You know the basics: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired blood disorder with fewer than 500 cases diagnosed each year in the United States. The condition causes red blood cells to break apart, releasing hemoglobin, which leads to symptoms like fatigue, racing heart, stomach contractions, trouble swallowing, and headaches.
PNH is diagnosed in all races and genders and at all ages. While there are no links between your race or ethnicity and your likelihood of developing the blood disease, your race does affect the risk of complications with PNH. Specifically, if you’re Black or Hispanic, you’re at a greater risk of thrombosis.
What Is Thrombosis?
Thrombosis is a condition that causes blood clots to block your blood vessels. Clots can also break free and travel to other parts of your body, which can cause complications like heart attack, stroke, or pulmonary embolism. It can cause several symptoms, including:
- Throbbing pain in the one leg, typically in your calf or inner thigh
- Swelling in your leg or arm
- Chest pain
- Skin that’s paler than other areas or cool to the touch
- Weakness, numbness, or tingling in your arms or legs
- Sharp chest pain
- Trouble breathing
- Slurred or garbled speech
- Dizziness
What Are the Types of Thrombosis?
There are two main types of thrombosis: arterial thrombosis and venous thrombosis.
- Arterial thrombosis is when a blood clot forms in an artery. This is the most common cause of heart attacks and strokes.
- Venous thrombosis is when a blood clot forms in a vein. It can cause pulmonary embolism, which is a blood clot in your lung.
With PNH, venous thrombosis is more common than arterial thrombosis. And the most common site for clots is the hepatic vein in your liver.
How Does Your Race Affect Your Thrombosis Risk?
Overall, there’s a 40% risk of having thrombosis if you have PNH. It’s the most common cause of PNH-related death.
The risk of complications from thrombosis differ based on race. Black people with PNH have a 73% risk while Hispanic people have a 50% risk. This is compared with a risk of just 36% among those who are White or Asian. It’s not clear why race affects thrombosis risk.
Managing Your Health
Since PNH puts you at higher risk of thrombosis, especially if you are Black or Hispanic, it’s important to take steps to manage your health. Here are some tips to doing so:
Reduce your risk. Some risks of thrombosis, like PNH, can’t be controlled, but there are lifestyle factors that increase the risks. These include your weight (obesity is a risk factor for thrombosis), tobacco use, high blood pressure, and high cholesterol. Ask your doctor about ways to manage these risks.
Understand treatment options. Seek emergency care if you experience any of the symptoms of thrombosis. Immediate treatment with anticoagulants (blood thinners) won’t get rid of the clot, but can prevent it from getting bigger.
New medications, called C5 complement inhibitors, have helped decrease the number of life-threatening thrombosis events in those with PNH.
Connect to the community. Support groups of peers with PNH can provide you with comfort and compassion when dealing with a rare disease. Connect with others who have PNH through organizations like the Aplastic Anemia and MDS International Foundation to feel less isolated in your disease-related challenges.
Talk to a PNH expert. The diagnosis and treatment of PNH can be complicated. Not all health care providers will have experience treating the blood disorder. It’s important to find a doctor who is a PNH expert, and don’t be afraid to ask for a second opinion. Having a medical team you trust is important.
If you’re Black or Hispanic and were diagnosed with PNH, it’s essential to understand the risk of thrombosis. The more information you know about the warning signs, treatments, importance of sticking with a treatment plan, and seeking support from your peers with PNH, the more empowered you’ll feel to take control of your health.
Show Sources
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SOURCES:
Aplastic Anemia and MDS International Foundation: “PNH—Paroxysmal Nocturnal Hemoglobinuria,” “Support Groups.”
National Organization for Rare Disorders: “Paroxysmal Nocturnal Hemoglobinuria.”
Shah, N. and Bhatt, H.: “Paroxysmal Nocturnal Hemoglobinuria,” StatPearls Publishing.
Johns Hopkins Medicine: “Thrombosis.”
Cleveland Clinic: “Thrombosis.”
Frontiers in Oncology: “Thromboembolic events in patients with paroxysmal nocturnal hemoglobinuria (PNH): Real world data of a Greek nationwide multicenter retrospective study.”
