[Idiopathic thrombotic thrombocytopenic purpura or Moschowitz syndrome: current physiopathologic and therapeutic perspectives].
BACKGROUND
The objective of this work was to review current data about the physiopathology, clinical features, and treatment of thrombotic thrombocytopenic purpura (Moschowitz's syndrome).


BACKGROUND
Thrombotic thrombocytopenic purpura is a rare disorder characterized by widespread thrombotic injuries of platelets in the microcirculation. Its physiopathology has been elucidated recently. Evidence of a deficiency of Von Willebrand's factor-cleaving protease would be due to either IgG antibodies in the acute form of the disease or constitutional deficiency in the chronic form of the disease.


CONCLUSIONS
Plasma exchange is the current reference treatment. However, in the light of recent publications, either infusions of concentrates of purified enzyme or more intensive immunosuppressive therapy would be more specific.
Retornaz
F
F
Service de médecine interne, hôpital de Sainte-Marguerite, CHRU, Marseille, France.
Durand
J M
JM
Poullin
P
P
Lefèvre
P
P
Soubeyrand
J
J
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000208:Acute Disease; D002908:Chronic Disease; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008666:Metalloendopeptidases / Q000172:deficiency; D010951:Plasma Exchange; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy*; D016896:Treatment Outcome
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human