PNH by Flow Cytometry

General Information

Lab Name

PNH by Flow Cytometry

Lab Code

PNHFLO

Epic Name

PNH by Flow Cytometry

Description

In Paroxysmal Nocturnal Hemoglobinuria (PNH), a clonal marrow stem cell population gives rise to circulating mature hematopoietic cells lacking the expression of a variety of different cell surface proteins whose common feature is their linkage to the cell membrane via a glycosyl-phosphatidyl-inositol (GPI) linkage, a linkage that is deficient in the PNH clone. Similar findings may also be seen in aplastic anemia and myelodysplasia, although the frequency of GPI-deficient cells is lower. GPI-deficiency is most easily assessed in the erythroid, granulocytic and monocytic lineages, and flow cytometry is the method of choice for their detection. The flow cytometric assay evaluates for a loss of expression of the following GPI-linked antigens: CD59 on red cells, CD14 and FLAER on monocytes, and CD24 and FLAER on granulocytes. The assay can detect as little as 0.01% GPI-deficient cells in each cell lineage.

References

Borowitz et al (2010) Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry. Cytometry Part B 78B:211 to 230.

Synonyms

CD59, GPI deficient, Paroxysmal Nocturnal Hemoglobin

Components