PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
- PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
- Start Date
- Expiration Date
- PNH, PI-Linked Antigen, Sugar Water
- CPT Codes
- 88184; 88185x5; 88187
- Reference Test
Lavender, EDTA Whole Blood
Collect Monday through Thursday only and not the day before a holiday
- Transport Info
Do not centrifuge
Do not remove plasma from cells
- Fasting Required?
- Patient Instructions
- Reference Range
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.