Cutaneous &ggr;&dgr; T-cell Lymphomas: A Spectrum of Presentations With Overlap With Other Cytotoxic Lymphomas
Cutaneous &ggr;&dgr; T-cell Lymphomas: A Spectrum of Presentations With Overlap With Other Cytotoxic Lymphomas
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皮肤的
DOI：
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发表时间：
2012
影响因子：
5.6
通讯作者：
Youn H. Kim
中科院分区：
文献类型：
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作者：
J. Guitart;D. Weisenburger;A. Subtil;Ellen J. Kim;G. Wood;M. Duvic;E. Olsen;J. Junkins;S. Rosen;U. Sundram;D. Ivan;M. Selim;L. Pincus;Janyana M D Deonizio;M. Kwasny;Youn H. Kim
我们回顾了首次出现在皮肤的γ - δ (&ggr;&dgr;) t细胞淋巴瘤的多中心治疗经验。53名中位年龄61岁（范围25至91岁）的受试者被诊断患有这种疾病。出现皮肤病变的中位持续时间为1.25年（范围1个月至20年）。最常见的表现是深斑块（38例），通常类似于泛膜炎，其次是类似牛皮癣或蕈样真菌病的斑块（10例）。这些病变随着时间的推移有溃烂的趋势（27例）。单一病灶或局部受累区域类似蜂窝织炎或脓皮病报告8例。最常见的受累解剖部位是腿部（40例），其次是躯干（30例）和手臂（28例）。54%（25/46）的患者报告了体质症状，包括一些有限的皮肤受累。主要合并症包括自身免疫（12例）、其他淋巴细胞增生性疾病（5例）、内部癌（4例）和病毒性肝炎（2例）。淋巴结病变（3/42例）和骨髓受累（5/28例）不常见，但血清乳糖脱氢酶（LDH）升高55%（22/39）。在20/37的受试者中，异常的正电子发射断层扫描和/或计算机断层扫描主要突出软组织或淋巴结。疾病进展与广泛溃疡病变相关，导致27人死亡，包括噬血细胞综合征并发症(4)和脑神经系统受累(3)。诊断后的中位生存时间为31个月。皮肤活检从页样到纯真皮或由中等大小淋巴细胞组成的泛膜浸润，组织证据显示细胞毒性。最常见的免疫表型为CD3+/CD4−/CD5−/CD8−/BF1−/&ggr；-M1+/TIA-1+/颗粒酶- b +/CD45RA−/CD7−，eb病毒阳性4例。这是迄今为止最大的关于皮肤的研究。t细胞淋巴瘤表现出多种临床和病理表现，预后不佳。
We reviewed our multicenter experience with gamma-delta (&ggr;&dgr;) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3+/CD4−/CD5−/CD8−/BF1−/&ggr;-M1+/TIA-1+/granzyme-B+/CD45RA−/CD7−, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous &ggr;&dgr; T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.
DOI：
10.1182/asheducation-2009.1.523
发表时间：
2009
期刊：
Hematology. American Society of Hematology. Education Program
影响因子：
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作者：
Jaffe ES
通讯作者：
Jaffe ES