Sensitive and accurate identification of PNH clones based on ICCS/ESCCA PNH Consensus Guidelines—A summary

Autor:	D. Robert Sutherland, Iuri Marinov, Andrea Illingworth
Rok vydání:	2019
Předmět:	Consensus Clinical Biochemistry Hemoglobinuria Paroxysmal CD59 Antigens CD59 030204 cardiovascular system & hematology medicine.disease_cause Bioinformatics 03 medical and health sciences 0302 clinical medicine Germline mutation hemic and lymphatic diseases medicine Humans Mutation CD55 Antigens business.industry Biochemistry (medical) Membrane Proteins Hematopoietic stem cell Hematology General Medicine Flow Cytometry medicine.disease medicine.anatomical_structure Practice Guidelines as Topic Paroxysmal nocturnal hemoglobinuria Identification (biology) business 030215 immunology
Zdroj:	International Journal of Laboratory Hematology. 41:73-81
ISSN:	1751-553X 1751-5521
Popis:	Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder resulting from the somatic mutation of the X-linked phosphatidyl-inositol glycan complementation Class A (PIG-A) gene. Depending on the severity of the mutation in the PIG-A gene, there is a partial or absolute inability to make glycosylphosphatidyl-inositol (GPI)-anchored proteins including complement-defense structures such as CD55 and CD59 on RBCs and WBCs. Flow cytometric detection of PNH clones has become the gold standard and has played an increasingly important role in the diagnosis, monitoring, and clinical management of patients with PNH. Recently, a 4-part set of Consensus Guidelines have been published by flow experts in the field to address the key assay-specific considerations for the identification of PNH clones in RBC and WBC, how to report such data and a full validation document for the assays described. Below, we have summarized the most significant aspects of this International effort.
Databáze:	OpenAIRE
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