Abstract
Objective
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA), and
therapeutic plasma exchange (TPE) is currently the standard treatment. However, TPE
sometimes cannot be implemented. The aim of this study was to systematically review
patients with a first TTP episode who were treated without TPE.
Method
The PubMed, Embase, Web of Science and Cochrane Library databases were searched by
two investigators independently to collect case reports and clinical studies on TTP
patients treated without TPE. After removing duplicate records and records that did
not meet the inclusion criteria, the patients’ data of eligible studies, including
the basic characteristics, treatment regimens, and outcomes were extracted for further
analysis.
Results
A total of 5338 potentially relevant original studies were identified, from which
21 studies, including 14 cases, 3 case series and 4 retrospective studies, met eligibility
requirements and were included. Treatment regimens in the absence of TPE were found
to vary based on individual information. Most patients recovered, with normal platelet
counts and ADAMT13 activity at discharge. In the meta-analysis of retrospective studies,
the TPE-free group had no higher mortality than the TPE-treated group.
Conclusion
Our study shows that TPE-free treatment may not increase the mortality of TTP patients,
which provides a new treatment concept for patients with first episodes of TTP. However,
the current evidence is not high due to the lack of randomized controlled trials,
so more well-designed prospective clinical trials are warranted to investigate the
safety and efficacy of TPE-free treatment regimens in TTP patients.
Keywords
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References
- Caplacizumab in adult patients with acquired thrombotic thrombocytopenic purpura.Therapeutic Adv Hematol. 2020; : 11
- Thrombotic thrombocytopenic purpura.Blood. 2017; 129: 2836-2846
- Congenital thrombotic thrombocytopenic purpura.Tidsskr Nor Laege. 2016; 136: 1452-1457
- Acquired autoimmune thrombotic thrombocytopenic purpura.Nursing. 2019; 49: 22-29
- Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura.N Engl J Med. 2019; 380: 335-346
- Effectiveness of caplacizumab nanobody in acquired thrombotic thrombocytopenic purpura refractory to conventional treatment.Acta Haematol. 2021; : 1-5
- Post approval experience with caplacizumab for acquired thrombotic thrombocytopenic purpura at a single institution.J Clin Med. 2021; : 10
- Caplacizumab for treatment of thrombotic thrombocytopenic purpura in a patient with anaphylaxis to fresh-frozen plasma.Transfusion. 2020; 60: 1666-1668
- Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.J Clin Apher. 2021; 36: 499-504
- Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab.J Thromb Haemost. 2020; 18: 3061-3066
- High incidence of relapses in thrombotic thrombocytopenic purpura. Clinical study of 38 patients.Am J Med. 1987; 83: 437-444
- Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group.N Engl J Med. 1991; 325: 393-397
- Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias.Br J Haematol. 2003; 120: 556-573
- Transition from fresh frozen plasma to solvent/detergent plasma in the Netherlands: comparing clinical use and transfusion reaction risks.Haematologica. 2020; 105: 1158-1165
- Efficacy and safety of plasma exchange using a double viral inactivated and prion reduced solvent/detergent fresh frozen plasma for the treatment of thrombotic microangiopathy: the first French experience in a single center.Transfus Apher Sci. 2020; 59102587
- Type of plasma preparation used for plasma exchange and clinical outcome of adult patients with acquired idiopathic thrombotic thrombocytopenic purpura: a French retrospective multicenter cohort study.Transfusion. 2015; 55: 2445-2451
- [Thrombotic thrombocytopenic purpura].Ugeskr Laege. 2021; : 183
- Safety and efficacy of cryoprecipitate-poor plasma as a replacement fluid for therapeutic plasma exchange in thrombotic thrombocytopenic purpura: a single center retrospective evaluation.J Clin Apher. 2014; 29: 311-315
- Cryoprecipitate-poor plasma fraction (cryosupernatant) in the treatment of thrombotic thrombocytopenic purpura at onset. A report of four cases.Haematologica. 1996; 81: 175-177
- Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet.Br J Haematol. 2005; 129: 79-86
- Pegylated bovine carboxyhaemoglobin utilisation in a thrombotic thrombocytopenic purpura patient.Transfus Med. 2017; 27: 300-302
- Successful management of a Jehovah's witness with thrombotic thrombocytopenic purpura unwilling to be treated with therapeutic plasma exchange.J Clin Apher. 2007; 22: 330-332
- Successful management of thrombotic thrombocytopenic purpura in a Jehovah's Witness without plasma exchange.J Clin Apher. 2015; 30: 46-49
- Survival of a Jehovah's Witness with thrombotic thrombocytopenic purpura without using plasma: a case report and review of the literature.J Clin Apher. 2019; 34: 623-630
- Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13.Br J Haematol. 2007; 136: 451-461
- Rituximab therapy for refractory thrombotic thrombocytopenic purpura.Blood Cells Mol Dis. 2002; 28: 385-391
- Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center.Crit Care Med. 2012; 40: 104-111
- A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP.Blood. 2021; 137: 733-742
- Caplacizumab: first global approval.Drugs. 2018; 78: 1639-1642
- Caplacizumab for acquired thrombotic thrombocytopenic purpura.N Engl J Med. 2016; 374: 511-522
- Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura.N Engl J Med. 2019; 380: 335-346
- Caplacizumab as an emerging treatment option for acquired thrombotic thrombocytopenic purpura.Drug Des Dev Ther. 2019; 13: 1251-1258
- Reversible posterior leukoencephalopathy syndrome in a case of adult onset Still's disease with concurrent thrombotic thrombocytopenic purpura: response to high dose immunoglobulin infusions.J Assoc Phys. India. 2012; 60: 59-62
- Drug-induced thrombotic thrombocytopenic purpura successfully treated with recombinant human soluble thrombomodulin.Intern Med. 2013; 52: 1111-1114
- Successful treatment of transplantation-associated thrombotic microangiopathy with recombinant human soluble thrombomodulin.Bone Marrow Transpl. 2010; 45: 803-805
- Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors.Blood. 2019; 134: 1037-1045
Article info
Publication history
Published online: February 28, 2023
Accepted:
February 23,
2023
Received in revised form:
February 21,
2023
Received:
October 10,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2023 Elsevier Ltd. All rights reserved.
