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{{ Paroxysmal nocturnal hemoglobinuria }}
{{Paroxysmal nocturnal hemoglobinuria}}
{{CMG}} {{shyam}}; {{AE}}  


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==Overview==
Fluorescent aerolysin (FLAER) can be used to diagnose There are no other diagnostic studies associated with paroxysmal nocturnal hemoglobinuria.


==Overview==
==Other Diagnostic Studies==
==Other Diagnostic Studies==
===Flow Cytometry===
Fluorescent aerolysin (FLAER) can be used to diagnose paroxysmal nocturnal hemoglobinuria.<ref name="pmid18063459">{{cite journal| author=Brodsky RA| title=Advances in the diagnosis and therapy of paroxysmal nocturnal hemoglobinuria. | journal=Blood Rev | year= 2008 | volume= 22 | issue= 2 | pages= 65-74 | pmid=18063459 | doi=10.1016/j.blre.2007.10.002 | pmc=2290854 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18063459  }} </ref> This test is based on the idea that FLAER binds to GPI anchors. In the absence of GPI anchors on the cell membrane, FLAER will not bind, so PNH cells are negative for FLAER.
 
Modern methods include [[flow cytometry]] for [[CD55]], [[CD16]], [[CD59]] and other GPI anchored proteins on [[white blood cells|white]] and[[red blood cells]]. <ref>Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socie G, International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. ''[[Blood (journal)|Blood]]2005;106:3699-709. PMID 16051736.</ref> Laboratories favor flow cytometry to evaluate PNH due to its high sensitivity and specificity.  Flow cytometry of the peripheral blood, not the bone marrow aspirate, is required to evaluate the presence or absence of GPI linked proteins. The bone marrow biopsy in PNH shows erythroid hyperplasia. In addition, because of the short life of granulocytes, the peripheral blood samples need to reach the lab in an expedited manner.  The most commonly used antibodies are CD59 (expressed on all hematocellular lineages), and CD55 but other GPI anchored antigens (CD14, CD16, CD24) can also be studied on leukocytes.  Dependent on the predominance of these molecules on the red blood cell surface, they are classified as type I, II or III PNH cells.


PNH type II & III cell populations; definitions.
Some patients may have erythrocytes with low but detectable GPI anchored proteins; these cells are designated PNH type II.  By contrast, cells that are completely devoid of GPI anchored proteins are referred to as PNH type III.  Patients with large populations of PNH type II erythrocytes may have less hemolysis than those with comparable populations of PNH III cells but these patients are still at risk for both hemolysis and thrombosis. 
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Hematology]]
[[Category:Rare diseases]]
[[Category:Genetic disorders]]
[[Category:Mature chapter]]


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Latest revision as of 22:28, 18 December 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief:

Overview

Fluorescent aerolysin (FLAER) can be used to diagnose There are no other diagnostic studies associated with paroxysmal nocturnal hemoglobinuria.

Other Diagnostic Studies

Fluorescent aerolysin (FLAER) can be used to diagnose paroxysmal nocturnal hemoglobinuria.[1] This test is based on the idea that FLAER binds to GPI anchors. In the absence of GPI anchors on the cell membrane, FLAER will not bind, so PNH cells are negative for FLAER.

References

  1. Brodsky RA (2008). "Advances in the diagnosis and therapy of paroxysmal nocturnal hemoglobinuria". Blood Rev. 22 (2): 65–74. doi:10.1016/j.blre.2007.10.002. PMC 2290854. PMID 18063459.

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