title: More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura.
abstract: BACKGROUND
  Thrombotic thrombocytopenic purpura (TTP) is characterized by a reduction in the von Willebrand cleavage protein ADAMTS-13, mainly as a consequence of autoimmunity. Plasma exchange (PEx) is standard, achieving complete remission (CR) in 77-83% of cases, but rates are variable depending on ADAMTS-13 activity and relapse is frequent in patients with <10%. Thus, an effective front-line immunosuppressive treatment is needed.
  
  
  METHODS
  We administered PEx daily until CR and rituximab 100 mg/dose/week for 4 consecutive weeks to 10 patients with a first TTP episode and 1 relapsed patient (8 females (72%) and 3 males (28%)). Median age was 34 years (15-46) and laboratory parameters at diagnosis were as follows: platelets 11 × 10(9)/l (range 7-27.4 × 10(9)/l), lactate dehydrogenase 1822 U/l (range 705-8220 U/l, normal 70-180 U/l), and haemoglobin 6 g/dl (range 4.2-11.8 g/dl). ADAMTS-13 activity was determined in eight patients and was <10% in all. ADAMTS-13 autoantibody titre was determined in seven patients and was >15 units/ml in all (ref: negative <12, undetermined 12-15, positive >15 units/ml); Shiga toxin was negative in all patients. The median number of PEx until CR was 7 (range 4-12); prednisone 1 mg/kg was administered to six patients.
  
  
  RESULTS
  The median follow-up was 22 months (range 4-49) and the estimated 2-year relapse-free survival was 89%; one HIV+ patient relapsed at 8 months follow-up. No complications related to PEx or rituximab were reported.
  
  
  CONCLUSIONS
  Our study suggests that low-dose rituximab and PEx are effective as front-line treatment for acute TTP; however, a prospective trial is needed to demonstrate whether low-dose rituximab is as effective as the conventional dose.
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Alberto
authors.initials: A
authors.lastname: Vazquez-Mellado
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Myrna
authors.initials: M
authors.lastname: Pequeño-Luévano
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Olga Graciela
authors.initials: OG
authors.lastname: Cantu-Rodriguez
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Laura
authors.initials: L
authors.lastname: Villarreal-Martínez
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: José Carlos
authors.initials: JC
authors.lastname: Jaime-Pérez
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Andres
authors.initials: A
authors.lastname: Gomez-De-Leon
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Fernando
authors.initials: F
authors.lastname: De La Garza-Salazar
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Oscar
authors.initials: O
authors.lastname: Gonzalez-Llano
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Perla
authors.initials: P
authors.lastname: Colunga-Pedraza
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: Guillermo
authors.initials: G
authors.lastname: Sotomayor-Duque
authors.affiliation: a Haematology Department, Hospital Universitario Jose Eleuterio Gonzalez , Autonomous University of Nuevo Leon , Monterrey , Mexico.
authors.forename: David
authors.initials: D
authors.lastname: Gomez-Almaguer
mesh_terms: D000293:Adolescent; D000328:Adult; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000401:mortality* / Q000628:therapy*; D000069283:Rituximab / Q000008:administration & dosage*; D015996:Survival Rate
chemical_list: D000069283:Rituximab
keywords: ADAMTS-13; Frontline therapy; Immunotherapy; Low-dose rituximab; Plasma exchange; Rituximab; TTP; Thrombotic thrombocytopenic purpura