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具有皮肤表现的前B表型淋巴母细胞淋巴瘤。

Lymphoblastic lymphoma of the pre-B phenotype with cutaneous presentation.

作者信息

Schmitt I M, Manente L, Di Matteo A, Felici F, Giangiacomi M, Chimenti S

机构信息

Department of Dermatology, University of L'Aquila, Italy.

出版信息

Dermatology. 1997;195(3):289-92. doi: 10.1159/000245966.

DOI:10.1159/000245966
PMID:9407185
Abstract

Lymphoblastic lymphoma (LBL) is a neoplasm of lymphoid precursors presenting usually as acute leukemia with bone marrow and peripheral blood involvement. Primary cutaneous involvement of LBL with a pre-B phenotype has to be considered an extremely uncommon occurrence, accounting for less than 1% of all non-Hodgkin lymphomas. A child with an LBL involving a single cutaneous manifestation of 6 months duration is presented. At the time of presentation, the lesion consisted of a rapidly enlarging deeply infiltrated tumor on the upper arm. Immunophenotypic analysis performed an paraffin-embedded and frozen tissue sections revealed 2 pre-B phenotype of the tumor cells. Similar results were obtained from lymph node and bone marrow biopsy specimens. After 26 months of polychemotherapy, the patient is currently in complete remission. We wish to add this case to the current literature of LBL with cutaneous involvement, emphasizing the importance of a correct diagnosis and the excellent response to the therapeutic regimen.

摘要

淋巴母细胞淋巴瘤(LBL)是一种淋巴样前体细胞的肿瘤,通常表现为伴有骨髓和外周血受累的急性白血病。具有前B表型的LBL原发性皮肤受累必须被视为极其罕见的情况,占所有非霍奇金淋巴瘤的比例不到1%。本文报告一名患有LBL的儿童,其单个皮肤表现持续6个月。就诊时,病变表现为上臂迅速增大的深部浸润性肿瘤。对石蜡包埋和冷冻组织切片进行的免疫表型分析显示肿瘤细胞具有2种前B表型。淋巴结和骨髓活检标本也得到了类似结果。经过26个月的多药化疗,患者目前处于完全缓解状态。我们希望将此病例补充到目前有关LBL皮肤受累的文献中,强调正确诊断的重要性以及对治疗方案的良好反应。

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