Granulomatous cutaneous T-cell lymphoma (G-CTCL) is a rarely encountered entity. Most G-CTCL is CD4+, with granulomatous mycosis fungoides representing the vast majority of cases. Because of the rarity of CD8+ G-CTCL, there is a paucity of data regarding the clinicopathologic features and expected course.
To describe the clinical and histopathologic features of G-CTCL.
This is a retrospective review of collected cases.
We present 4 cases of CD8+ G-CTCL. Patients presented with papules and nodules on the trunk and extremities without antecedent patch or plaque disease. In all cases, biopsy specimens were obtained, and these revealed a dense granulomatous infiltrate accompanied by an atypical lymphoid infiltrate of CD8+ T cells. T-cell clonality studies were positive in 3 of 4 cases. Staging was negative for nodal involvement, but lung granulomas were seen in all cases. In all 4 cases, the patient's medical history was significant for immunodeficiency, either primary or iatrogenic. All 4 patients had slowly progressive disease.
This is a small retrospective case series.
CD8+ G-CTCL appears to be associated with immunodeficiency. The finding of a CD8+ G-CTCL should prompt an evaluation for underlying immunodeficiency. Additional studies are required to validate these conclusions.Le texte complet de cet article est disponible en PDF.
Key words : common variable immunodeficiency, cutaneous T-cell lymphoma, cytotoxic, granulomatous, immunodeficiency, mycosis fungoides, X-linked agammaglobulinemia
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| Conflicts of interest: None declared.