Double relapse of incomplete form of thrombotic thrombocytopenic purpura.
BACKGROUND
Although several etiologies can be identified in thrombotic thrompocytopenic purpura (TTP), idiopathic cases are still frequent. Incomplete forms are rare and the diagnosis should be suspected in case of thrombocytopenia and microangiopathic haemolytic anaemia. Relapses are frequent in the complete classic form but rarely reported in incomplete forms. According to the literature, mortality and morbidity are significantly improved with plasma exchange (PE). Nevertheless, the management and treatment of relapses remain problematic.


METHODS
A 35-year-old woman presented with a double relapse of an incomplete TTP form in the second (24 months) and the fourth year (40 months) after the initial episode. The patient underwent plasma infusion, PE, and was then started on corticosteroids. She also received antiplatelet agents. The main biological indicators were platelet count, haematocrit, lactate dehydrogenase level and schistocytes. Following this treatment, the patient's condition rapidly improved.


CONCLUSIONS
In this incomplete form of TTP, two relapses occurred, with the same presentation. Standard therapy was effective in this case.
Chamouni
P
P
Unité d'Hémovigilance, Département d'Epidémiologie et de Santé Publique, CHU de Rouen, 1, rue de Germont, 76031 Cedex, Rouen, France. pierre.chamouni@chu-rouen.fr <pierre.chamouni@chu-rouen.fr>
Lenain
P
P
Girault
C
C
Tamion
F
F
Buchonnet
G
G
Czernichow
P
P
D000328:Adult; D019468:Disease Management; D005260:Female; D006801:Humans; D010951:Plasma Exchange; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D012008:Recurrence; D013156:Splenectomy; D013256:Steroids / Q000627:therapeutic use
D013256:Steroids