ICD-10 Code D61.02 – Shwachman-Diamond syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D61.02 – Shwachman-Diamond syndrome
What it is
D61.02 identifies Shwachman-Diamond syndrome, an inherited disorder that affects bone marrow function and the pancreas. It is used when the diagnosis is documented and you need the specific ICD-10-CM code.
Clinical signs
Typical findings include neutropenia, recurrent infections, and exocrine pancreatic insufficiency with poor growth or steatorrhea. Skeletal abnormalities may occur; clinical features vary, so refer to documentation.
When to use this code
Use D61.02 when the provider documents Shwachman-Diamond syndrome as the confirmed diagnosis. It may appear in hematology, genetics, or gastroenterology records, especially when marrow failure and pancreatic involvement are both noted.
Code it for encounters focused on chronic management, evaluation of complications, or family history workups only if the patient is affected. If the record is unclear, check documentation before assigning the code.
Do not use for
Do not use this code for isolated neutropenia, malabsorption, or pancreatic insufficiency without a documented Shwachman-Diamond syndrome diagnosis. Check documentation if the condition is only suspected.
Coding tip
Assign D61.02 only when the syndrome is explicitly documented; otherwise, code the specific confirmed findings.