ABSTRACT
Thrombotic thrombocytopenic purpura is an uncommon disease that often presents with
a striking and acute clinical picture. Although first described more than 80 years
ago, the therapy has changed little since the discovery that plasma, and then plasma
exchange, could bring about a remarkable response, changing survival rates from 20%
to more than 80%. However, although plasma exchange is now considered the standard
of care, there is still considerable discussion concerning the appropriate schedules
for therapy and the types of fluids that should be used. Because plasma exchange (PE)
is not effective in all patients, and at least 30% of patients will have one or more
relapses, several adjuvant therapies have also been used. Most recently, this has
included the use of rituximab to achieve an immunomodulatory effect.
KEYWORDS
Thrombotic thrombocytopenic purpura (TTP) - plasma exchange - cryosupernatant - rituximab
REFERENCES
- 1
Moschcowitz E.
Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed
disease.
Proc NY Pathol Soc.
1924;
24
21-24
- 2
Moschcowitz E.
An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles
and capillaries.
Arch Intern Med.
1925;
36
89-93
- 3
Rubinstein M A, Kagan B M, MacGillviray M H, Merliss R, Sacks H.
Unusual remission in a case of thrombotic thrombocytopenic purpura syndrome following
fresh blood exchange transfusions.
Ann Intern Med.
1959;
51
1409-1419
- 4
Bukowski R M, King J W, Hewlett J S.
Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura.
Blood.
1977;
50
413-417
- 5
Amorosi E I, Ultmann J E.
Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature.
Medicine (Baltimore).
1966;
45
139-159
- 6
Rock G, Shumak K H, Buskard N A and the Members of the CASG et al.
Comparison of plasma exchange with plasma infusion in the treatment of thrombotic
thrombocytopenic purpura.
N Engl J Med.
1991;
325
393-397
- 7
Bell W R, Braine H G, Ness P M, Kickler T S.
Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
Clinical experience in 108 patients.
N Engl J Med.
1991;
325
398-403
- 8
Byrnes J J, Khurana M.
Treatment of thrombotic thrombocytopenic purpuras with plasma.
N Engl J Med.
1977;
297
1386-1389
- 9
Moake J L, Rudy C K, Troll J H et al..
Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing
thrombotic thrombocytopenic purpura.
N Engl J Med.
1982;
307
1432-1435
- 10
Rock G, Shumak K H, Sutton D M et al..
Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic
purpuras. Members of the Canadian Apheresis Group.
Br J Haematol.
1996;
94
383-386
- 11
Rock G, Anderson D, Clark W et al..
Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura?
No answer yet?.
Br J Haematol.
2005;
129
79-86
- 12
Zeigler Z R, Shadduck R K, Gryn J F et al..
Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic
thrombocytopenic purpura (TTP).
J Clin Apheresis.
2001;
16
19-22
- 13
Tsai H M, Lian E C.
Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic
purpura.
N Engl J Med.
1998;
339
1585-1594
- 14
Furlan M, Robles R, Galbusera M et al..
Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and
the hemolytic-uremic syndrome.
N Engl J Med.
1998;
339
1578-1584
- 15 Rock G, Yousef H, Neurath D, Lu M. Levels of ADAMTS-13 in fresh, stored and solvent
detergent treated plasma. Paper presented at: the CSTM Meeting 2005 Banff, Canada;
- 16
Yarranton H, Lawrie A S, Purdy G, Mackie I J, Machin S J.
Comparison of von Willebrand factor antigen, von Willebrand factor-cleaving protease
and protein S in blood components used for treatment of thrombotic thrombocytopenic
purpura.
Transfus Med.
2003;
14
39-44
- 17
Moake J L, Chintagumpala M, Turner N et al..
Solvent/detergent-treated plasma suppresses shear-induced platelet aggregation and
prevents episodes of TTP.
Blood.
1994;
84
490-497
- 18
Tandon N N, Rock G, Jamieson G A.
Anti-CD36 antibodies in thrombotic thrombocytopenic purpura.
Br J Haematol.
1994;
88
816-825
- 19
Morrison J, McMillan R.
Elevated platelet-associated lgG in thrombotic thrombocytopenic purpura.
JAMA.
1977;
238
1944-1945
- 20
Leung D Y, Moake J L, Havens P L, Kim M, Pober J S.
Lytic anti-endothelial cell antibodies in haemolytic-uraemic syndrome.
Lancet.
1988;
2
183-186
- 21
Gilcher R O, Strauss R G, Clavarella D et al..
