PNH

From WikiMD.com - Food, Medicine & Wellness Encyclopedia

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the immune system. It is a type of hemolytic anemia and it leads to the release of hemoglobin into the urine (hemoglobinuria), primarily at night (paroxysmal).

Signs and symptoms[edit | edit source]

The clinical features of PNH are highly variable and can occur at any age. The most common symptoms are fatigue, shortness of breath, hemolytic anemia, dark urine, and abdominal pain. Other symptoms can include difficulty swallowing, erectile dysfunction, and thrombosis.

Causes[edit | edit source]

PNH is caused by a mutation in the PIGA gene, which leads to a deficiency of glycosylphosphatidylinositol (GPI), a molecule that anchors proteins to the cell surface. This deficiency makes the red blood cells susceptible to attack by the immune system.

Diagnosis[edit | edit source]

The diagnosis of PNH is typically made by flow cytometry, a test that measures the amount of GPI-linked proteins on the surface of red blood cells. Other tests that may be used include a complete blood count (CBC), liver function tests, and a bone marrow biopsy.

Treatment[edit | edit source]

Treatment for PNH is primarily aimed at managing the symptoms and preventing complications. This may include blood transfusions, iron supplements, and medications to suppress the immune system. In severe cases, a bone marrow transplant may be considered.

Prognosis[edit | edit source]

The prognosis for individuals with PNH varies. Some people may have a mild form of the disease and live a normal life span, while others may have severe disease and die from complications such as thrombosis or leukemia.

See also[edit | edit source]

References[edit | edit source]


WHO Rod.svg
This article is a medical stub. You can help WikiMD by expanding it!
PubMed
Wikipedia
WikiMD
Navigation: Wellness - Encyclopedia - Health topics - Disease Index‏‎ - Drugs - World Directory - Gray's Anatomy - Keto diet - Recipes

Transform your life with W8MD's budget GLP1 injections from $125

W8mdlogo.png
W8MD weight loss doctors team

W8MD offers a medical weight loss program NYC and a clinic to lose weight in Philadelphia. Our W8MD's physician supervised medical weight loss centers in NYC provides expert medical guidance, and offers telemedicine options for convenience.

Why choose W8MD?

Book Your Appointment

Start your NYC weight loss journey today at our NYC medical weight loss, and Philadelphia medical weight loss Call (718)946-5500 for NY and 215 676 2334 for PA

Search WikiMD

Ad.Tired of being Overweight? Try W8MD's NYC physician weight loss.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available. Call 718 946 5500.
Advertise on WikiMD

WikiMD's Wellness Encyclopedia

Let Food Be Thy Medicine
Medicine Thy Food - Hippocrates

Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates, categories Wikipedia, licensed under CC BY SA or similar.

Contributors: Prab R. Tumpati, MD