Enteropathy‐associated T‐cell lymphoma presenting as cutaneous deposits
Enteropathy‐associated T‐cell lymphoma presenting as cutaneous deposits
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肠病相关 T 细胞淋巴瘤表现为皮肤沉积物
DOI：
10.1111/bjh.14375
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发表时间：
2017
影响因子：
6.5
通讯作者：
F. Miall
中科院分区：
文献类型：
--
作者：
A. Webster;Philip Crea;M. Bamford;Roger Hew;Y. Griffin;F. Miall
一位70岁男性，表现为体重减轻、盗汗、便秘和广泛的明显皮肤病变（左）。正电子发射断层扫描-计算机断层扫描（PET-CT）显示多发性氟脱氧葡萄糖（FDG）-亲合性皮肤和小肠病变（标准化摄取值最大值22），无淋巴结或其他器官受累（右侧）。他的全血细胞计数显示轻度贫血（血红蛋白浓度128 g/l），但其他方面无异常。生化检查正常，包括正常的乳酸脱氢酶。皮肤活检显示淋巴细胞密集浸润，延伸到皮下组织。免疫组化CD 2、CD 3、CD 7、CD 8、TIA 1（T细胞胞内抗原1）和CD 56阳性。CD 5和CD 4染色少数细胞，EBER（EB病毒编码的小RNA）和CD 30阴性。T细胞受体γ基因克隆重排。分期骨髓活检显示无浸润。通过血清学和十二指肠活检排除腹腔疾病。诊断为2型肠病相关T细胞淋巴瘤（EATL）。患者接受CHOP（环磷酰胺、阿霉素、长春新碱、泼尼松龙）联合化疗。随后将治疗升级为异环磷酰胺、表阿霉素、依托泊苷/高剂量甲氨蝶呤，反应良好。计划进行高剂量治疗和自体干细胞移植。EATL是一种罕见的T细胞淋巴瘤亚群，仅占患者的5%。它是一种侵袭性淋巴瘤，预后差，皮肤受累是罕见的。
A 70-year-old male presented with weight loss, nights sweats, constipation and widespread distinct skin lesions (left). Positron emission tomography–computed tomography (PET-CT) showed multiple F-fluorodeoxyglucose (FDG)-avid skin and small bowel lesions (standardized uptake value maximum 22) without nodal or other organ involvement (right). His full blood count showed mild anaemia (haemoglobin concentration 128 g/l) but was otherwise unremarkable. Biochemical investigations were normal, including a normal lactate dehydrogenase. Skin biopsy showed a dense infiltrate of lymphoid cells extending into the subcutaneous tissue. Immunohistochemistry was positive for CD2, CD3, CD7, CD8, TIA1 (T cell intracellular antigen 1) and CD56. CD5 and CD4 stained a minority of cells with negative EBER (Epstein–Barr virus-encoded small RNA) and CD30. T-cell receptor gamma genes were clonally re-arranged. Staging bone marrow biopsy showed no infiltration. Coeliac disease was excluded by serology and duodenal biopsy. A diagnosis of type 2 enteropathy associated T-cell lymphoma (EATL) was made. He received combination chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone). Treatment was subsequently escalated to ifosphamide, epirubicin, etoposide/high dose methotrexate with good response. High dose therapy and autologous stem cell transplantation is planned. EATL is a rare subgroup of T-cell lymphoma, comprising only 5% of patients. It is an aggressive lymphoma with a poor prognosis and skin involvement at presentation is rare.