title: Outcome and relapse risks of thrombotic thrombocytopaenic purpura: an Egyptian experience.
abstract: BACKGROUND
  Thrombotic thrombocytopaenic purpura (TTP) is a rare life-threatening disease. Plasma exchange has significantly decreased the mortality from this disease, which still tends to recur in a substantial proportion of patients. This study describes the clinical spectrum and response to treatment and explores the risks of relapse in a cohort of patients.
  
  
  METHODS
  Patients treated for TTP at the Clinical Haematology Unit, Cairo University, Egypt, between 2000 and 2008 were identified. Complete demographic, clinical history and full clinical examination, laboratory, treatment modalities and duration, and outcome data were collected and analysed. The follow-up duration was 24 months.
  
  
  RESULTS
  30 patients; 13 men (43%) and 17 women (57%) with a median age of 42 years were treated for 46 episodes of TTP. The median duration of disease onset to diagnosis for the first episode was 7 days. Twenty-three patients (76.66%) were diagnosed as idiopathic primary and seven patients (23.33%) were secondary TTP. Four patients died during the first 24 h. Of the 26 patients, 22 (85.6%) achieved remission with an average of 7.55 plasma exchange sessions, Another nine patients had 25 relapses (mean 2.7). Splenectomy was performed in three patients (11.5%). The 24-month overall survival was 80%. The initial low platelet count and high LDH were the only two statistically significant relapse predictors.
  
  
  CONCLUSIONS
  The current results conform to the reported literature on the outcome of TTP. The very early mortality due to late referral highlights the need of education about the disease among primary healthcare providers.
authors.affiliation: The Clinical Haematology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Cairo, Egypt. dr_noha2002@yahoo.com
authors.forename: Noha M
authors.initials: NM
authors.lastname: El-Husseiny
authors.forename: Hady
authors.initials: H
authors.lastname: Goubran
authors.forename: Hala M
authors.initials: HM
authors.lastname: Fahmy
authors.forename: Nehad M
authors.initials: NM
authors.lastname: Tawfik
authors.forename: Heba
authors.initials: H
authors.lastname: Moustafa
authors.forename: Sherif N
authors.initials: SN
authors.lastname: Amin
authors.forename: Magdy
authors.initials: M
authors.lastname: El-Ekiaby
mesh_terms: D000328:Adult; D015331:Cohort Studies; D004534:Egypt; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010951:Plasma Exchange / Q000379:methods* / Q000401:mortality; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D012307:Risk Factors; D013156:Splenectomy / Q000379:methods* / Q000401:mortality; D016896:Treatment Outcome