ICD-10 Code D76.1 – Hemophagocytic lymphohistiocytosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D76.1 – Hemophagocytic lymphohistiocytosis
What it is
D76.1 identifies hemophagocytic lymphohistiocytosis, a severe hyperinflammatory syndrome in which the immune system becomes overactive and damages tissues. Use it when the diagnosis is documented by the clinician.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include persistent fever, enlarged spleen or liver, cytopenias, and evidence of excessive immune activation.
When to use this code
Use D76.1 when the record specifically states hemophagocytic lymphohistiocytosis, including HLH or hemophagocytic syndrome. It may apply to primary or secondary HLH if the provider documents the condition. If the note only describes suspected HLH, confirm the final diagnosis before coding.
Do not use for
Do not use this code for nonspecific fever, sepsis, or cytopenia alone without documented HLH. Check documentation if the chart mentions hemophagocytosis on pathology but does not diagnose hemophagocytic lymphohistiocytosis.
Coding tip
Code D76.1 only when the provider clearly names HLH; otherwise, query or wait for definitive documentation.