title: Retrospective review of 25 cases of thrombotic thrombocytopenic purpura in Pakistan.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. Because of the rarity of TTP, no comprehensive data is available in the Pakistani population. The present study aimed to review the therapeutic interventions, relapses and mortality rate in patients with TTP treated at a tertiary care hospital in Pakistan. This was a retrospective review of patients treated over a period of more than nine years (2001-2010). Medical charts were retrieved using the ICD coding system version 9 and each file was reviewed by the principal author for clinical and laboratory details, along with the therapy utilized and the outcome. Twenty-five patients were diagnosed with TTP, including nine males (36%) and 16 females (64%) with a median age of 30 ± 18.4 years for all patients. Idiopathic TTP was seen in 17 patients (68%) and secondary causes were identified in eight (32%). Patients were treated with plasma exchange once the diagnosis of TTP was established. Only neurological and renal involvement at the time of presentation emerged as important indicators in determining the outcome and response to treatment. Most of our patients tolerated plasmapheresis very well; however, delay in starting plasmapheresis due to late presentation was a major hurdle in our set up.
authors.affiliation: Department of Medicine and Pathology, Aga Khan University Hospital, Karachi, Pakistan. drsafoorah@hotmail.com
authors.forename: Safoorah
authors.initials: S
authors.lastname: Sagheer
authors.forename: Bushra
authors.initials: B
authors.lastname: Moiz
authors.forename: Mohammad
authors.initials: M
authors.lastname: Usman
authors.forename: Mohammad
authors.initials: M
authors.lastname: Khurshid
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000628:therapy*; D055815:Young Adult