Table of Contents
- Overview
- Symptoms
- Treatment
- Prevention
- Images
Paroxysmal nocturnal hemoglobinuria is a rare disease in which red blood cells break down earlier than normal.
Alternative Names
PNH
Causes, incidence, and risk factors
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
Risk factors, except for prior aplastic anemia, are not known.
Images
Review Date: 03/28/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of
General Medicine, Department of Medicine, University of Washington
School of Medicine; and James R. Mason, MD, Oncologist, Director,
Blood and Marrow Transplantation Program and Stem Cell Processing
Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by
David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)
