An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura.

Abstract:

:Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. Areas covered: VWF/ADAMTS13 interaction is specific, and ADAMTS13 conformation has been elucidated recently. ADAMTS13 deficiency is congenital or acquired. Several targets are used in TTP treatment. Expert opinion: Differential diagnosis of TTP may be challenging and ADAMTS13 investigations are needed. ADAMTS13 conformation could be a promising parameter for TTP diagnosis and prognosis in the coming years. Plasma therapy remains the first-line treatment of the acute phase of TTP. Additional curative and preemptive rituximab are used in acquired TTP. Clinical trials dedicated to innovative drugs are promising.

journal_name

Expert Rev Hematol

authors

Joly BS,Coppo P,Veyradier A

doi

10.1080/17474086.2019.1611423

subject

Has Abstract

pub_date

2019-06-01 00:00:00

pages

383-395

issue

6

eissn

1747-4086

issn

1747-4094

journal_volume

12

pub_type

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