原发性皮肤淋巴瘤(primarycutaneouslymphoma,PCL)是一类结外非霍奇金淋巴瘤,具有独特的临床表现和组织病理学特征。2005年世界卫生组织-欧洲癌症研究和治疗组织(World Health Organization-European Organization for Research and Tre...原发性皮肤淋巴瘤(primarycutaneouslymphoma,PCL)是一类结外非霍奇金淋巴瘤,具有独特的临床表现和组织病理学特征。2005年世界卫生组织-欧洲癌症研究和治疗组织(World Health Organization-European Organization for Research and Treatment of Cancer,WHO-EORTC)发表的PCL分类是该病的重要诊断标准和分类依据。随着研究进展,2018年WHO-EORTC更新了PCL分类,新增了慢性活动性EB病毒感染、原发性皮肤肢端CD8^+T细胞淋巴瘤、EB病毒阳性皮肤黏膜溃疡3种PCL类型,并对部分PCL的亚型进行了修订,其中涉及淋巴瘤样丘疹病、原发性皮肤边缘区淋巴瘤、原发性皮肤CD4^+小/中T细胞淋巴组织增殖性疾病等亚型。本文对PCL WHO-EORTC分类的最新进展进行解读.展开更多
The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed ...The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed at the goal of achieving and sustaining remission. Increasingly, low dose total skin electron beam therapy (TSEBT) is being utilized as a skin directed component in combination therapy for advanced stage CTCL. Researchers are seeking to better define the utility of low dose TSEBT as a method of debulking skin disease while simultaneously treating other disease compartments and in combination with sustained maintenance therapies of both the skin directed and systemic varieties. Data exists showing the efficacy of low dose TSEBT in early and advanced disease. There is also data documenting prolonged treatment responses with TSEBT plus adjuvant skin directed therapies such as PUVA and topical nitrogen mustard. Emerging data examining the role of low dose TSEBT in the prestem cell transplant preparation is also promising. This brief review summarizes the utility of low dose TSEBT in multiagent treatment regimens in CTCL.展开更多
Atypical cutaneous lymphoproliferative disorder (ACLD) is a rare condition that has been associated with HIV infection. Patients with ACLD present with diffuse, erythematous and pruritic skin lesions accompanied by ge...Atypical cutaneous lymphoproliferative disorder (ACLD) is a rare condition that has been associated with HIV infection. Patients with ACLD present with diffuse, erythematous and pruritic skin lesions accompanied by generalized lymphadenopathy. The clinical characteristics of ACLD overlap most notably with several other conditions including Mycosis Fungoides/Sézary Syndrome (MF/SS), a cutaneous lymphoma of T-cell lineage. Unlike Mycosis Fungoides, the noxious infiltrates of ACLD are not monoclonal but polyclonal and consist of cytotoxic CD8+ T-cells instead of CD4+ T-cells or B-cells. Highly active antiretroviral therapy (HAART) has been reported to improve ACLD. We describe the case of a Caucasian man with longstanding HIV infection who presented with severe erythroderma. Skin and lymph node biopsies showed polyclonal CD8+ T-cell infiltrates. Gene rearrangement studies did not reveal an obvious clonal disorder. Hallmark peripheral blood findings consisting of a severe depletion of CD4+ T-lymphocytes and markedly elevated CD8+ cells provided an important diagnostic clue. Despite the purported benefits of HAART in ameliorating this disorder, erythroderma and extreme pruritus improved only after the patient began taking mycophenolate mofetil and hydroxyurea. Unfortunately, he succumbed to complications of methicillin-resistant Staphylococcus aureus septicemia. We alert readers to this rare HIV-associated condition which may mimic other benign and malignant skin conditions and briefly discuss diagnostic and therapeutic options.展开更多
为了研究基因诊断在皮肤 T 细胞淋巴瘤(CTCL)的临床应用,采用聚合酶链反应(PCR)技术,对60例皮肤淋巴细胞浸润疾患进行了 T 细胞受体(TCR)基因重排的检测。结果显示,检出TCR-β和(或)TCR-γ基因克隆重排的有:36/40例 CTCL、4/6例可疑蕈...为了研究基因诊断在皮肤 T 细胞淋巴瘤(CTCL)的临床应用,采用聚合酶链反应(PCR)技术,对60例皮肤淋巴细胞浸润疾患进行了 T 细胞受体(TCR)基因重排的检测。结果显示,检出TCR-β和(或)TCR-γ基因克隆重排的有:36/40例 CTCL、4/6例可疑蕈样肉芽肿/Sézary 综合征和1/1例淋巴瘤样丘疹病;未检出 TCR-β或γ基因克隆重排的有:2例皮肤 B 细胞淋巴瘤、1例皮肤淋巴细胞瘤、8例良性皮肤淋巴细胞浸润疾患和2例正常人皮肤。这表明:该方法在确定 CTCL 的克隆性、细胞源性以及早期诊断方面是有帮助的。展开更多
