Table 2.

Minimal essential criteria required for diagnosis and categorization


  • Evidence of a population of peripheral blood cells (erythrocytes, granulocytes, or preferably both) deficient in GPI-APs. Flow cytometric analysis of peripheral blood erythrocytes, granulocytes, or both using primary antibodies against GPI-APs or the FLAER* assay reveals a population of hematopoietic cells that is partially or completely deficient in all GPI-APs.

  • Complete blood count, reticulocyte count, serum concentration of lactate dehydrogenase (LDH), bilirubin (fractionated), and haptoglobin

  • Bone marrow aspirate, biopsy, and cytogenetics


 

  • Evidence of a population of peripheral blood cells (erythrocytes, granulocytes, or preferably both) deficient in GPI-APs. Flow cytometric analysis of peripheral blood erythrocytes, granulocytes, or both using primary antibodies against GPI-APs or the FLAER* assay reveals a population of hematopoietic cells that is partially or completely deficient in all GPI-APs.

  • Complete blood count, reticulocyte count, serum concentration of lactate dehydrogenase (LDH), bilirubin (fractionated), and haptoglobin

  • Bone marrow aspirate, biopsy, and cytogenetics


 
*

FLAER (fluorescently labeled aerolysin). Aerolysin is a bacterial protein that binds selectively to GPI-APs. When used for flow cytometric analysis, leukocytes that express GPI-APs bind the fluorescently labeled reagent. PNH leukocytes do not bind FLAER because they do not express GPI-APs. Therefore, PNH leukocytes are identified in the flow cytometric histogram as a population of cells with absent or dim fluorescence. FLAER cannot be used for analysis of erythrocytes because, for unknown reasons, the reagent does not bind well to human red cells under the conditions required for the assay.

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