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皮下脂膜炎样T细胞淋巴瘤——5例病例回顾

Subcutaneous panniculitic T-cell lymphoma-review of five cases.

作者信息

Ng T G, Ayadurai K, Gangaram H B, Hussein S H

机构信息

Department of Dermatology, Hospital Kuala Lumpur, Jalan Pahang, 50586 Kuala Lumpur.

出版信息

Med J Malaysia. 2006 Dec;61(5):586-91.

Abstract

Subcutaneous panniculitic T-cell lymphoma (SPTL) is a rare variant of cutaneous T-cell lymphoma where lymphoma cells infiltrate preferentially into subcutaneous tissue. Five cases of SPTL were seen during the period from 2001-2004 at the Department of Dermatology, Hospital Kuala Lumpur. All five presented with multiple subcutaneous nodules on the face, trunk and limbs of one week to six months duration with associated fever and loss of weight. Physical examination showed multiple tender, erythematous indurated plaques and subcutaneous nodules on their face, trunk and limbs. One patient also presented with unhealing ulcerated nodules. Two patients had hepatosplenomegaly and one hepatomegaly. Two patients had pancytopaenia while the other three had leucopaenia. One patient had deranged liver function. Out of the five patients, three had bone marrow examination with haemophaegocytosis in two and one hypocellular marrow. Skin biopsy of all patients showed infiltration with atypical lymphoid cells in the upper dermis and subcutaneous fat. These neoplastic cells showed positivity for CD3 and CD30 in three patients with CD8, TIA-1 and LCA (Leucocyte common antigen) being positive in one patient. One patient treated with prednisolone and subcutaneous Roferon 3Mu three times a week since 2001 was in remission. Two patients who were planned for chemotherapy had deteriorated rapidly and succumbed to septicaemia from pancytopaenia. Subcutaneous panniculitic T-cell lymphoma has been reported to show two distinct clinical presentations. The first is characterized by an indolent course with good prognosis and the second with rapid clinical deterioration, haemophaegocytosis and death. Both presentations were seen in our five patients seem to demonstrate these two subtypes of SPTL.

摘要

皮下脂膜炎样T细胞淋巴瘤(SPTL)是皮肤T细胞淋巴瘤的一种罕见变体,其中淋巴瘤细胞优先浸润到皮下组织。2001年至2004年期间,吉隆坡医院皮肤科共诊治了5例SPTL。所有5例患者均表现为面部、躯干和四肢出现多个皮下结节,病程为1周-6个月,伴有发热和体重减轻。体格检查发现面部、躯干和四肢有多个压痛性、红斑性硬结斑块及皮下结节。1例患者还出现不愈合的溃疡性结节。2例患者有肝脾肿大,1例有肝肿大。2例患者全血细胞减少,另外3例白细胞减少。1例患者肝功能紊乱。5例患者中,3例进行了骨髓检查,2例有噬血细胞现象,1例骨髓细胞减少。所有患者的皮肤活检均显示真皮上部和皮下脂肪有非典型淋巴细胞浸润。这些肿瘤细胞在3例患者中CD3和CD30呈阳性,1例患者CD8、TIA-1和LCA(白细胞共同抗原)呈阳性。1例自2001年起接受泼尼松龙和皮下注射干扰素3Mu每周3次治疗的患者病情缓解。2例计划进行化疗的患者病情迅速恶化,死于全血细胞减少引起的败血症。据报道,皮下脂膜炎样T细胞淋巴瘤有两种不同的临床表现。第一种表现为病程缓慢,预后良好;第二种表现为临床迅速恶化、噬血细胞现象和死亡。我们的5例患者似乎都表现出了这两种SPTL亚型。

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