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| __NOTOC__ | | __NOTOC__ |
| {{Paroxysmal nocturnal hemoglobinuria}} | | {{Paroxysmal nocturnal hemoglobinuria}} |
| {{CMG}}; {{AE}} | | {{CMG}}; {{AE}} {{AEL}} |
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| ==Overview== | | ==Overview== |
| | Surgical intervention is not recommended for the management of paroxysmal nocturnal hemoglobinuria. |
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| ==Surgery== | | ==Surgery== |
| | | Surgical intervention is not recommended for the management of paroxysmal nocturnal hemoglobinuria. |
| === Hematopoietic cell transplantation ===
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| * Hematopoietic stem cell transplantation is an important curative therapy for paroxysmal nocturnal hemoglobinuria.
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| * Stem cell transplantation is usually reserved for the severely affected patients and is indicated for the following type of patients:<ref name="pmid28013015">{{cite journal| author=DeZern AE, Zahurak M, Symons H, Cooke K, Jones RJ, Brodsky RA| title=Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia. | journal=Biol Blood Marrow Transplant | year= 2017 | volume= 23 | issue= 3 | pages= 498-504 | pmid=28013015 | doi=10.1016/j.bbmt.2016.12.628 | pmc=5373094 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28013015 }}</ref>
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| ** Patients with severe aplastic anemia who are HLA matched donor.
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| ** Patients with myelodysplastic syndromes
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| ** Patients who are unresponsive to the anti complement therapy (eculizumab)
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| * 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>
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| ** Acute Gravt-Versus-Host Disease (GVHD) occurs in almost third of the PNH patients treated with transplant.
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| ** Survival rate of the PNH patients treated with transplantation is from 50% to 60%.
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| ***
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| ==References== | | ==References== |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]
Overview
Surgical intervention is not recommended for the management of paroxysmal nocturnal hemoglobinuria.
Surgery
Surgical intervention is not recommended for the management of paroxysmal nocturnal hemoglobinuria.
References
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