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Paroxysmal nocturnal hemolobinurea (PNH)
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Paroxysmal nocturnal hemolobinurea is a rare disease in which red blood cells
break down earlier than normal.
Persons with this disease have blood cells that are missing a gene called PIG-A.
This gene allows a substance called glycosyl-phosphatidylinositol (GPI) to help
certain proteins stick to cells.
Without PIG-A, important proteins cannot connect to the cell surface and protect
the cell from substances in the blood called complement. As a result, red blood
cells break down too early. The red cells leak hemoglobin into the blood, which
can pass into the urine. This can happen at any time, but is more likely to occur
during the night or early morning.
The disease can affect people of any age. It may lead to aplastic anemia,
myelodysplastic syndrome, or acute myelogenous leukemia.
Risk factors, except for prior aplastic anemia, are not known.
Blood clots -- may form in some people
Dark urine -- comes and goes
Easy bruising or bleeding
Shortness of breath
Exams and Tests
Red and white blood cell counts and platelet counts may be low.
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Red or brown urine signals the breakdown of red blood cells and
that hemoglobin is being released into the body's circulation and eventually into
Tests that may be done to diagnose this condition may include:
Complete blood count (CBC)
Flow cytometry to measure certain proteins
Ham's (acid hemolysin) test
Serum hemoglobin and haptoglobin
Sucrose hemolysis test
Steroids or other drugs that suppress the immune system may help slow the break
down of red blood cells. Blood transfusions may be needed. Supplemental iron
and folic acid are provided. Blood thinners may also be needed to prevent clot
Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red
Bone marrow transplantation can cure this disease.
All patients with PNH should receive vaccinations against certain types of
bacteria to prevent infection. Ask your doctor which ones are right for you.
The outcome varies. Most people survive greater than 10 years after their
diagnosis. Death can result from complications such as blood clot formation
(thrombosis) or bleeding.
In rare cases, the abnormal cells may decrease over time.
Acute myelogenous leukemia
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Iron deficiency anemia