Bryan Gammon1, Alistair Robson2, Janyana Deonizio3, Lisa Arkin3, Joan Guitart3. 1. Department of Dermatology, Northwestern University, Chicago, Illinois; Luminous Dermatology, Santa Barbara, California. Electronic address: bryan.gammon@gmail.com. 2. Department of Dermatopathology, St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom. 3. Department of Dermatology, Northwestern University, Chicago, Illinois.
Abstract
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.
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.
Authors: Dita Gratzinger; Elaine S Jaffe; Amy Chadburn; John K C Chan; Daphne de Jong; John R Goodlad; Jonathan Said; Yasodha Natkunam Journal: Am J Clin Pathol Date: 2017-02-01 Impact factor: 2.493