Journal of the American Academy of Dermatology · 2014
CD8(+) granulomatous cutaneous T-cell lymphoma: a potential association with immunodeficiency.
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Gammon Bryan, Robson Alistair, Deonizio Janyana, Arkin Lisa, Guitart Joan
Аннотация
BACKGROUND: 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.
OBJECTIVE: To describe the clinical and histopathologic features of G-CTCL.
METHODS: This is a retrospective review of collected cases.
RESULTS: 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.
LIMITATIONS: This is a small retrospective case series.
CONCLUSIONS: 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.
OBJECTIVE: To describe the clinical and histopathologic features of G-CTCL.
METHODS: This is a retrospective review of collected cases.
RESULTS: 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.
LIMITATIONS: This is a small retrospective case series.
CONCLUSIONS: 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.