C86.40

HCC Category Mapping

What This Code Means

A rare, aggressive lymphoma caused by abnormal NK-cells with blast-like features that has not achieved remission.

Coding Tips

• •Verify blastic morphology is confirmed in pathology report
• •Ensure active disease status is documented

Clinical Significance

Blastic NK-cell lymphoma (now more precisely classified as blastic plasmacytoid dendritic cell neoplasm) is an extremely rare, aggressive hematologic malignancy with immature blast-like morphology. It often presents with skin involvement followed by rapid dissemination to bone marrow, lymph nodes, and other organs. Non-remission status carries a very poor prognosis with median survival typically under 18 months.

Documentation Requirements

• ✓Pathology confirmation with immunohistochemistry demonstrating blastic NK-cell markers (CD56, CD4, CD123) is essential.
• ✓Documentation must include disease extent, sites of involvement (skin, marrow, nodes), treatment regimen, response assessment, and explicit statement that remission has not been achieved.

Commonly Confused Codes