Paroxysmal nocturnal hemoglobinuria diagnostic study of choice
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]
Overview
Diagnostic Study of Choice
Minimal essential diagnostic criteria
- Diagnosis of paroxysmal nocturnal hemoglobinuria is based on the minimal essential diagnostic criteria. The main diagnostic test for this criteria is flow cytometry.[1]
- The flow cytometry approach is used to reveal the deficiency of GPI anchor on the RBCs.
- Other measures used in the diagnostic criteria include the folllowing:
- Assessment of the hemolytic parameters
- Bone marrow examination
- Supporting tests are included also in order to detect the PNH category. Below tables conclude both supporting tests and categories of PNH.[2]
Category of PNH | Characteristics |
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1- Classic PNH |
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2- PNH in the setting of another specified bone marrow disorder |
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3- Subclinical PNH |
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Supporting approaches |
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1- Obtaining the history of symptoms of hemoglobinuria, thromboembolic disease, dysphagia, and abdominal pain.
2- Identifying the proportion of the PNH I, PNH II, and PNH III erythrocytes by the flow cytometric analysis 3- Identifying the proportion of the GPI deficient blood cells 4- Serum Iron levels 5- Kidney function tests 6- Serum level of erythropoietin 7- Urine hemosiderin 8- HLA class identification |
References
- ↑ 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.
- ↑ Pu JJ, Brodsky RA (2011). "Paroxysmal nocturnal hemoglobinuria from bench to bedside". Clin Transl Sci. 4 (3): 219–24. doi:10.1111/j.1752-8062.2011.00262.x. PMC 3128433. PMID 21707954.