Talk:Paroxysmal nocturnal hemoglobinuria
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[edit] I've
I've been diagnosed with PNH but I think that many of my symptoms point to a G6PD deficiency.
- Well, your haematologist should surely be able to disentangle your haemolysis. Flow cytometry results? JFW | T@lk 14:39, 20 September 2005 (UTC)
[edit] Eculizumab
PMID 15985537 - eculizumab works. Worth putting this in? JFW | T@lk 14:39, 20 September 2005 (UTC)
[edit] Work
I'll be expanding this article with the consensus statement in Blood 1 Dec 2005. JFW | T@lk 21:45, 20 December 2005 (UTC)
[edit] Acclaim
Excellent article, and is easy to read...great for a second year medical student.
- I hope it is understandable also to those without a medical background. Feel free to show it to your tutors & encourage them to improve it. It's a wiki! JFW | T@lk 22:07, 8 March 2006 (UTC)
[edit] Chromosome 12 mutations underlying clonality
It has been unclear why PNH is a clonal bone marrow disorder. PMID 16940417 gives data from 2 PNH patients with chromosome 12 abnormalities leading to ectopic expression of HMGA2 in bone marrow. I don't think we should cite this until the findings have been replicated in larger cohorts of PNH patients. JFW | T@lk 05:05, 21 December 2006 (UTC)
[edit] Lethal
An anonymous editor insists that PNH is life-threatening. I disagree. Parker et al state clearly that there are subclinical variants of PNH, and obviously these are not life-threatening at all. I have asked 84.64.113.176 (talk · contribs) to justify further changes of this kind. JFW | T@lk 22:04, 14 February 2007 (UTC)
[edit] Update
doi:10.1111/j.1365-2141.2007.06554.x lists a host of symptoms I don't normally associate with PNH. Hoping to get the fulltext to update this article. JFW | T@lk 21:36, 5 April 2007 (UTC)
[edit] give classic case scenario for medical students
even if it often doesn't present with classical symptoms you should still give the classic presentation to help with medical students on exams, some are too lazy to go and research it on their own and need such help.
[edit] Clonality
The following reference was listed to support some of the pathophysiology data:
Hu R, Mukhina GL, Piantadosi S, Barber JP, Jones RJ, Brodsky RA (2005). "PIG-A mutations in normal hematopoiesis". Blood 105 (10): 3848–54. doi:. PMID 15687243. Full text at PMC: 1895084
However, it actually addresses the more unusual point that many healthy people have small PNH clones but that they have polyclonal PIGA mutations as opposed to "real" PNH patients, whose PIGA mutations are monoclonal. We need to reconsider the relevance of this study before reinserting it into the article. JFW | T@lk 12:01, 5 March 2008 (UTC)
[edit] Some sources
Argh, I need to give a case presentation on this subject on Friday! Some sources I've found are the following; we could use them to expand the article.
- Narrative review Ann Intern Med http://www.annals.org/cgi/content/full/148/8/587
- From the same author: http://asheducationbook.hematologylibrary.org/cgi/content/full/2006/1/24
- Blinded RCT of eculizumab (NEJM) PMID 16990386
- Thrombosis risk is also reduced in Soliris http://bloodjournal.hematologylibrary.org/cgi/content/full/110/12/4123
- Recent developments: http://www.blackwell-synergy.com/doi/abs/10.1111/j.1365-2141.2007.06554.x
Wonder how this will go. JFW | T@lk 07:40, 5 May 2008 (UTC)
- http://books.google.co.uk/books?id=27qcOn07USkC - entire book on PGI & PNH. ISBN 0127729402. JFW | T@lk 15:39, 6 May 2008 (UTC)
[edit] Removed
Animal studies suggest that infusing membrane-targeted CD59 might restore protection against complement-mediated lysis, and is being developed further for use in patients.[1]
[edit] Eculizumab again
http://www.blackwell-synergy.com/doi/abs/10.1111/j.1365-2141.2008.07183.x - anaemia and fatigue improve beyond improvement in transfusion requirements. JFW | T@lk 22:25, 22 May 2008 (UTC)