Mogamulizumab是一种定向作用于CC趋化因子受体4的重组人源化单克隆抗体,用于治疗至少接受过1次预先全身治疗后的复发性或难治性蕈样肉芽肿或Sézary综合征的成年患者。与伏立诺他相比,本品可延长患者无进展生存期。本品于2018年8...Mogamulizumab是一种定向作用于CC趋化因子受体4的重组人源化单克隆抗体,用于治疗至少接受过1次预先全身治疗后的复发性或难治性蕈样肉芽肿或Sézary综合征的成年患者。与伏立诺他相比,本品可延长患者无进展生存期。本品于2018年8月获美国食品药品监督管理局(Food and Drug Administration,FDA)批准在美国上市。本文就其作用机制、药代动力学、临床评价、不良反应、用法用量等进行综述,旨在为临床用药提供参考。展开更多
Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been show...Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been shown to result in long-term disease-free intervals, with even curative potential. Radiation is also effective as palliative treatment for the localized lesion resistant to the topic or other treatments. In the current study, we report using high dose rate (HDR) radiation treatment for a patient with resistant mycosis fungoides involving the wrist. We report a convenient treatment with an ideal radiation dose distribution, and a excellent clinical outcome.展开更多
Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and viscera...Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and visceral organs in later stage and ultimately progression to systemic lymphoma.It can result in secondary skin infection which is a frequent cause of death. At present,no curative therapy existed.Therapeutic purpose is to induce remission,reduce tumor burden and protect immune function of patients.A case of patient with advanced severe mycosis fungoides receiving CHOP plus interferon a -2a was reported here,with disease-free survival of 7 months and overall survival of over 17.0 months,and current status as well as developments of mycosis fungoides were briefly introduced.展开更多
Sezary syndrome （SS） is an erythrodermic, aggressive, cutaneous T-cell lymphoma （CTCL） characterized by a malignant T-cell clone that localizes in the blood and skin, and its diagnosis is based on clinical, histol...Sezary syndrome （SS） is an erythrodermic, aggressive, cutaneous T-cell lymphoma （CTCL） characterized by a malignant T-cell clone that localizes in the blood and skin, and its diagnosis is based on clinical, histological and biological features. Here we describe a typical case of SS.展开更多
文摘原发性皮肤淋巴瘤(primarycutaneouslymphoma,PCL)是一类结外非霍奇金淋巴瘤,具有独特的临床表现和组织病理学特征。2005年世界卫生组织-欧洲癌症研究和治疗组织(World Health Organization-European Organization for Research and Treatment of Cancer,WHO-EORTC)发表的PCL分类是该病的重要诊断标准和分类依据。随着研究进展,2018年WHO-EORTC更新了PCL分类,新增了慢性活动性EB病毒感染、原发性皮肤肢端CD8^+T细胞淋巴瘤、EB病毒阳性皮肤黏膜溃疡3种PCL类型,并对部分PCL的亚型进行了修订,其中涉及淋巴瘤样丘疹病、原发性皮肤边缘区淋巴瘤、原发性皮肤CD4^+小/中T细胞淋巴组织增殖性疾病等亚型。本文对PCL WHO-EORTC分类的最新进展进行解读.
文摘The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed at the goal of achieving and sustaining remission. Increasingly, low dose total skin electron beam therapy (TSEBT) is being utilized as a skin directed component in combination therapy for advanced stage CTCL. Researchers are seeking to better define the utility of low dose TSEBT as a method of debulking skin disease while simultaneously treating other disease compartments and in combination with sustained maintenance therapies of both the skin directed and systemic varieties. Data exists showing the efficacy of low dose TSEBT in early and advanced disease. There is also data documenting prolonged treatment responses with TSEBT plus adjuvant skin directed therapies such as PUVA and topical nitrogen mustard. Emerging data examining the role of low dose TSEBT in the prestem cell transplant preparation is also promising. This brief review summarizes the utility of low dose TSEBT in multiagent treatment regimens in CTCL.
