Clinical Presentation and Follow-up of Eighteen Patients with Thrombotic Thrombocytopenic Purpura

  • Ahmed Khudair Yaseen Lecturer in medicine and clinical hematology : Hawler medical university-colllege of medicine /Erbil-Iraq.
  • Ahmed Abdulmajeed Elsaffar Consultant internist and clinical hematologist : Medical city – Baghdad Teaching hospital /Baghdad-Iraq.
Keywords: Thrombotic thrombocytopenic purpura, Iraq, Plasma Exchange


Background and Objectives: Major advances achieved during the past 30years in under-standing the pathophysiology of Thrombotic Thrombocytopenic Purpura (TTP). More recently treatment outcomes were revolutionized by the introduction of plasma exchange. In this study a descriptive analysis of clinical presentation and treatment outcomes with a modified Plasma Exchangee protocol (PE) is presented.

Methods: During the period from November 2004 to July 2006, eighteen patients with acute episode of TTP were enrolled. After clinical and Laboratory evaluation, patients were assigned to receive modified “low volume plasma exchange “, and their response was monitored for a mean of 64 weeks.

Results: The classical Pentad of TTP was only documented in 27% cases. Of patients enrolled, one died before starting PE, while of the remaining 17 put on PE Complete remission (CR) was achieved in 70% with a mean follow up of 64 weeks. Four patients died during the first few days of starting PE. Of patients who achieved CR none had a relapse.

Conclusions: The classical “pentad” of TTP is present in small proportion of patients at presentation. Our results with the modified “low volume exchange ” may suggest that even small volume of plasma exchange can be life saving .


Moake JL. Thrombotic Microangiopathies. N. Eng J Med 2002; 347:589-98

Rock GA . Managment of Thrombotic Thrombo-cytopenic Purpura . Br J Hematol 2000;109:496-507

Levine SP. ,Greer JP. RodgrsGM ,Foerster J. , Thrombotic Thrombocytopenic Purpura and other forms of Non - Immunologic Platelets Destruction : Wintrobe’s Clinical Hematology; Eleventh edition Lippincott Williams & Wilkins. 2004: 1555-1564

George JN . How I Treat Patients with Throm-botic Thrombocytopenic Purpura- Hemolytic Uremic Syndrome.Blood;2000:1223-29

Furlan M., Robles R., Galbursera M., Remuzzi G , Kyrle PA, Brenner B et al. Von Willebr and factor-Cleaving Protease in Thrombotic Thrombocyto-penic Purpura and the Hemolytic -Uremic Syn-drome. N Eng J Med.1998;339:1578-84

Veyradier A ., Obert B ., HoulierA ., Grima JP . Specific Von Willebrand factor - CleavingProtease in Thrombotic Microan-giopathies: a study of 111 cases.Blood 2001;98:1765-72

Moake JL.Idiopathic Thrombotic Thrombocyto-penic ASH Year book 2004:407-12

Mannuci PM.,Canciani MT.,Forza I., et al. Changes in Health and Disease of Metalloprotease that Cleaves Von Willebrand Factor. Blood 2002; 98:2730-35.

ToffelmireEB, Clark WF, Cordy PE, Lin-tonAL ,Lohmann RC: Plasma exchange in throm-botic thrombocytopenic Purpura.Canad Med Ass J;1984:131:1371-1376.

Moake JL: Moschcowitz, Multimers, and Metallo-protease.N Eng J Med:1998;339:1629-1631.

Rock GA.,Shumak KH., Buskard NA., et al (The Canadian Apheresis Study Group). Comparison of Plasma Exchange with Plasma Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura. N Eng J Med 1991; 325:393-97.

Vesely SK ., George JN. , Lammle B., et al .ADAMTS13 activity in TTP-HUS: Relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003;102:60-68.

Thompson CE ., Damon LE . , Ries CA , Linker CA. Thrombotic Microangiopathies in the 1980 s : Clinical Features , Response to Treatment and the Impact of Human Immunodeficiency Vi-rus Epidemic. Blood 1992;80 : 1890-95

George JN., Diagnosis of Thrombotic Throm-bocytopenic Purpura in ASH year Book 2004 :416-21

Rick ME. , Austin H. ,Leitman SF., et al . Clinical Usefulness a Functional Assay for TheVon Wille-brand Factor Cleaving Protease (ADAMTS13) and Its’ inhibitor in a Patient with TTP. Am J of Hematol.2003; 75:96-100

Bell WR.,Braine HG.,Ness PM. Et al Improved Survival in Thrombotic Thrombocytopenic Pur-pura : clinical experience in 108 patients. N Eng J Med 1991; 325:398-403

Taft EG., Thrombotic Thrombocytopenic Purpura and dose of Plasma exchange.Blood 1979 ; 54 : 842 - 49.

Bukowski RM.,King JW., Hewlett JS. Plas-mapheresis in the treatment of Thrombotic Thrombocytopenic Purpura. Blood 1977; 50:413-17.

Tsai HM . , Lian ECY. Antibodies to Von Wille-brand f actor – Cleaving Protease In Acute Thrombotic Thrombocytopenic Purpura. N Eng J M.1998;339:1585- 94

Rock GA . ,Anderson D . ,Clark W . ,Leblond P, Palmer D,Sternbach M et al :Does Cryosuper-natant Plasma Improve outcome in TTP,No an-swer yet.Br JHematol :2005;129:79-86.

Yomotovian R., Niklinski W., Silver B.,Sarode R, Tsai HM Rituximab for Chronic RecurringTTP.:A case report and review of the literature .Br J He-matol .2004;124:787-95

Burns EA. , lou Y. , Pathak A. , Morphologic Diagnosis of Thrombotic Thrombocytopenic Purpura . Am. J. of Hematol. 2003; 75 :18-21

Rock GA . , John GK . , Kenneth HS , et al . Laboratory Abnormalities in Thrombotic Throm-bocytopenic Purpura. Br J Hematol :1998; 103:1031-36

Wyllie BF.,Grge AX.,Macnab J., et al . TTP-HUS: anew index predicting response to plasmaex-change. Br J Hematol 2005; 132:20

How to Cite
Yaseen, A., & Elsaffar, A. (2018). Clinical Presentation and Follow-up of Eighteen Patients with Thrombotic Thrombocytopenic Purpura. Zanco Journal of Medical Sciences (Zanco J Med Sci), 14(1), 55 - 60. Retrieved from
Original Articles