title: Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura: a retrospective multicenter study.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP. Also, TTP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in TTP patients.
  
  
  OBJECTIVE
  We aimed to present our experience in 163 patients with TTP treated with TPE during the past 5years from 10 centers of Turkey.
  
  
  METHODS
  One hundered and sixty-three patients with TTP treated with TPE during the past 5years from 10 centers of Turkey were retrospectively evaluated. TPE was carried out 1-1.5times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150×10(9)/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded.
  
  
  RESULTS
  Fifty-eight percent (95/163) of the patients were females. The median age of the patients was 42years (range; 16-82). The median age of male patients was significantly higher than female (53 vs. 34years; p<0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary TTP comprised of 85.9% (140/163) of the patients and secondary TTP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary TTP. The median number of TPE was 13 (range; 1-80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p<0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary TTP (85% vs. 87%, respectively; p=0.806). There was no advantage of TPE+prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p=0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TTP [15% (21/140) vs. 13% (3/23); p=0.806]. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE+prednisolone [14% (12/86) vs. 3% (2/67), p<0.001].
  
  
  CONCLUSIONS
  TPE is an effective treatment for TTP and is associated with high CR rate in both primary and secondary TTP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of TTP and if these two criteria met in a patient, TPE should be performed immediately.
authors.affiliation: Cumhuriyet University, Department of Hematology, Sivas, Turkey. baranserdalkorkmaz@gmail.com
authors.forename: Serdal
authors.initials: S
authors.lastname: Korkmaz
authors.forename: Muzaffer
authors.initials: M
authors.lastname: Keklik
authors.forename: Serdar
authors.initials: S
authors.lastname: Sivgin
authors.forename: Rahsan
authors.initials: R
authors.lastname: Yildirim
authors.forename: Anil
authors.initials: A
authors.lastname: Tombak
authors.forename: Mehmet Emin
authors.initials: ME
authors.lastname: Kaya
authors.forename: Didar Yanardag
authors.initials: DY
authors.lastname: Acik
authors.forename: Ramazan
authors.initials: R
authors.lastname: Esen
authors.forename: Sibel Kabukcu
authors.initials: SK
authors.lastname: Hacioglu
authors.forename: Mehmet
authors.initials: M
authors.lastname: Sencan
authors.forename: Ilhami
authors.initials: I
authors.lastname: Kiki
authors.forename: Eyup Naci
authors.initials: EN
authors.lastname: Tiftik
authors.forename: Irfan
authors.initials: I
authors.lastname: Kuku
authors.forename: Vahap
authors.initials: V
authors.lastname: Okan
authors.forename: Mehmet
authors.initials: M
authors.lastname: Yilmaz
authors.forename: Cengiz
authors.initials: C
authors.lastname: Demir
authors.forename: Ismail
authors.initials: I
authors.lastname: Sari
authors.forename: Fevzi
authors.initials: F
authors.lastname: Altuntas
authors.forename: Ali
authors.initials: A
authors.lastname: Unal
authors.forename: Osman
authors.initials: O
authors.lastname: Ilhan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange / Q000379:methods*; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000628:therapy*; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase