CD8+ granulomatous cutaneous T-cell lymphoma: A potential association with immunodeficiency

被引:24
作者
Gammon, Bryan [1 ,2 ]
Robson, Alistair [3 ]
Deonizio, Janyana [1 ]
Arkin, Lisa [1 ]
Guitart, Joan [1 ]
机构
[1] Northwestern Univ, Dept Dermatol, Evanston, IL 60208 USA
[2] Luminous Dermatol, Santa Barbara, CA 93105 USA
[3] St Thomas Hosp, St Johns Inst Dermatol, Dept Dermatopathol, London, England
关键词
common variable immunodeficiency; cutaneous T-cell lymphoma; cytotoxic; granulomatous; immunodeficiency; mycosis fungoides; X-linked agammaglobulinemia; OF-THE-LITERATURE; MYCOSIS-FUNGOIDES; ATAXIA-TELANGIECTASIA; SEZARY-SYNDROME; SKIN; BEXAROTENE; DISORDERS; FEATURES; PATIENT; STAGE;
D O I
10.1016/j.jaad.2014.03.028
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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.
引用
收藏
页码:555 / 560
页数:6
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