Management of renal disorders.
J Clin Apheresis.
1993;
8
258-269
- 22 AABB Extracorporeal Therapy Committee .Guidelines for Therapeutic Hemapheresis:
1992-3. Bethesda, MD; American Association of Blood Banks 1992
- 23
Bandarenko N, Brecher M E.
United States Thrombotic Thrombocytopenic Purpura Apheresis Study Group (US TTP ASG):
multicenter survey and retrospective analysis of current efficacy of therapeutic plasma
exchange.
J Clin Apheresis.
1998;
13
133-141
- 24
Stegmayr B G.
Plasmapheresis in severe sepsis or septic shock.
Blood Purif.
1996;
14
94-101
- 25
Stegmayr B G, Banga R, Berggren L, Norda R, Rydvall A, Vikerfors T.
Plasma exchange as rescue therapy in multiple organ failure including acute renal
failure.
Crit Care Med.
2003;
31
1730-1736
- 26 Valbonesi M, Giannini G, Carlier P et al.. TTP treated with the automatic cascade
filtration module (DIDECO AutoCFM). WAA September 2002 (abst)
- 27
Gaddis T G, Guthrie T HJ, Drew M J, Sahud M, Howe R B, Mittelman A.
Treatment of plasma refractory thrombotic thrombocytopenic purpuras with protein A
immunoabsorption.
Am J Hematol.
1997;
55
55-58
- 28
Snyder H W, Mittelman A, Oral A et al..
Treatment of cancer chemotherapy associated thrombotic thrombocytopenia purpura/hemolytic
uremic syndrome by protein A immunoabsorption of plasma.
Cancer.
1993;
71
1882-1892
- 29
Bukowski R M, Hewlett J S, Reimer R R, Groppe C W, Weick J K, Livingston R B.
Therapy of thrombotic thrombocytopenic purpura: an overview.
Semin Thromb Hemost.
1981;
7
1-14
- 30
Amir J, Krauss S.
Treatment of thrombotic thrombocytopenic purpura with antiplatelet drugs.
Blood.
1973;
42
27-33
- 31
Amorosi E L, Karpatkin S.
Antiplatelet treatment of thrombotic thrombocytopenic purpura.
Ann Intern Med.
1977;
86
102-106
- 32
Zheng X L, Kaufman R M, Goodnough L T, Sadler J E.
Effect of plasma exchange on plasma ADAMTS-13 metalloprotease activity, inhibitor
level, and clinical outcome in patients with idiopathic and non-idiopathic thrombotic
thrombocytopenic purpura.
Blood.
2004;
103
4043-4049
- 33
Cataland S R, George I N, Kraut E H et al..
Improved clinical outcome and ADAMTS-13 activity following cyclosporine therapy in
patients with TTP.
Blood.
2004;
104
243
, (abst)
- 34
Zheng X L, Pallera A M, Goodnough I T et al..
Remission of chronic thrombotic thrombocytopenic purpura treated with cyclophosphamide
and rituximab.
Ann Intern Med.
2003;
138
105-108
- 35
Rock G, Kelton J G, Shumak K H, Buskard N A, Sutton D MC. and Members of the CAG .
Laboratory abnormalities in thrombotic thrombocytopenic purpura.
Br J Haematol.
1998;
103
1031-1036
- 36
Boye J, Elter T, Engert A.
An overview of the current clinical use of the anti-CD20 monoclonal antibody rituximab.
Ann Oncol.
2003;
14
520-526
- 37
Maloney D G, Grillo-Lopez A J, Bodkin D J et al..
IDEC-C2B8: results of phase I multiple dose trail in patients with relapsed non-Hodgkin's
lymphoma.
J Clin Oncol.
1997;
10
3266-3274
- 38
Davis T A, White C A, Grillo-Lopez A J et al..
Single-agent monoclonal antibody efficacy in bulky non-Hodgkin's lymphoma: results
of phase II trail of rituximab.
J Clin Oncol.
1999;
6
1851-1857
- 39
Ciiffier B, Lapage E, Briere J et al..
CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with
diffuse large B-cell lymphoma.
N Engl J Med.
2002;
346
235-242
- 40
Hainsworth J D, Litchy S, Barton J H et al..
Single agent rituximab as first line and maintenance treatment for patients with chronic
lymphocytic leukemia or small lymphocytic lymphoma: a phase II trial of the Minnie
Pearl Cancer Research Network.
J Clin Oncol.
