Subcutaneous panniculitis-like T-cell…

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Subcutaneous panniculitis-like T-cell lymphoma

ORPHA:86884Disease

Also called SPTCL · Subcutaneous panniculitic T-cell lymphoma

What it is

A rare cytotoxic cutaneous alpha-beta T-cell lymphoma characterized by solitary or multiple erythematous subcutaneous nodules and plaques that can be localized to the lower extremities or generalized. It has been recognized as a distinct subset of peripheral T-cell lymphomas originating and presenting primarily in the subcutaneous fat tissue and often associated with hemophagocytic lymphohistiocytosis.

Key facts

Age of onset
All ages
Inheritance
Not applicable
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

HAVCR2Disease-causing germline mutation(s)

ICD-10 codes

C86.3ICD-10 names this disease exactly

Cross-references

GARD 10193MESH C537503MONDO 0019475OMIM 618398UMLS C0522624

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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