A Case of Cutaneous B-cell Lymphoma Treated Successfully with MACOP-B

A case of cutaneous B-cell lymphoma successfully treated by MACOP-B therapy is described. The patient was a 43-year-old man with reddish tumors measuring 3 to 7 cm in diameter on the right cheek and the post-auricles. Histopathologically, massive infiltrations of medium-sized atypical lymphoid cells were found in the reticular dermis and subcutis. A clear zone beneath the epidermis was also detected. The atypical lymphoid cells were positive for CD19, CD20, CD22 and HLA-DR but negative for CD3, CD4, CD5, CD10, CD43, CD45RO and CDw75. The patient was treated successfully with the MACOP-B protocol from March of 1990 to May of 1990. Since April of 1990, he has been free of disease.     

Cutaneous Manifestations and Treatment Advances of Adult T-Cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell lymphoma caused by the human T lymphotropic virus type-1. The skin is affected in approximately half of ATLL patients, and skin lesions may be the first manifestation of the disease. The skin lesions of ATLL are polymorphous, and depend on the type of skin eruption, which makes it possible for doctors to predict the prognosis of the disease based on the characteristics of skin lesions. In this review article, we describe th...     

A Case of Adult T-cell Leukemia/Lymphoma (ATLL) with Angiocentric and Angiodestructive Features

This report describes a case of adult T-cell leukemia/lymphoma (ATLL) with angiocentric and angiodestructive features. The patient was a 66-year-old Japanese woman who began developing widespread skin lesions ten months prior to admission. The diagnosis of ATLL was made on the basis of her having an antibody to human T-cell lymphotropic virus type -1 (HTLV-1) and typical flower cells (ATLL cells) in peripheral blood smears. Once hospitalized, the course of her disease was very acute and severe, as is seen with angiocentric lymphoma. Based on histological features, this case was judged not to be angiocentric lymphoma; however, it may lie within the spectrum of angiocentric immunoproliferative lesions (AIL). The findings in this case strongly suggest that HTLV-1 can be a pathogenic factor in the expression of angiocentric and angiodestructive features in ATLL, as is Epstein-Barr virus (EBV) (1–4). To our knowledge, the present case is the sixth reported in the literature of lymphoma in which these features are associated with HTLV-1 infection (5–7).     

中国人皮肤型成人T细胞白血病/淋巴瘤一例报道

目的 报道1例皮肤型成人T细胞白血病/淋巴瘤(cATLL)。方法 检测皮肤损害的组织病理、直接免疫荧光和免疫表型的特点,同时应用酶联免疫吸附试验、蛋白印迹法以及聚合酶链反应分别检测患者外周血中嗜人T淋巴细胞病毒Ⅰ型(HTLV-Ⅰ)抗体和淋巴样细胞的HTLV-Ⅰ前病毒DNA。结果 此例患者的皮肤损害多形,除表现丘疹、斑块外,并发生疱壁紧张或松弛的大疱。组织病理检查示表皮下水疱,疱底真皮乳头和疱边缘处小至中等大不典型淋巴样细胞浸润。不典型淋巴样细胞示CD45⁺、CD45RO⁺,直接免疫荧光检查阴性。外周血中HTLV-Ⅰ抗体阳性;淋巴样细胞中HTLV-Ⅰ前病毒DNA阳性。患者最后死亡,病程1年10个月。结论 ATLL在我国并非极罕见,cATLL也存在,应加以警惕。     

Adult T-Cell Leukemia/Lymphoma and Cutaneous T-Cell Lymphoma Are They Related?

Comparative studies were performed on clinical and laboratory features of four patients with different types of T-cell lymphoma of the skin; adult T-cell leukemia/lymphoma (ATLL), Sézary syndrome, mycosis fungoides, and Ki-1-positive lymphoma. All neoplastic cells studied showed a helper-inducer T-cell phenotype. A Ki-1-positive lymphoma is distinct from other types of cutaneous lymphomas because of unique morphologic and phenotypic features. Clonal proliferation of lymphocytes infected by human T-cell lymphotrophic virus (HTLV)-1 distinguishes ATLL from other T-cell lymphomas of the skin, especially in the endemic area of ATLL. From the pathogenic point of view, ATLL should not be included in a group with mycosis fungoides and Sézary syndrome.     

