Paroxysmal nocturnal hemoglobinuria surgery: Difference between revisions
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** Patients with myelodysplastic syndromes | ** Patients with myelodysplastic syndromes | ||
** Patients who are unresponsive to the anti complement therapy (eculizumab) | ** Patients who are unresponsive to the anti complement therapy (eculizumab) | ||
* Transplantation related issues:<ref name="pmid22689687">{{cite journal| author=Peffault de Latour R, Schrezenmeier H, Bacigalupo A, Blaise D, de Souza CA, Vigouroux S et al.| title=Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria. | journal=Haematologica | year= 2012 | volume= 97 | issue= 11 | pages= 1666-73 | pmid=22689687 | doi=10.3324/haematol.2012.062828 | pmc=3487438 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22689687 }}</ref> | * Transplantation related issues:<ref name="pmid22689687">{{cite journal| author=Peffault de Latour R, Schrezenmeier H, Bacigalupo A, Blaise D, de Souza CA, Vigouroux S et al.| title=Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria. | journal=Haematologica | year= 2012 | volume= 97 | issue= 11 | pages= 1666-73 | pmid=22689687 | doi=10.3324/haematol.2012.062828 | pmc=3487438 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22689687 }}</ref><ref name="pmid16051736">{{cite journal| author=Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R et al.| title=Diagnosis and management of paroxysmal nocturnal hemoglobinuria. | journal=Blood | year= 2005 | volume= 106 | issue= 12 | pages= 3699-709 | pmid=16051736 | doi=10.1182/blood-2005-04-1717 | pmc=1895106 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16051736 }}</ref> | ||
** Acute Gravt-Versus-Host Disease (GVHD) occurs in almost third of the PNH patients treated with transplant. | ** Acute Gravt-Versus-Host Disease (GVHD) occurs in almost third of the PNH patients treated with transplant. | ||
** Survival rate of the PNH patients treated with transplantation is from 50% to 60%. | ** Survival rate of the PNH patients treated with transplantation is from 50% to 60%. | ||
Revision as of 16:21, 15 August 2018
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Paroxysmal nocturnal hemoglobinuria Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Surgery
Hematopoietic cell transplantation
- Hematopoietic stem cell transplantation is an important curative therapy for paroxysmal nocturnal hemoglobinuria.
- Stem cell transplantation is usually reserved for the severely affected patients and is indicated for the following type of patients:[1]
- Patients with severe aplastic anemia who are HLA matched donor.
- Patients with myelodysplastic syndromes
- Patients who are unresponsive to the anti complement therapy (eculizumab)
- Transplantation related issues:[2][3]
- Acute Gravt-Versus-Host Disease (GVHD) occurs in almost third of the PNH patients treated with transplant.
- Survival rate of the PNH patients treated with transplantation is from 50% to 60%.
References
- ↑ DeZern AE, Zahurak M, Symons H, Cooke K, Jones RJ, Brodsky RA (2017). "Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia". Biol Blood Marrow Transplant. 23 (3): 498–504. doi:10.1016/j.bbmt.2016.12.628. PMC 5373094. PMID 28013015.
- ↑ Peffault de Latour R, Schrezenmeier H, Bacigalupo A, Blaise D, de Souza CA, Vigouroux S; et al. (2012). "Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria". Haematologica. 97 (11): 1666–73. doi:10.3324/haematol.2012.062828. PMC 3487438. PMID 22689687.
- ↑ Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R; et al. (2005). "Diagnosis and management of paroxysmal nocturnal hemoglobinuria". Blood. 106 (12): 3699–709. doi:10.1182/blood-2005-04-1717. PMC 1895106. PMID 16051736.