Abstract
Thrombotic microangiopathies (TMAs) are a group of rare but potentially lethal hematologic
disorders characterized by platelet-rich thrombi in the microvasculature. In evaluating
patients with TMA, it is crucial to identify those who have thrombotic thrombocytopenic
purpura (TTP), a subtype of TMA that occurs due to a severe deficiency in ADAMTS13
(a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13). Individuals with TTP require urgent therapeutic plasma exchange, which is associated
with a significant reduction in mortality; however, at most centers, the results of
ADAMTS13 activity testing are usually not available for 3 to 5 days. Given the possible
need for urgent intervention and the lack of timely test results to guide therapy,
the assessment of patients with TMA represents an ideal situation for the deployment
of diagnostic scoring systems to predict the presence of severe ADAMTS13 deficiency.
Here, we review the literature surrounding clinical prediction tools in the diagnosis
and prognostication of patients with TMA, describe the experience at our center with
evaluating TMA patients, and discuss the utility of clinical scoring systems for TMA
in the context of patient care.
Keywords
thrombotic microangiopathy - clinical scoring system - prediction tool - thrombotic
thrombocytopenic purpura - treatment recommendations
