Why is it called paroxysmal nocturnal hemoglobinuria?
The condition gets its name from one of its symptoms: dark or bright red blood in your urine at night or in the morning. “Paroxysmal” means “sudden,” “nocturnal” means “at night,” and “hemoglobinuria” means “blood in the urine.” It happens in up to 50% of people with PNH.
What is FLAER test?
From Wikipedia, the free encyclopedia. Fluorescein-labeled proaerolysin (FLAER) is used in a flow cytometric assay to diagnose paroxysmal nocturnal hemoglobinuria (PNH). The assay takes advantage of the action of proaerolysin, a prototoxin of aerolysin, a virulence factor of the bacterium Aeromonas hydrophila.
What is PNH blood disease?
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood. The disease is characterized by destruction of red blood cells, blood clots, and impaired bone marrow function. PNH is closely related to aplastic anemia.
What is Flaer test for PNH?
FLAER is an Alexa488-labeled inactive variant of aerolysin that does not cause lysis of cells. Our goals were to develop a FLAER-based assay to diagnose and monitor patients with PNH and to improve detection of minor populations of PNH clones in other hematologic disorders.
What is the difference between hematuria and hemoglobinuria?
If freshly collected urine from a patient with hematuria is centrifuged, red blood cells settle at the bottom of the tube, leaving a clear yellow urine supernatant. If the red color is due to hemoglobinuria, the urine sample remains clear red after centrifugation.
Is PNH an autoimmune disease?
Although the exact relationship among these disorders is unknown, researchers now believe that PNH arises from autoimmune bone marrow failure, which is the cause of most cases of acquired aplastic anemia and some cases of myelodysplasia.
Is PNH curable?
The only way to cure PNH is with what’s called an allogenic stem cell transplant. Also called a bone marrow transplant, it replaces your damaged stem cells with healthy ones from a donor, usually a close relative because they’re often the best match.
Do red blood cells split?
Doctors call this breaking apart “hemolysis.” It happens because the surface of a person’s blood cells are missing a protein that protects them from the body’s immune system. When red blood cells break apart, the hemoglobin inside is released.
Why does PNH cause early morning hematuria?
Signs and symptoms The classic sign of PNH is red discoloration of the urine due to the presence of hemoglobin and hemosiderin from the breakdown of red blood cells. As the urine is more concentrated in the morning, this is when the color is most pronounced.
What is PNH with Flaer high sensitivity?
Paroxysmal Nocturnal Hemoglobinuria (PNH) with FLAER (High Sensitivity) – The high sensitivity and quantitative flow cytometry assay is used in the diagnosis and follow-up of patients with paroxysmal nocturnal hemoglobinuria (PNH).