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📋Overview
Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired hematologic disorder characterized by destruction of red blood cells due to a defect in the surface proteins that protect them from complement-mediated lysis. It is caused by somatic mutations in the PIGA gene in hematopoietic stem cells, leading to deficiency of glycosylphosphatidylinositol (GPI)-anchored proteins. PNH is classified as a clonal bone marrow disorder and is associated with hemolytic anemia, thrombosis, and bone marrow failure. The term PNH remains the preferred and modern designation.
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Medical codes (for reference)
UMLS CUI: C0024790ICD-10-CM
D59.5
MeSH
D006457
SNOMED CT (US)
1963002
Codes are provided for reference and interoperability. They are not a diagnosis.
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