Adult T-cell leukemia/lymphoma (ATL) –clinical, histopathological, immunological and immunohistochemical characteristics

Twenty-one patients with ATL were assessed. The predominant physical findings were lymph node and bone marrow involvement, skin involvement, hepatosplenomegaly and leukemic manifestations. The predominant histopathological findings in both skin and lymph node specimens were the diffuse medium-sized cell type and the diffuse mixed cell type. Some phenotypic discrepancy was found between the neoplastic cells in the peripheral blood, lymph nodes and skin of patients with ATL with respect to CD45RA and CD45RO, and CD7, CD29, CD25 and HLA-DR. That is, the predominant neoplastic cell phenotype was the helper T-cell, which was CD3+, CD4+, CD7+, CD25+, CD45RA+ and HLA-DR+, and CD29- and CD45RO- in peripheral blood and lymph nodes, and CD3+, CD4+, CD7+, CD29+, CD45RO+ and HLA-DR+, and CD45RA- in the skin. In other words, we have described the phenotypic heterogeneity of ATL cells and demonstrated the heterogeneity of CD45R isoform expression on ATL cells in different organs--the skin, peripheral blood and lymph nodes--of the same patient.     

A Case of Aggressive NK/T-cell Lymphoma/Leukemia with Cutaneous Involvement in Adolescence

NK/T-cell lymphoma (NKTCL) is characterized by the expression of the NK-cell antigen CD56. Non-nasal NK/T-cell lymphomas are subdivided into primary cutaneous and 4 subtypes of secondary cutaneous lymphomas; nasal type, aggressive, blastic (blastoid), and other specific NK-like cell lymphoma. Aggressive NK/T-cell lymphoma/leukemia is a rare leukemic variant of nasal type NKTCL. We herein report a rare case of aggressive NK/T-cell lymphoma/leukemia with cutaneous involvement in adolescence.     

多发性结外鼻型NK/T细胞淋巴瘤1例

患者男,60岁,藏族。全身斑块、结节及肿块6月,肿块破溃伴疼痛2月。皮损病理组织示:表皮变薄,基底层液化变性,瘤细胞在真皮及皮下脂肪层呈弥漫性浸润,瘤细胞形态不一,核深染,可见血管中心性浸润及凝固性坏死。免疫组化标记瘤细胞CD3,CD3ε,CD56,TIA-1,粒酶B均为(+),CD30约10%(+),CD8(-),CD79a(-),EBER1/2原位杂交(+)。TCR-γ重排未见明确克隆性重排条带。诊断:皮肤结外鼻型NK/T细胞淋巴瘤。     

Case of adult T-cell leukemia with pulmonary involvement presenting as nodular shadows

A 60-year-old man with a diagnosis of smoldering adult T-cell leukemia (ATL) had been treated successfully for 4 years with psoralen and ultraviolet A therapy, gamma-interferon, oral etoposide and sobuzoxane. He subsequently developed rapidly-growing skin nodules over his entire body. Chest X-ray and thoracic computed tomography showed nodular shadows in the right lower lung field and nodules in both lower lung lobes. Despite combined chemotherapy, he died. Upon autopsy, numerous nodules were found in the bilateral lower lobes; microscopically, the nodules were diffusely infiltrated by ATL cells. Our review of the published work found only two previously reported cases of ATL with pulmonary involvement manifested as nodular shadows. Herein, we present details on the third case.     

皮肤的结外鼻型NK/T细胞淋巴瘤1例及文献复习

目的报道1例皮肤结外鼻型NK/T细胞淋巴瘤,以引起临床和病理医师对此病的关注。方法通过临床病理分析结合免疫组化染色、EB病毒原位杂交及T细胞受体基因重排的PCR检测分析确诊。结果左膝内后方皮损初次活检诊断为结节性脂膜炎,1个月内再次活检示真皮和皮下脂肪内肿瘤大片坏死,瘤细胞异型性明显,血管中心性浸润和血管坏死。瘤细胞表达CD2,CD8,CD45RO,CD56,TIA-1,GranzymeB和LMP-1,EB病毒(+);未检测到TCR-γ的克隆性基因重排。诊断为皮肤的结外鼻型NK/T细胞淋巴瘤。结论皮肤的结外鼻型NK/T细胞淋巴瘤恶性度高、易误诊、预后差;诊断有赖于常规组织病理结合分子病理技术。     

Successful Combination Treatment of a Patient with Progressive Juvenile Localized Scleroderma (Morphea) Using Imatinib,Corticosteroids, and Methotrexate

We report a case of progressive juvenile localized scleroderma (JLS or morphea) treated with a combination of imatinib, corticosteroids, and methotrexate. This therapy halted the progressive skin thickening and the hand and finger joint deformity in the early stages of the disease. We conclude that imatinib used in addition to standard treatment with systemic corticosteroids and methotrexate may be of therapeutic benefit for individuals with JLS.     

