title: Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment.
abstract: The remission rate with plasma exchange (PE) in thrombotic thrombocytopenic purpura (TTP) exceeds 80%, but the disease relapses in up to 20-30% of the cases. Clinical characteristics and response to treatment of relapsed TTP are not well defined. The objective of the present study was to compare the clinical and biological characteristics at presentation and the response to treatment between de novo and relapsed TTP. For such purpose, a total of 102 episodes of idiopathic TTP (70 de novo and 32 relapses) included in a recent multicentric prospective cohort study were analysed. All patients were homogeneously treated with daily PE and costicosteroids. In comparison with de novo TTP, episodes of relapsed TTP showed a higher Hb level (median, 122 g/l versus 91 g/l, p < 0.001) and lower serum lactate dehydrogenase (2.2- versus 4.5-fold above the upper limit of normality, p < 0.001). Neurological symptoms and fever were less frequently observed in patients with relapsed TTP than in patients with de novo TTP. Patients with relapsed TTP needed fewer PE sessions (five versus ten, p = 0.02) and a smaller volume of plasma (221 ml/kg versus 468 ml/kg, p = 0.004) to achieve remission than those with de novo TTP. There was no significant difference in the rate of recrudescence under treatment, the need of complementary treatments or the frequency of refractoriness to PE therapy. In conclusion, relapsed TTP has a milder clinical profile and responds more easily to PE than de novo TTP.
authors.affiliation: Hospital del Mar, Barcelona, Spain.
authors.forename: Alberto
authors.initials: A
authors.lastname: Alvarez-Larrán
authors.forename: Julio
authors.initials: J
authors.lastname: del Río-Garma
authors.forename: Misericòrdia
authors.initials: M
authors.lastname: Pujol
authors.forename: Javier
authors.initials: J
authors.lastname: de la Rubia
authors.forename: Manuel
authors.initials: M
authors.lastname: Hernández-Jodra
authors.forename: Montserrat
authors.initials: M
authors.lastname: Borrell
authors.forename: José R
authors.initials: JR
authors.lastname: González-Porras
authors.forename: José M
authors.initials: JM
authors.lastname: García-Gala
authors.forename: Aurora
authors.initials: A
authors.lastname: Viejo
authors.forename: Lourdes
authors.initials: L
authors.lastname: Enríquez
authors.forename: Cristina
authors.initials: C
authors.lastname: Arbona
authors.forename: José A
authors.initials: JA
authors.lastname: García-Erce
authors.forename: Ana G
authors.initials: AG
authors.lastname: Noblejas
authors.forename: Arturo
authors.initials: A
authors.lastname: Pereira
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D005334:Fever / Q000209:etiology; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology; D010951:Plasma Exchange; D011446:Prospective Studies; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000473:pathology / Q000628:therapy*; D012008:Recurrence; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D006454:Hemoglobins; D007770:L-Lactate Dehydrogenase