually presents acutely with rapid deterioration
of the patient and has a high mortality and recurrence rate in those who
survive an acute episode. Neurologic (headache, seizures and coma),
cardiac (ST- elevation and non-ST elevation myocardial infarction, heart
failure) and renal manifestations have been documented and many patients
develop multi organ failure leading to their eventual demise within
weeks if not adequately managed (5, 6). The bedrock of treatment is the
use of therapeutic plasma exchange with fresh frozen plasma as this
reduces the autoantibodies against ADAMTS 13 and improves the levels of
functional vWF.(7)