TY - JOUR
T1 - CD8+ granulomatous cutaneous T-cell lymphoma
T2 - A potential association with immunodeficiency
AU - Gammon, Bryan
AU - Robson, Alistair
AU - Deonizio, Janyana
AU - Arkin, Lisa
AU - Guitart, Joan
PY - 2014/9
Y1 - 2014/9
N2 - 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.
AB - 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.
KW - X-linked agammaglobulinemia
KW - common variable immunodeficiency
KW - cutaneous T-cell lymphoma
KW - cytotoxic
KW - granulomatous
KW - immunodeficiency
KW - mycosis fungoides
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U2 - 10.1016/j.jaad.2014.03.028
DO - 10.1016/j.jaad.2014.03.028
M3 - Article
C2 - 24813299
AN - SCOPUS:84906314870
SN - 0190-9622
VL - 71
SP - 555
EP - 560
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 3
ER -