Primary cutaneous gamma/delta t cell lymphoma C84.5
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
DefinitionThis section has been translated automatically.
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/EpidemiologyThis section has been translated automatically.
More common in organ transplants, in patients with Hodgkin's disease or lupus erythematosus in the medical history.
ManifestationThis section has been translated automatically.
In younger patients (average age in larger studies: 34 years).
Clinical featuresThis section has been translated automatically.
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.
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
Poor prognosis; mean survival time for systemic infestation = 16 months. Poor response to chemotherapy.
LiteratureThis section has been translated automatically.
- El-Shabrawi-Caelen L et al (2000) The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Sem Cutan Surg 19: 118-123
- 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
- Jaffe ES et al (2003) Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol 40: 175-184