Alessandra Mongia, Francesca Romano, Sara Ciullini Mannurita, Benedetta Peruzzi, Sara Bencini, Daniela Parrini, Laura Fasano, Alessandra Fanelli
{"title":"慢性髓性白血病中罕见的 T 淋巴细胞暴发危象与两种不同的暴发群","authors":"Alessandra Mongia, Francesca Romano, Sara Ciullini Mannurita, Benedetta Peruzzi, Sara Bencini, Daniela Parrini, Laura Fasano, Alessandra Fanelli","doi":"10.3390/hematolrep16030040","DOIUrl":null,"url":null,"abstract":"<p><p>Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by bone marrow expansion and the proliferation of one or more myeloid cell lineages, predominantly driven by the expression of the constitutively active fusion product tyrosine kinase BCR:ABL1. Rarely, CML patients directly develop a blast crisis (BC), mostly of myeloid origin. CML at blast crisis with a T-cell phenotype at diagnosis, without any prior history of CML, is extremely rare. Herein, we describe one rare CML case, in a young man showing an unusual and early T-lymphoid blastic crisis at diagnosis, as the first onset of a previously unknown CML. The multidisciplinary collaboration between laboratorians and clinicians for the diagnosis and management of this atypical case was crucial in outlining both a targeted pharmacological treatment and a successful hematopoietic stem cell transplantation.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"16 3","pages":"413-420"},"PeriodicalIF":1.1000,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11431131/pdf/","citationCount":"0","resultStr":"{\"title\":\"A Rare Onset of T-Lymphoid Blast Crisis in Chronic Myeloid Leukemia with Two Distinct Blast Populations.\",\"authors\":\"Alessandra Mongia, Francesca Romano, Sara Ciullini Mannurita, Benedetta Peruzzi, Sara Bencini, Daniela Parrini, Laura Fasano, Alessandra Fanelli\",\"doi\":\"10.3390/hematolrep16030040\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by bone marrow expansion and the proliferation of one or more myeloid cell lineages, predominantly driven by the expression of the constitutively active fusion product tyrosine kinase BCR:ABL1. Rarely, CML patients directly develop a blast crisis (BC), mostly of myeloid origin. CML at blast crisis with a T-cell phenotype at diagnosis, without any prior history of CML, is extremely rare. Herein, we describe one rare CML case, in a young man showing an unusual and early T-lymphoid blastic crisis at diagnosis, as the first onset of a previously unknown CML. The multidisciplinary collaboration between laboratorians and clinicians for the diagnosis and management of this atypical case was crucial in outlining both a targeted pharmacological treatment and a successful hematopoietic stem cell transplantation.</p>\",\"PeriodicalId\":12829,\"journal\":{\"name\":\"Hematology Reports\",\"volume\":\"16 3\",\"pages\":\"413-420\"},\"PeriodicalIF\":1.1000,\"publicationDate\":\"2024-06-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11431131/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hematology Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.3390/hematolrep16030040\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematology Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3390/hematolrep16030040","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
慢性髓性白血病(CML)是一种骨髓增生性肿瘤,其特点是骨髓增生和一个或多个髓系细胞增殖,主要由组成型活性融合产物酪氨酸激酶 BCR:ABL1 的表达驱动。极少数情况下,CML 患者会直接出现鼓风危象 (BC),多数为髓系细胞来源。诊断时具有 T 细胞表型的爆发危象 CML,且之前没有任何 CML 病史,这种情况极为罕见。在此,我们描述了一个罕见的 CML 病例,患者是一名年轻男性,诊断时表现出不寻常的早期 T 淋巴细胞暴发性危象,是之前未知的 CML 首次发病。在诊断和处理这一非典型病例的过程中,实验室医生和临床医生之间的多学科合作对于制定有针对性的药物治疗方案和成功进行造血干细胞移植至关重要。
A Rare Onset of T-Lymphoid Blast Crisis in Chronic Myeloid Leukemia with Two Distinct Blast Populations.
Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by bone marrow expansion and the proliferation of one or more myeloid cell lineages, predominantly driven by the expression of the constitutively active fusion product tyrosine kinase BCR:ABL1. Rarely, CML patients directly develop a blast crisis (BC), mostly of myeloid origin. CML at blast crisis with a T-cell phenotype at diagnosis, without any prior history of CML, is extremely rare. Herein, we describe one rare CML case, in a young man showing an unusual and early T-lymphoid blastic crisis at diagnosis, as the first onset of a previously unknown CML. The multidisciplinary collaboration between laboratorians and clinicians for the diagnosis and management of this atypical case was crucial in outlining both a targeted pharmacological treatment and a successful hematopoietic stem cell transplantation.