D59.5
Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]
Clinical Classification Guidelines
Excludes Type 1
- hemoglobinuria NOS (R82.3)
Medical Intelligence & Overview
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired blood disorder characterized by the destruction of red blood cells, leading to a range of health issues. This condition occurs when abnormal blood cells lack certain protective proteins, making them more vulnerable to destruction by the body’s immune system. PNH can cause hemolytic anemia, blood clots, and other complications, affecting individuals of all ages, though it is more commonly diagnosed in adults.
Causes & Symptoms
Clinical Causes: Genetic mutation in the PIGA gene that occurs randomly in blood stem cells Development of blood cells that lack protective proteins on their surface Absence of glycosylphosphatidylinositol (GPI) anchors, which normally attach protective proteins to cell surfaces
Key Symptoms: Episodes of dark-colored urine, especially in the morning or after physical activity Fatigue and weakness resulting from anemia Shortness of breath and dizziness abdominal pain, blood clots, and back pain in severe cases Pain or swelling in the extremities due to blood clots Bleeding issues or easy bruising
Diagnostic & Treatment
Diagnosis Path: Diagnosis of PNH involves several laboratory tests, primarily focusing on detecting the absence of specific proteins on the surface of blood cells. These tests include:
Treatment Protocols: While there is no universal cure for PNH, several treatments aim to manage symptoms, reduce hemolysis, and prevent complications. These include:
Clinical Advice & FAQs
Billing Guidance
Is D59.5 a billable ICD-10 code?
Yes, D59.5 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D59.5?
Clinical documentation must specify the nature of Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli] and any associated comorbidities for accurate reporting.
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