文摘Atypical cutaneous lymphoproliferative disorder (ACLD) is a rare condition that has been associated with HIV infection. Patients with ACLD present with diffuse, erythematous and pruritic skin lesions accompanied by generalized lymphadenopathy. The clinical characteristics of ACLD overlap most notably with several other conditions including Mycosis Fungoides/Sézary Syndrome (MF/SS), a cutaneous lymphoma of T-cell lineage. Unlike Mycosis Fungoides, the noxious infiltrates of ACLD are not monoclonal but polyclonal and consist of cytotoxic CD8+ T-cells instead of CD4+ T-cells or B-cells. Highly active antiretroviral therapy (HAART) has been reported to improve ACLD. We describe the case of a Caucasian man with longstanding HIV infection who presented with severe erythroderma. Skin and lymph node biopsies showed polyclonal CD8+ T-cell infiltrates. Gene rearrangement studies did not reveal an obvious clonal disorder. Hallmark peripheral blood findings consisting of a severe depletion of CD4+ T-lymphocytes and markedly elevated CD8+ cells provided an important diagnostic clue. Despite the purported benefits of HAART in ameliorating this disorder, erythroderma and extreme pruritus improved only after the patient began taking mycophenolate mofetil and hydroxyurea. Unfortunately, he succumbed to complications of methicillin-resistant Staphylococcus aureus septicemia. We alert readers to this rare HIV-associated condition which may mimic other benign and malignant skin conditions and briefly discuss diagnostic and therapeutic options.
文摘为了研究基因诊断在皮肤 T 细胞淋巴瘤(CTCL)的临床应用,采用聚合酶链反应(PCR)技术,对60例皮肤淋巴细胞浸润疾患进行了 T 细胞受体(TCR)基因重排的检测。结果显示,检出TCR-β和(或)TCR-γ基因克隆重排的有:36/40例 CTCL、4/6例可疑蕈样肉芽肿/Sézary 综合征和1/1例淋巴瘤样丘疹病;未检出 TCR-β或γ基因克隆重排的有:2例皮肤 B 细胞淋巴瘤、1例皮肤淋巴细胞瘤、8例良性皮肤淋巴细胞浸润疾患和2例正常人皮肤。这表明:该方法在确定 CTCL 的克隆性、细胞源性以及早期诊断方面是有帮助的。
文摘Mogamulizumab是一种定向作用于CC趋化因子受体4的重组人源化单克隆抗体,用于治疗至少接受过1次预先全身治疗后的复发性或难治性蕈样肉芽肿或Sézary综合征的成年患者。与伏立诺他相比,本品可延长患者无进展生存期。本品于2018年8月获美国食品药品监督管理局(Food and Drug Administration,FDA)批准在美国上市。本文就其作用机制、药代动力学、临床评价、不良反应、用法用量等进行综述,旨在为临床用药提供参考。
文摘Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been shown to result in long-term disease-free intervals, with even curative potential. Radiation is also effective as palliative treatment for the localized lesion resistant to the topic or other treatments. In the current study, we report using high dose rate (HDR) radiation treatment for a patient with resistant mycosis fungoides involving the wrist. We report a convenient treatment with an ideal radiation dose distribution, and a excellent clinical outcome.
基金supported by the Jiangsu province Natural Science Foundation of China(No.BK2010579)
文摘Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and visceral organs in later stage and ultimately progression to systemic lymphoma.It can result in secondary skin infection which is a frequent cause of death. At present,no curative therapy existed.Therapeutic purpose is to induce remission,reduce tumor burden and protect immune function of patients.A case of patient with advanced severe mycosis fungoides receiving CHOP plus interferon a -2a was reported here,with disease-free survival of 7 months and overall survival of over 17.0 months,and current status as well as developments of mycosis fungoides were briefly introduced.
文摘Sezary syndrome （SS） is an erythrodermic, aggressive, cutaneous T-cell lymphoma （CTCL） characterized by a malignant T-cell clone that localizes in the blood and skin, and its diagnosis is based on clinical, histological and biological features. Here we describe a typical case of SS.
