PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

Code
900.1767
Name
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
Category
None
Department
Send-Out
Start Date
Expiration Date
Synonyms
PNH, PI-Linked Antigen, Sugar Water
CPT Codes
88184; 88185x5; 88187
Site
SBMF
Reference Test
36161
ATLAS Test Code

Specimen Information

Type

Lavender, EDTA Whole Blood
Collect Monday through Thursday only and not the day before a holiday

Volume

5.0 ml

Transport Info

Do not centrifuge
Do not remove plasma from cells
Refrigerated

Fasting Required?
False
Patient Instructions

Reference Range

See report

Methodology

Flow Cytometry

Clinical Significance

Screening for or confirming a diagnosis of PNH. Evaluation of patients with hemolytic anemia or pancytopenia of undetermined cause.
Paroxysmal Nocturnal Hemoglobinuria (PNH) is an uncommon acquired hematologic disorder characterized by nocturnal hemoglobinuria, chronic hemolytic anemia with acute episodes, pancytopenia, thrombosis and, in some patients, acute or chronic myeloid malignancies. It is a disorder of hematopoietic stem cells and affects erythroid, myeloid and megakaryocytic cell lines. It is caused by mutations in the phosphatidylinositol glycan A (PIG-A) gene of the stem cell. This results in disruption of the synthesis of GPI and a deficiency in all GPI-anchored proteins in the affected cells. A consequential absence of the GPI linked complement inhibitors CD55 and CD59 causes the blood cells to be susceptible to complement mediated hemolysis.

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