D58.0
Hereditary spherocytosis
Clinical Classification Guidelines
Inclusion Terms
- Acholuric (familial) jaundice
- Congenital (spherocytic) hemolytic icterus
- Minkowski-Chauffard syndrome
Medical Intelligence & Overview
Hereditary spherocytosis is a genetic blood disorder characterized by the production of abnormally shaped red blood cells called spherocytes. These spherical cells are less flexible and more prone to breaking apart, leading to hemolytic anemia. This condition is often inherited and can vary in severity from mild to severe. It is sometimes referred to by other terms such as Minkowski-Chauffard syndrome or familial jaundice, and is associated with congenital hemolytic jaundice. Recognizing and understanding this disorder is vital for managing its effects on health.
Causes & Symptoms
Clinical Causes: Genetic mutations affecting proteins that maintain red blood cell membrane structure Inheritance patterns, often autosomal dominant, passed from parent to child Familial cases showing a hereditary tendency to develop spherocytes Potentially linked to other inherited hemolytic disorders, though specifically caused by mutations affecting membrane proteins like ankyrin or spectrin
Key Symptoms: Anemia symptoms such as fatigue, weakness, and pallor Jaundice, particularly during episodes of increased red blood cell breakdown Enlarged spleen (splenomegaly) Gallstones caused by excess bilirubin due to increased breakdown of red blood cells Episodes of anemia that may worsen during infections or physical stress
Diagnostic & Treatment
Diagnosis Path: Diagnosis involves a combination of blood tests and clinical evaluations including:
Treatment Protocols: While there is no universal cure for hereditary spherocytosis, various management strategies are employed:
Clinical Advice & FAQs
Billing Guidance
Is D58.0 a billable ICD-10 code?
Yes, D58.0 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D58.0?
Clinical documentation must specify the nature of Hereditary spherocytosis and any associated comorbidities for accurate reporting.
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