Myeloid and lymphoid neoplasm with novel complex translocation: unusual case report with T-lymphoblastic lymphoma, myeloid hyperplasia, eosinophilia, basophilia, and t(1;8;10)( (p31;q24;q11.2).
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引用次数: 0
Abstract
Myeloid and lymphoid neoplasms with eosinophilia (M/Ls-Eo) encompass heterogeneous but aggressive hematopoietic disorders triggered by fusion genes or mutations that typically lead to constitutive overexpression of tyrosine kinase. The occurrence of T-lymphoblastic lymphoma in the setting of M/Ls-Eo has been reported rarely in the literature. Herein, we present an unusual case of a 28-year-old male patient who presented with massive lymphadenopathy and T-lymphoblastic lymphoma in the lymph node occurring concurrently with myeloid hyperplasia, eosinophilia and basophilia in peripheral blood and bone marrow biopsy. The syndrome was associated with a novel complex karyotype involving der(8)t(1;8;10)(p31;q24;q11.2). The FISH study was negative for BCR::ABL1, JAK2, PDGFRA, PDGFRB, and FGFR1 rearrangements. The patient's clinical course was aggressive and resistant to multiple lines of intensive chemotherapy regimens. Therefore, he underwent allogenic stem cell transplantation with a fully matched donor. A brief review of the occurrence of T-LBL in conjunction with M/Ls-Eo neoplasm was made with a special focus on molecular aspects.
嗜酸性粒细胞和淋巴细胞肿瘤(M/Ls-Eo)包括由融合基因或突变引发的异质性侵袭性造血疾病,这些基因或突变通常会导致酪氨酸激酶的组成性过表达。文献中很少报道在M/Ls-Eo的情况下发生T淋巴细胞淋巴瘤。在此,我们介绍了一例不寻常的病例:一名 28 岁的男性患者出现大量淋巴结肿大,淋巴结中出现 T 淋巴细胞淋巴瘤,同时外周血和骨髓活检中出现骨髓增生、嗜酸性粒细胞增多和嗜碱性粒细胞增多。该综合征与新的复杂核型有关,涉及der(8)t(1;8;10)(p31;q24;q11.2)。FISH检查结果显示,BCR::ABL1、JAK2、PDGFRA、PDGFRB和FGFR1重排均为阴性。患者的临床病程具有侵袭性,对多线强化化疗方案产生耐药性。因此,他接受了完全匹配供体的异基因干细胞移植。本文简要回顾了T-LBL与M/Ls-Eo肿瘤的结合情况,并特别关注了分子方面的问题。
期刊介绍:
The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system.
The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases.
The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.