Difference between revisions of "Acquired thrombotic thrombocytopenic purpura"
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# Balduini CL, Gugliotta L, Luppi M, Laurenti L, Klersy C, Pieresca C, Quintini G, Iuliano F, Re R, Spedini P, Vianelli N, Zaccaria A, Pogliani EM, Musso R, Bobbio Pallavicini E, Quarta G, Galieni P, Fragasso A, Casella G, Noris P, Ascari E; Italian TTP Study Group. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol. 2010 Jun;89(6):591-6. Epub 2009 Dec 23. [http://link.springer.com/article/10.1007%2Fs00277-009-0877-5 link to original article] '''contains protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/20033409 PubMed] | # Balduini CL, Gugliotta L, Luppi M, Laurenti L, Klersy C, Pieresca C, Quintini G, Iuliano F, Re R, Spedini P, Vianelli N, Zaccaria A, Pogliani EM, Musso R, Bobbio Pallavicini E, Quarta G, Galieni P, Fragasso A, Casella G, Noris P, Ascari E; Italian TTP Study Group. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol. 2010 Jun;89(6):591-6. Epub 2009 Dec 23. [http://link.springer.com/article/10.1007%2Fs00277-009-0877-5 link to original article] '''contains protocol''' [https://www.ncbi.nlm.nih.gov/pubmed/20033409 PubMed] | ||
− | ==Rituximab | + | ==Rituximab monotherapy {{#subobject:4cadec|Regimen=1}}== |
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Revision as of 21:41, 18 August 2017
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Is there a regimen missing from this list? Would you like to share a different dosage/schedule or an additional reference for a regimen? Have you noticed an error? Do you have an idea that will help the site grow to better meet your needs and the needs of many others? You are invited to contribute to the site.
5 regimens on this page
7 variants on this page
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Initial therapy
Plasma exchange
Regimen
Study | Evidence | Comparator | Efficacy |
Rock et al. 1991 | Phase III | Plasma infusion | Seems to have superior OS |
- Total plasma exchange as follows (minimum of 7 treatments in first 9 hospital days):
- Treatments 1 to 3: Exchange of 1.5 times the predicted plasma volume
- Treatments 4 onwards: Exchange of 1.0 times the predicted plasma volume
Supportive medications
- Dipyridamole (Persantine) 400 mg (route not specified) once per day for at least two weeks
- Aspirin 325 mg PO once per day for at least two weeks
References
- Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991 Aug 8;325(6):393-7. link to original article contains verified protocol PubMed
Relapsed/Refractory
These treatments are usually used in addition to therapeutic plasma exchange, not in its place. There are also many treatments reported at the case report or case series level; the reader is guided to the 2015 Blood article, "How I treat refractory thrombotic thrombocytopenic purpura" for further details.
Methylprednisolone (Solumedrol)
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Regimen #1, "High-dose"
Study | Evidence | Comparator | Efficacy |
Balduini et al. 2009 | Phase III | Standard-dose methylprednisolone | Might have superior CR rate |
Therapy
- Methylprednisolone (Solumedrol) as follows:
- Days 1 to 3: 10 mg/kg/day IV
- Days 4 to 23: 2.5 mg/kg/day IV
Regimen #2, "Standard-dose"
Study | Evidence | Comparator | Efficacy |
Balduini et al. 2009 | Phase III | High-dose methylprednisolone | Might have inferior CR rate |
Therapy
- Methylprednisolone (Solumedrol) as follows:
- Days 1 to 3: 1 mg/kg/day IV
- Days 4 to 23: 2.5 mg/kg/day IV
References
- Balduini CL, Gugliotta L, Luppi M, Laurenti L, Klersy C, Pieresca C, Quintini G, Iuliano F, Re R, Spedini P, Vianelli N, Zaccaria A, Pogliani EM, Musso R, Bobbio Pallavicini E, Quarta G, Galieni P, Fragasso A, Casella G, Noris P, Ascari E; Italian TTP Study Group. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol. 2010 Jun;89(6):591-6. Epub 2009 Dec 23. link to original article contains protocol PubMed
Rituximab monotherapy
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Regimen #1
Study | Evidence |
de la Rubia et al. 2010 | Non-randomized |
Clark et al. 2015 | Phase II |
Full text of de la Rubia et al. 2010 is not available for review.
Chemotherapy
- Rituximab (Rituxan) 375 mg/m2 IV once per week
Supportive medications
- Prednisone (Sterapred) 50 mg PO once prior to Rituximab (Rituxan)
- Diphenhydramine (Benadryl) 50 mg PO once prior to Rituximab (Rituxan)
- Acetaminophen (Tylenol) 325 mg PO once prior to Rituximab (Rituxan)
4-week course
Regimen #2
Study | Evidence |
Froissart et al. 2012 | Non-randomized |
Chemotherapy
- Rituximab (Rituxan) (dose not specified) IV
Four doses in 15 days
References
- de la Rubia J, Moscardó F, Gómez MJ, Guardia R, Rodríguez P, Sebrango A, Zamora C, Debén G, Goterris R, López R, Peña F, Pujol M, Vidaller A, Del Río-Garma J, Sanz MA; Grupo Español de Aféresis. Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura: results of a Spanish multicenter study. Transfus Apher Sci. 2010 Dec;43(3):299-303. Epub 2010 Oct 12. link to original article contains protocol PubMed
- Froissart A, Buffet M, Veyradier A, Poullin P, Provôt F, Malot S, Schwarzinger M, Galicier L, Vanhille P, Vernant JP, Bordessoule D, Guidet B, Azoulay E, Mariotte E, Rondeau E, Mira JP, Wynckel A, Clabault K, Choukroun G, Presne C, Pourrat J, Hamidou M, Coppo P; French Thrombotic Microangiopathies Reference Center. 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 Jan;40(1):104-11. PubMed
- Review: Lim W, Vesely SK, George JN. The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura. Blood. 2015 Mar 5;125(10):1526-31. Epub 2015 Jan 8. Review. link to original article link to PMC article PubMed
- Clark WF, Rock G, Barth D, Arnold DM, Webert KE, Yenson PR, Kelton JG, Li L, Foley SR; members of the Canadian Apheresis Group. A phase-II sequential case-series study of all patients presenting to four plasma exchange centres with presumed relapsed/refractory thrombotic thrombocytopenic purpura treated with rituximab. Br J Haematol. 2015 Jul;170(2):208-17. Epub 2015 Apr 8. link to original article contains verified protocol PubMed
Vincristine (Oncovin)
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Regimen
Study | Evidence |
Ferrara et al. 1999 | Pilot, <20 patients |
Chemotherapy
- Vincristine (Oncovin) 1.4 mg/m2 IV once on day 1, then 1 mg IV once per day on days 4 & 7
References
- Ferrara F, Copia C, Annunziata M, Spasiano A, Di Grazia C, Palmieri S, Prossomariti L, Mele G. Vincristine as salvage treatment for refractory thrombotic thrombocytopenic purpura. Ann Hematol. 1999 Nov;78(11):521-3. contains protocol PubMed