Paroxysmal Nocturnal Hemoglobinuria
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Paroxysmal Nocturnal Hemoglobinuria is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder. The classic finding is the premature destruction of red blood cells (hemolysis), resulting in repeated episodes of hemoglobin in the urine (hemoglobinuria). Hemoglobin is the red, iron-rich pigment of blood. Individuals with hemoglobinuria may exhibit dark-colored or bloody urine. In addition to hemolysis, individuals with PNH are also susceptible to developing repeated, potentially life-threatening blood clots (thromboses).
Affected individuals also have some degree of underlying bone marrow dysfunction or insufficiency. Bone marrow, the spongy center of the large bones of the body, produces hematopoietic stem cells, which grow and eventually develop into red blood cells (erythrocytes), white blood cells (leukocytes), and platelets. Severe bone marrow dysfunction potentially results in low levels of red and white blood cells and platelets (pancytopenia). The specific symptoms of PNH vary greatly, and affected individuals usually do not exhibit all of the symptoms potentially associated with the disorder.
Aplastic Anemia & MDS International Foundation, Inc.
100 Park Avenue
Rockville, MD 20850
NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
Bethesda, MD 20892-6612
NIH/Hematology Branch, National Heart, Lung and Blood Institute (NHLBI)
10 Center Dr, Building 10-CRC
Bethesda, MD 20892-1202
PNH Support Group
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
PO Box 241956
Los Angeles, CA 90024
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