Paroxysmal nocturnal haemoglobinuria

Keywords: PNH, Flow cytometry for PNH

Description:

See also Flow cytometry.

Specimen:

5 mL blood in lithium heparin, ACD or EDTA.

Method:

Red cells and neutrophils are stained with directly conjugated antibodies specific for two of several GPI-linked proteins eg, CD55, CD58, CD59 and CD66b. Alternatively, a fluorescently labelled bacterial aerolysin (FLAER) which binds normal GPI expressing granulocytes and monocytes but not GPI deficient PNH cells, may be used.

The stained cells are then analysed by Flow cytometry.

The percentage of the affected cells are reported.

Application:

Diagnosis of suspected Paroxysmal nocturnal haemoglobinuria (PNH).

Interpretation:

The report will include identification and quantitation of PNH clone and an interpretative comment from the pathologist.

Small PNH clones may be detected in Myelodysplastic syndromes and Aplastic anaemia. 

Reference:

Bain et al. Dacie and Lewis Practical Haematology. 11th Ed. 2012.

Hillmen P et al. BJH. 2000; 108:470-479.

Sutherland et al. Cytometry Part B (Clinical Cytometry). 2007; 72B: 167-177.