Description

Paroxsmal nocturnal hemoglobinuria (PNH) is a condition that causes hemolytic anemia, bone marrow failure, and a proclivity to develop thrombosis. PNH occurs when the PIG-A gene of bone marrow stem cells undergoes mutation. Stems cells develop into erythrocytes (RBCs), white blood cells (WBCs), platelets, and mature cells that contain the PIG-A gene mutation, and as a result, lack a sufficient number of glycosylphosphatidylinositol-anchored protein (GPI-AP). The GPI-APs have various roles, some of which are to help to prevent RBC destruction, fight infection, and promote thrombosis.https://www.hopkinsmedicine.org/kimmel_cancer_center/types_cancer/paroxysmal_nocturnal_hemoglobinuria_PNH.html CD14, 16, 24, and 59 are GPI-linked proteins that are used as markers for the presence of GPI, as is fluorescent aerolysin (FLAER), which binds to GPI-APs. Patients with PNH lack sufficient GPIs and therefore have reduced expression of these cell markers and FLAER compared to patients without PNH. Patients with complete loss of CD59 are more likely to have hemolysis compared to patients with partial loss of CD59. Source: Regenstrief LOINC

Basic Part Properties

Part Name
Paroxysmal nocturnal hemoglobinuria GPI-Linked WBC & RBC Ag
Part Display Name
PNH GPI-Linked WBC and RBC Ag
Part Type
Component (Describes the core component or analyte measured)
Created On
2018-09-28
Construct for LOINC Short Name
PNH GPI-Lkd WBC + RBC Ag

LOINC Terminology Service (API) using HL7® FHIR® Get Info

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=LP269302-8

Language Variants Get Info

TagLanguageTranslation
fr-FRFrench (France)Hémoglobinurie paraxystique nocturne GPI-Linked leucocytaire et érythrocytaire antigène
pl-PLPolish (Poland)Napadowa nocna hemoglobinuria GPI-związane WBC & RBC Ag
Synonyms: Antygeny związane z GPI na WBC i RBC w napadowej nocnej hemoglobinurii