Description

A patient with paroxysmal nocturnal hemoglobinuria usually falls into one of two clinical phenotypes based on laboratory features.


 

Phenotypes:

(1) classic

(2) hypoplastic (cytopenic)

 

Laboratory testing:

(1) complete blood count (CBC)

(2) bone marrow cellularity

(3) reticulocyte count

(4) serum LDH (associated with hemolysis)

(5) percent granulocytes positive for PNH markers (clone of affected cells)

Test

Classic

Hypoplastic

CBC

normal to moderate cytopenias

moderate to severe cytopenias

bone marrow cellularity

normal to hypercellular

hypocellular

reticulocyte count

increased

decreased

serum LDH

increased

normal

percent granulocytes

> 30%

< 20%

 


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