https://www.researchgate.net/journal/Rambam-Maimonides-Medical-Journal-2076-9172
Thrombotic
microangiopathies (TMAs) comprise a group of distinct disorders
characterized by microangiopathic hemolytic anemia, thrombocytopenia,
and microvascular thrombosis. For many years distinction between these
TMAs, especially between thrombotic thrombocytopenic purpura (TTP) and
hemolytic uremic syndrome (HUS), remained purely clinical and hard to
make. Recent discoveries shed light on different pathogenesis of TTP and
HUS. Ultra-large von Willebrand factor (UL-VWF) platelet thrombi,
resulting from the deficiency of cleavage protease which is now known as
ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin
type 1 motif, member 13), were found to cause TTP pathology, while Shiga
toxins or abnormalities in regulation of the complement system cause
microangiopathy and thrombosis in HUS. TMAs may appear in various
conditions such as pregnancy, inflammation, malignancy, or exposure to
drugs. These conditions might cause acquired TTP, HUS, or other TMAs, or
might be a trigger in individuals with genetic predisposition to
ADAMTS-13 or complement factor H deficiency. Differentiation between
these TMAs is highly important for urgent initiation of appropriate
therapy. Measurement of ADAMTS-13 activity and anti-ADAMTS-13 antibody
levels may advance this differentiation resulting in accurate diagnosis.
Additionally, assessment of ADAMTS-13 levels can be a tool for
monitoring treatment efficacy and relapse risk, allowing consideration
of therapy addition or change. In the past few years, great improvements
in ADAMTS-13 assays have been made, and tests with increased
sensitivity, specificity, reproducibility, and shorter turnaround time
are now available. These new assays enable ADAMTS-13 measurement in
routine clinical diagnostic laboratories, which may ultimately result in
improvement of TMA management.
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