ICD-10 Code E75.241 – Niemann-Pick disease type B (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.241 – Niemann-Pick disease type B
What it is
E75.241 identifies Niemann-Pick disease type B, an inherited lysosomal storage disorder caused by acid sphingomyelinase deficiency. It leads to sphingomyelin accumulation, mainly affecting the spleen, liver, lungs, and blood system.
Clinical signs
Typical findings include hepatosplenomegaly, low platelet counts, and lung involvement with cough or shortness of breath. Clinical features vary; refer to documentation for the confirmed phenotype and organ involvement.
When to use this code
Use this code when the record specifically documents Niemann-Pick disease type B or acid sphingomyelinase deficiency with a type B phenotype. Apply it for confirmed diagnoses, including genetic, enzymatic, or specialist-supported documentation. If the chart only says Niemann-Pick disease without type, check documentation.
Do not use for
Do not use this code for Niemann-Pick disease types A, C, or other unspecified sphingolipidoses. Check documentation if the provider has not clearly identified type B.
Coding tip
Code the most specific Niemann-Pick type documented, and query when the subtype is missing or inconsistent.