Table 6.

Recommendations for flow cytometric analysis in diagnosis and management of PNH

  • For patients with clinical evidence of hemolysis (classic PNH and PNH/aplastic anemia)

    • At diagnosis, flow cytometric analysis of both erythrocytes* and granulocytes† is recommended.

    • After establishment of the diagnosis, flow cytometric analysis is recommended every 6 months for 2 years and yearly thereafter if the parameters are stable.

    • If there is evidence of clinical progression (or amelioration), more immediate analysis should be performed.

  • For patients with aplastic anemia or refractory anemia-MDS‡without clinical evidence of hemolysis

    • At diagnosis, analysis of erythrocytes and granulocytes using high-sensitivity flow cytometry.

    • Every year, even in the absence of clinical evidence of hemolysis (including patients treated with immunosuppressive therapy).

 

  • For patients with clinical evidence of hemolysis (classic PNH and PNH/aplastic anemia)

    • At diagnosis, flow cytometric analysis of both erythrocytes* and granulocytes† is recommended.

    • After establishment of the diagnosis, flow cytometric analysis is recommended every 6 months for 2 years and yearly thereafter if the parameters are stable.

    • If there is evidence of clinical progression (or amelioration), more immediate analysis should be performed.

  • For patients with aplastic anemia or refractory anemia-MDS‡without clinical evidence of hemolysis

    • At diagnosis, analysis of erythrocytes and granulocytes using high-sensitivity flow cytometry.

    • Every year, even in the absence of clinical evidence of hemolysis (including patients treated with immunosuppressive therapy).

 
*

Best performed prior to transfusion or, if clinically feasible, during a period of transfusion abstinence. The results of the analysis for erythrocytes should include the percentage type I, type II, and type III cells.

†

Analysis of granulocytes is needed for the following 2 reasons: (1) analysis provides the best estimate of the size of the PNH clone(s); (2) analysis is unaffected by red cell transfusion.

‡

Regardless of marrow cellularity.

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