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