2003;
21
1746-1751
- 41
van der Kolk L, Baars J, Prins M, van Oers M.
Rituximab treatment results in impaired secondary humoral immune responses.
Blood.
2002;
100
2257-2259
- 42
Koulova L, Alexandrescu D, Dutcher J P, O'Boyle K P, Eapen S, Wiernik P H.
Rituximab for the treatment of refractory idiopathic thrombocytopenic purpura (ITP)
and thrombotic thrombocytopenic purpura (TTP): report of three cases.
Am J Hematol.
2005;
78
49-54
- 43
Chemnitz J, Draube A, Scheid C et al..
Successful treatment of severe thrombotic thrombocytopenic purpura with the monoclonal
antibody rituximab.
Am J Hematol.
2002;
71
105-108
- 44
Shumak K H, Rock G A, Nair R C.
Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura.
Canadian Apheresis Group.
Ann Intern Med.
1995;
122
569-572
- 45
Schneppenheim R, Budde U, Oyen F et al..
Von Willebrand factor cleaving protease and ADAMTS-13 mutations in childhood TTP.
Blood.
2003;
101
1845-1850
- 46
Mannucci M, Canciani M T, Forza I, Lussana F, Lattuada A, Rossi E.
Changes in health and disease of ADAMTS-13 that cleaves von Willebrand factor.
Blood.
2001;
98
2730-2735
- 47
Moore J C, Haward C PM, Warentin T E, Kelton J G.
Decreased von Willebrand factor protease activity associated with thrombocytopenic
disorders.
Blood.
2001;
98
1842-1846
- 48
Studt J D, Kremer Hovinga J A, Rodonic R et al..
Familial acquired thrombotic thrombocytopenic purpura: ADAMTS-13 inhibitory autoantibodies
in identical twins.
Blood.
2004;
103
4195-4197
- 49
Veyradier A, Obert B, Houllier A, Meyer D, Girma J P.
Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies:
a study of 111 cases.
Blood.
2001;
98
1765-1772
- 50
Motto D, Zhang W, Zhu G et al..
Additional environmental and/or genetic factors are required to trigger TTP in ADAMTS-13-deficient
mice.
Blood.
2004;
104
77
, (abst)
- 51
Gerritsen H E, Robles R, Lämmle B, Furlan M.
Partial amino acid sequence of purified von Willebrand factor-cleaving protease.
Blood.
2001;
98
1654-1661
- 52
Fujikawa K, Suzuki H, McMullen B, Chung D.
Purification of human von Willebrand factor-cleaving protease and its identification
as a new member of the metalloproteinase family.
Blood.
2001;
98
1662-1666
- 53
Furlan M, Lämmle B.
Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic ursemic
syndrome: the role of von Willebrand factor-cleaving protease.
Best Pract Res Clin Haematol.
2001;
14
437-454
- 54
Studt J D, Kremer Hovinga J A, Alberio L, Bianchi V, Lämmle B.
von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies:
diagnostic experience 2001/2002 of a single research laboratory.
Swiss Med Wkly.
2003;
133
325-332
- 55
Barbot J, Costa E, Guerra M et al..
Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic
relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency
of von Willebrand factor-cleaving protease.
Br J Haematol.
2001;
113
649-651
- 56
Bohm M, Vigh T, Scharrer I.
Evaluation and clinical application of a new method for measuring activity of von
Willebrand factor-cleaving metalloprotease (ADAMTS-13).
Ann Hematol.
2002;
81
430-435
- 57
Mori Y, Wada H, Gabazza E C et al..
Predicting response to plasma exchange in patients with thrombotic thrombocytopenic
purpura with measurement of vWF-cleaving protease activity.
Transfusion.
2002;
42
572-580
- 58
Vesely S K, George J N, Lämmle B et al..
ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:
relation to presenting features and clinical outcomes in a prospective cohort of 142
patients.
Blood.
2003;
102
60-68
- 59
Rock G, Shumak K, Kelton J, Blanchette V, Buskard N, Spasoff R. and the Members of
the CASG .
Thrombotic thrombocytopenic purpura: outcome in 24 patients with renal impairment
treated with plasma exchange.
Transfusion.
1992;
32
710-714
- 60
George J N.
ADAMTS-13 Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Curr Hematol Rep.
2005;
4
167-169
Gail RockM.D. Ph.D. F.R.C.P.
Civic Campus, The Ottawa Hospital, 1053 Carling Avenue, Ottawa, Ontario K1Y 4E9, Canada
Email: grock@ottawahospital.on.ca