以播散性带状疱疹为首发表现的血管免疫母细胞性T细胞淋巴瘤1例

患者男,68岁,右侧头部疱疹伴疼痛1周,躯干、四肢皮疹3 d。既往身体健康。体格检查:右侧头部可见成簇疱疹,部分破溃,部分已结痂。双侧颈部及双侧腋窝可触及肿大淋巴结。躯干及四肢散在红色丘疹,上有小水疱、周围绕以红晕,部分结痂。皮损组织病理示:淋巴结活检可见淋巴结结构破坏,未见明显淋巴滤泡,淋巴结内多形性细胞浸润,瘤细胞生长活跃,核分裂象易见。淋巴结免疫组织化学显示CD3广泛阳性;CD10片状阳性;BCL6片状阳性;CXCL13广泛阳性;Ki-67(70%~80%阳性);PD1广泛阳性;EBER散在阳性。     

Case of adult T-cell leukemia/lymphoma manifesting marked purpura

We present a case of a 62-year-old woman with marked purpura, first appearing on both legs, then spreading over the whole body, including the face. At presentation, the patient was thought to have Henoch-Schonlein purpura. However, a skin biopsy from a purpuric lesion revealed prominent infiltrations of atypical lymphocytes into the papillary dermis and marked extravasation of erythrocytes through the epidermis and upper dermis. Antibody to human T-lymphotropic virus type 1 (HTLV-1) was present in the serum and samples from skin lesions revealed HTLV-1 proviral DNA integration, as well as a clonal T-cell receptor Cbeta1 gene rearrangement. We therefore diagnosed this case as adult T-cell leukemia/lymphoma (ATL), and the purpuric lesions as ATL-specific. Soon after the initiation of chemotherapy, these purpuric lesions began to resolve with pigmentation.     

Successful Treatment of Molluscum Contagiosum in the Immunosuppressed Adult with Topical Injection of Streptococcal Preparation OK-432

A 37-year-old Japanese woman received an anticancer drug for 5 years following resection of mammary cancer and then developed widespread mollusca contagiosa, which we considered to be caused by immunosuppression induced by the chemotherapy. Because OK-432 (penicillin-treated and heat-treated lyophilized powder of a substrain of Streptococcus pyogenes A) was expected to be effective for immunosuppression, we tried its topical injection. The skin lesions disappeared almost completely within three months. OK-432 therapy is considered hopeful for treating viral skin diseases, even in immunosuppressed patients.     

Successful Treatment of Severe Iatrogenic Calcinosis Cutis with Intravenous Sodium Thiosulfate in a Child Affected by T-Acute Lymphoblastic Leukemia

Abstract:   Sodium thiosulfate has been successfully used to treat calcyphilaxis in adults and children, but its effect on iatrogenic calcinosis cutis secondary to extravasation of calcium solutions is less known. We describe a 5-year-old boy with acute lymphoblastic leukemia who developed severe calcinosis cutis in the right forearm and hand, and in the left leg and foot after extravasation of calcium gluconate during treatment for tumor-lysis-syndrome-related hypocalcaemia. Surgical debridement, local wound care, hyperbaric oxygen therapy, and sodium thiosulfate infusion achieved a complete healing of all lesions in an eight-month period with a short discontinuation of chemotherapy. No functional or sensitive impairment remained.     

原发皮肤的儿童结外NK/T细胞淋巴瘤-鼻外型1例及文献复习

目的报道1例原发皮肤的儿童结外NK/T细胞淋巴瘤,鼻外型,并回顾文献,学习该病的临床特征、组织病理、免疫组化及治疗预后特点,以提高临床医生对该病的认识。方法分析本例原发皮肤的儿童结外NK/T细胞淋巴瘤-鼻外型患者的临床表现、辅助检查、病理组化及治疗预后,并复习近年国内外相关文献。结果 11岁男性患儿表现为皮肤结节、斑块伴乏力,肝脾淋巴结明显肿大。实验室检查示白细胞和血小板降低,肝酶升高,乳酸脱氢酶升高,凝血时间延长,血液EBV-PCR高度复制。组织病理提示局部或弥漫大小不一淋巴细胞浸润,可见核碎裂,部分细胞核大深染。免疫组化:CD3(+)、CD20(-)、CD56(+++)、颗粒酶B(+)、T细胞胞浆内抗原-1(+)、Ki-67约30%~40%(+);EB病毒编码RNA原位杂交(+++)。诊断:原发皮肤的结外NK/T细胞淋巴瘤,鼻外型。告知病情后家属放弃治疗。结论不同于鼻型,本病的临床表现特异性不高,易误诊为脂膜炎等,多合并系统受累,确诊依靠组织病理、免疫组化、EBV病原学检验及结合临床表现。尽管采用强势化疗,本病仍预后不良。