title: Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcome of 24 patients treated at a Tertiary Care Center in Saudi Arabia.
abstract: OBJECTIVE
  Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. The primary aim was overall response rate (ORR) assessment in the treated patients.
  
  
  METHODS
  This retrospective study included 24 patients treated during 2006-2015. TTP patients with microangiopathic hemolysis (MAHA) and thrombocytopenia were included. We analyzed clinical features, laboratory characteristics and treatment outcomes of 24 TTP patients treated at our tertiary care center (KFMC).
  
  
  RESULTS
  Twenty-four TTP patients (18 females; 6 males) had a mean age of 33.5±13.9 years; 22(91%) had neurologic features, 7(29%) fever, 10(42%) renal impairment; 4(20.83%) cardiac manifestations; 22(91.7%) had triad with additional neurologic abnormalities; only 2(8.2%) had pentad of TTP. Majority (54.16%) had idiopathic TTP. All patients received therapeutic plasma exchange (TPE); 23(95.8%) received adjunctive corticosteroids and 13(54.2%) received rituximab either due to refractoriness to TPE on ~day7, or earlier. Twenty-one out of 24 (87.5%) achieved complete remission (CR) without any subsequent relapse. At 22 months (median, range 1-113), 20 patients (83.3%) are alive at the time of report. Three patients died during acute episode because of sever disease or delayed treatment and one died in CR.
  
  
  CONCLUSIONS
  TPE, steroids and or rituximab was very effective in preventing high risk of mortality and achieving durable CR in 87.5% of patients. More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities..
authors.affiliation: Shahid Iqbal MBBS, FCPS, MRCP (UK), Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia.
authors.forename: Shahid
authors.initials: S
authors.lastname: Iqbal
authors.affiliation: Syed Z. A. Zaidi, MBBS, FRCP, FCPS, FRCPath, FACP, Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia, Faculty of Medicine, King Saud Bin AbdulAziz University of Health Sciences, Riyadh, Saudi Arabia.
authors.forename: Syed Z A
authors.initials: SZ
authors.lastname: Zaidi
authors.affiliation: Ibraheem H Motabi, MRCP, MD, Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia, Faculty of Medicine, King Saud Bin AbdulAziz University of Health Sciences, Riyadh, Saudi Arabia.
authors.forename: Ibraheem H
authors.initials: IH
authors.lastname: Motabi
authors.affiliation: Nawal Faiez Alshehry, MD, SBIM, SBAH, Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia.
authors.forename: Nawal Faiez
authors.initials: NF
authors.lastname: Alshehry
authors.affiliation: Mubarak S. AlGhamdi, MD, Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia.
authors.forename: Mubarak S
authors.initials: MS
authors.lastname: AlGhamdi
authors.affiliation: Imran Khan Tailor, MBBS, MD, MRCP, FRCPath, Department of Adult Hematology / BMT, CCC, King Fahad Medical City, Riyadh, Saudi Arabia.
authors.forename: Imran Khan
authors.initials: IK
authors.lastname: Tailor
keywords: Rituximab; Thrombotic thrombocytopenic purpura (TTP); therapeutic plasma exchange (TPE)