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Lymphoid · AggressiveICD-10 C84.4

Primary cutaneous γδ T-cell lymphoma

PCGD-TCL; cutaneous γδ T-cell lymphoma; the aggressive γδ subset previously included in SPTCL until WHO 2008

Primary cutaneous γδ T-cell lymphoma is a rare, highly aggressive cytotoxic T-cell lymphoma derived from mature activated γδ T-cells of the skin. The 2008 WHO Classification removed γδ T-cell lesions from the indolent αβ subcutaneous panniculitis-like T-cell lymphoma (SPTCL) umbrella to recognise this distinct, aggressive entity. Patients present with rapidly evolving infiltrated patches, plaques and ulcerated tumours on the limbs and trunk, with frequent ulceration and necrosis. Haemophagocytic lymphohistiocytosis (HLH) — a life-threatening immune dysregulation syndrome — complicates 30–50% of cases. Median survival is only 12–24 months even with intensive multi-agent chemotherapy. Allogeneic haematopoietic stem-cell transplantation offers the best chance of long-term remission for fit patients.

CurrentLast reviewed 26 April 2026

Clinical features

  • Rapidly evolving, infiltrated, indurated patches, plaques and ulcerated tumours.
  • Predominantly on the limbs and trunk; mucosal sites less common.
  • Often with necrosis, eschar and crusting; mistaken for vasculitis or pyoderma gangrenosum early.
  • B symptoms (fever, weight loss, night sweats) are common.
  • Median age 50–60; both sexes; immunosuppressed patients (HIV, transplant) over-represented.
  • Subcutaneous, dermal-only and epidermotropic patterns described — all behave aggressively.
  • Frequent association with haemophagocytic lymphohistiocytosis (HLH) — hyperferritinaemia, cytopenias, hepatosplenomegaly, hypofibrinogenaemia, hypertriglyceridaemia.

Histology & immunophenotype

  • Atypical lymphoid infiltrate spanning the dermis ± subcutis ± epidermis with frequent angiocentric / angiodestructive growth and necrosis.
  • "Rimming" of adipocytes (similar to SPTCL) may be present in subcutaneous-predominant cases.
  • Phenotype distinguishes γδ from αβ SPTCL:
    • CD3+, CD4−, CD8−, βF1− (αβ TCR negative)
    • TCRγ / TCRδ positive (the diagnostic marker; demands fresh tissue or specialised IHC)
    • CD56+ in many cases
    • Cytotoxic markers (TIA-1, granzyme B, perforin) positive
    • EBER (EBV) negative — distinguishes from extranodal NK/T-cell lymphoma
  • Clonal T-cell receptor γ rearrangement.
  • Differential: SPTCL (αβ phenotype, indolent); extranodal NK/T-cell lymphoma (EBV+, CD56+); primary cutaneous CD8+ aggressive epidermotropic CTCL (αβ phenotype, CD8+); transformed mycosis fungoides; subcutaneous panniculitis (no atypia, no clonality).

Staging

  • CT chest/abdomen/pelvis ± PET-CT.
  • Bone marrow biopsy — extracutaneous involvement common.
  • HLH screen at diagnosis: FBC, LDH, ferritin, fibrinogen, triglycerides, soluble IL-2R.
  • EBV PCR (to exclude EBV-driven lymphoproliferation).
  • HIV testing.
  • EORTC TNM staging system for cutaneous lymphoma.
  • Refer to a national cutaneous lymphoma MDT (UK: Guy's, St George's, Manchester).

Management

  • Multi-agent chemotherapy — CHOP, CHOEP, gemcitabine-based regimens, brentuximab vedotin in CD30+ disease (off-licence; rare).
  • Allogeneic haematopoietic stem-cell transplantation in fit patients with chemo-sensitive disease — offers the best chance of long-term remission.
  • Localised radiotherapy for symptomatic lesions.
  • Haemophagocytic lymphohistiocytosis: urgent haematology referral; HLH-94 / HLH-2004 protocol (etoposide, dexamethasone, cyclosporine).
  • Clinical trial enrolment encouraged given paucity of effective treatments.
  • Palliative care input early in unfit patients.

Prognosis

Poor — median overall survival 12–24 months; 5-year overall survival ~10–20%. Adverse factors: subcutaneous involvement, HLH at presentation, advanced stage, immunosuppression. Allogeneic transplantation in selected patients can produce long-term remissions.

References

  1. Toro JR et al. γδ T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood; 2003.
  2. Willemze R et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood; 2019.
  3. WHO Classification of Haematolymphoid Tumours, 5th edition (WHO-HAEM5); 2022. International Consensus Classification of Mature Lymphoid Neoplasms; 2022.

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