[Paediatric case report of an acquired autoimmune thrombotic thrombocytopenic purpura].
Thrombotic thrombocytopenic purpura (TTP), when accompanied by regenerative anaemia with schizocytosis, thrombopenia and neurological manifestations, is a disease whose main characteristic is the absence of the von Willebrand factor (vWF) cleaving protease. The two types of TTP are distinguishable by the presence or absence of antiprotease inhibitors, which are, respectively, either acquired or constitutional. The acquired autoimmune form is most frequently observed in adults.


METHODS
An adolescent with a previous history of moderate, isolated thrombopenia first showed symptoms of TTP at the age of 14. Positive antiprotease inhibitors in combination with a degeneration of protease activity confirmed the diagnosis of acquired autoimmune TTP. A treatment consisting of daily plasma exchange led to rapid improvement; however, a failed attempt to space out plasma exchanges necessitated the introduction of 4 weekly injections of Rituximab beginning on day 40, which was successful. Indeed, since the second injection of Rituximab on day 51, the number of platelets stabilized at a normal level, thereby allowing for the complete cessation of plasma exchange. At this writing - day 89 - the patient remains in persistent remission.


CONCLUSIONS
Given the different therapeutic and prognostic implications of the 2 types of TTP in child patients, it is mandatory to end at an accurate biological diagnosis: whereas the constitutional form is effectively treated with plasma injections, the acquired form, while initially requiring plasma exchange, often necessitates the use of immunosuppressors during acute or relapse phase. The present study concerns a paediatric case of acquired TTP treated successfully with Rituximab during an acute dependant phase.
Curtillet
C
C
Service Hématologie Pédiatrique du Professeur-Michel, Hôpital Timone-Enfants Marseille, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France. catherine.curtillet@mail.ap-hm.fr
Poullin
P
P
Doré
E
E
Fossat
C
C
Lefevre
P
P
Michel
G
G
D000293:Adolescent; D000367:Age Factors; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D010951:Plasma Exchange; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy; D012074:Remission Induction; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab