Primary cutaneous gamma/delta t cell lymphoma C84.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Cutaneous cytotoxic gamma-delta positive T-cell lymphoma; Cutaneous cytotoxic T-cell lymphoma gamma-delta positive; Cutaneous cytotoxic T-cell lymphoma (gamma-delta positives); Cytotoxic (gamma-delta positive) cutaneous T-cell lymphoma

Definition
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Since 1994 recognized lymphoma entity (REAL classification) with predominantly systemic manifestations. Very rarely skin infestation (about 3% of cutaneous T-cell lymphomas). By definition, tumor cells express the gamma-delta T-cell receptor on the surface.

Occurrence/Epidemiology
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More common in organ transplants, in patients with Hodgkin's disease or lupus erythematosus in the medical history.

Manifestation
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In younger patients (average age in larger studies: 34 years).

Clinical features
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Clinic similar to mycosis fungoides or pagetoid reticulosis. A described subcutaneous form of gamma-delta positive cutaneous T-cell lymphoma is similar in course to panniculitis-like cutaneous T-cell lymphoma. Systemic infestation is frequent, especially in the liver, spleen and upper intestinal tract.

Therapy
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Progression/forecast
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Poor prognosis; mean survival time for systemic infestation = 16 months. Poor response to chemotherapy.

Literature
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  1. El-Shabrawi-Caelen L et al (2000) The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Sem Cutan Surg 19: 118-123
  2. Guizzardi M et al (2003) Cytotoxic gamma/delta subcutaneous panniculitis-like T-cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy. J Eur Acad Dermatol Venereol 17: 219-222
  3. Jaffe ES et al (2003) Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol 40: 175-184

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Last updated on: 29.10.2020