Subcutaneous panniculitis-like T-cell lymphoma
ICD-10 C86.3 · ICD-11 2B00&XH3NV1

Subcutaneous Panniculitis-like T-Cell Lymphoma with Hemophagocytic Lymphohistiocytosis — When First-Line Therapy Does Not Achieve Response

Clinical Scenario

This protocol applies to patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) presenting with hemophagocytic lymphohistiocytosis (HLH), systemic disease, or high tumor burden (widespread subcutaneous involvement) who did not achieve a complete response (CR) or partial response (PR) with initial treatment.

HLH & High-Risk Features

SPTCL with hemophagocytic lymphohistiocytosis, systemic spread, or extensive subcutaneous disease defines a high-risk presentation. In cases complicated by HLH, concurrent management of HLH alongside lymphoma-directed therapy is an important consideration.

Prior Treatment & Escalation Criterion

Patients in this scenario have previously received first-line therapy — which may include single-agent approaches with or without prednisone, etoposide-based combination chemotherapy, or involved-site radiation for limited disease. Failure to reach complete response (CR) or partial response (PR) to that first-line treatment is the criterion that escalates management to this next-step protocol.

Next-Step Approach (Overview Only)

For patients who relapse or do not respond adequately to first-line therapy, the structured approach involves systemic retreatment — drawing on either a prior regimen or an alternative not yet used. The full selection criteria, sequencing, and individualized considerations remain in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

SPTCL with HLH, Systemic Disease, or High Tumor Burden (Widespread Subcutaneous Disease)

In cases complicated by HLH, treatment of HLH concurrently with treatment of lymphoma is recommended.

Patients with disease relapse (after a period of observation or maintenance therapy) should receive treatment with either the same regimen previously given or an alternate regimen that was not given as first-line therapy.

Patients who do not achieve an adequate response to first-line therapy may be given a different first-line therapy or the same therapy at a higher dose to achieve disease control.

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