Understanding PNH
Who gets PNH?
For every one million people, there are about 12-13 patients with PNH each year
Both men and women can get PNH
PNH can occur at any age; with diagnosis generally in the early 30s
PNH affects people of every racial and ethnic group
What is PNH?
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that affects your red and white blood cells and platelets. In PNH, red blood cells are destroyed. This destruction is called hemolysis.
PNH is a chronic (long-term) disease that is progressive, which means it can get worse over time.
What causes PNH?
PNH is an acquired disease, which means you don't inherit it. Instead, it may develop over time.
PNH is caused by a change or mutation in the body's stem cells, which are located in the bone
marrow.
The bone marrow is where red blood cells are produced.
What are the effects
of hemolysis?
What are the
effects of
hemolysis?
Hemolysis, the destruction of red blood cells, is the main cause of the symptoms of PNH, including blood clots and organ damage. When you have PNH, hemolysis is always happening. There are blood tests that measure how much hemolysis is happening at a given time. Regular hemolysis testing can help you and your doctor monitor your PNH.
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Testing for PNH |
PNH can be difficult to diagnose. But there are tests that can help. If you have hemolysis, your doctor may order a high sensitivity flow cytometry test (HSFC)
An HSFC test is a blood test that measures the percentage of blood cells affected by PNH, cells which are missing a protective protein that healthy blood cells have. This percentage is known as clone size.
What is clone size?
Clone size is a percentage(or number) of blood cells affected by PNH. A larger clone size means you have a higher percentage of PNH blood cells, but even small clone size can lead to health problems. So, it's important to monitor your clone size on a regular basis.
Some tests your doctor may order
Lactate dehydrogenase (LDH) level This test measures the level of LDH (a type of protein) in your red blood cells. Because LDH is released during hemolysis, LDH levels can show how much hemolysis is happening in your body. A high LDH level can mean a lot of your red cells have been destroyed.
Complete blood count (CBC) This test measures different parts of your blood, including red blood cells, white blood cells, and hemoglobin.
Track your test results
There are a number of different tests used to monitor and track PNH. Your doctor will use these results to better manage your disease. Download a Lab tracker to keep your own records of tests results, and discuss any changes or concerns you have with your doctor.
Testing for PNH
PNH can be difficult to diagnose. But there are tests that can help. If you have hemolysis, your doctor may order a high sensitivity flow cytometry test (HSFC)
An HSFC test is a blood test that measures the percentage of blood cells affected by PNH, cells which are missing a protective protein that healthy blood cells have. This percentage is known as clone size.
What is clone size?
Clone size is a percentage (or number) of blood cells affected by PNH. A larger clone size means you have a higher percentage of PNH blood cells, but even small clone size can lead to health problems. So, it's important to monitor your clone size on a regular basis.
Some tests your doctor may order
Lactate dehydrogenase (LDH) level This test measures the level of LDH (a type of protein) in your red blood cells. Because LDH is released during hemolysis, LDH levels can show how much hemolysis is happening in your body. A high LDH level can mean a lot of your red cells have been destroyed.
Complete blood count (CBC) This test measures different parts of your blood, including red blood cells, white blood cells, and hemoglobin.
Track your test results
There are a number of different tests used to monitor and track PNH. Your doctor will use these results to better manage your disease. Download a Lab tracker to keep your own records of tests results, and discuss any changes or concerns you have with your doctor.