Out of state clients use this requisition when sending PNH testing
Paroxysmal Nocturnal Hemoglobinuria (PNH) Requisition for Out of State Clients

Clinical Significance:
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare but life threatening and debilitating disease.  Clinically, PNH is characterized by chronic intravascular hemolysis, bone marrow failure and life threatening thrombosis.  PNH evolves from a hematopoietic stem cell defect in which a somatic mutation of an x-linked gene (PIG-A) results in a partial or absolute deficiency of GPI-linked proteins.  Absent or markedly diminished expression of GPI-linked antigens is specific for all patients with PNH. 

Who should be tested:
Patients with aplastic anemia (AA), myelodysplastic syndrome (MDS), bone marrow failure syndromes or unexplained hemolysis.

Antibody Panels:
RBC panel:     CD235a-CD59
WBC panel:     FLAER-CD24-CD15-CD64-CD14-CD45 (monocytes and granulocytes)

Specimens: 
1-2 ml peripheral blood in EDTA or heparin (4 °C or room temperature).  

Forms: 
Dahl-Chase Flow Cytometry requisition form, demographic sheet and recent CBC results.

Transport:
Specimens need to be delivered and analyzed within 24-48 hours.
Flow Cytometry lab should be notified of coming specimen with shipment alert
UniShip courier pick-up available for in-state specimens
FedEx pick-up available for out-of-state specimens

CPT codes:
88184, 88185 x 7, 88187

Test performed: 
Monday through Saturday.

Tet reported:
Faxed or mailed within 24 hours after sample is received.

References:
1.     Borowitz et al: Guidelines for the Diagnosis and Monitoring of PNH and Related Disorders, Clin Cytometry 2010, 211-230
2.     Sutherland et al: Practical guidelines for the high-sensitivity detection and monitoring of PNH clones by flow cytometry. Cytometry B Clin Cytom 2012; 82:195-208.
3.     ICCS/ESCCA PNH consensus Guidelines 2018, 4 part series in Cytometry B