{"title":"鲁索利替尼治疗epr重排急性淋巴细胞白血病:病例报告和文献复习。","authors":"Yiling Dai, Yuan Ai, Shuwen Sun, Maoting Tang, Jinrong Li, Ju Gao, Mingyan Jiang","doi":"10.1007/s00277-025-06596-4","DOIUrl":null,"url":null,"abstract":"<p><p>Acute lymphoblastic leukemia (ALL) with erythropoietin receptor (EPOR) gene rearrangement is rarely reported. These patients test negative for the Philadelphia chromosome but exhibit a gene expression profile similar to that of Philadelphia chromosome-positive acute lymphoblastic leukemia, leading to their designation as Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL). EPOR-rearranged ALL exhibits aggressive clinical course and dismal prognosis with a short remission duration and high level of minimal residual disease (MRD). The addition of tyrosine kinase inhibitors or Janus kinases inhibitors to conventional chemotherapy is an effective treatment approach for patients with Ph-like ALL. Here, we present two pediatric cases of Ph-like ALL with EPOR rearrangement that were treated with ruxolitinib and provide a literature review to assess the effectiveness of this treatment.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":" ","pages":""},"PeriodicalIF":2.4000,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Treatment of EPOR-rearranged acute lymphoblastic leukemia with ruxolitinib: case reports and review of the literature.\",\"authors\":\"Yiling Dai, Yuan Ai, Shuwen Sun, Maoting Tang, Jinrong Li, Ju Gao, Mingyan Jiang\",\"doi\":\"10.1007/s00277-025-06596-4\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Acute lymphoblastic leukemia (ALL) with erythropoietin receptor (EPOR) gene rearrangement is rarely reported. These patients test negative for the Philadelphia chromosome but exhibit a gene expression profile similar to that of Philadelphia chromosome-positive acute lymphoblastic leukemia, leading to their designation as Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL). EPOR-rearranged ALL exhibits aggressive clinical course and dismal prognosis with a short remission duration and high level of minimal residual disease (MRD). The addition of tyrosine kinase inhibitors or Janus kinases inhibitors to conventional chemotherapy is an effective treatment approach for patients with Ph-like ALL. Here, we present two pediatric cases of Ph-like ALL with EPOR rearrangement that were treated with ruxolitinib and provide a literature review to assess the effectiveness of this treatment.</p>\",\"PeriodicalId\":8068,\"journal\":{\"name\":\"Annals of Hematology\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":2.4000,\"publicationDate\":\"2025-09-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Annals of Hematology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00277-025-06596-4\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Hematology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00277-025-06596-4","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Treatment of EPOR-rearranged acute lymphoblastic leukemia with ruxolitinib: case reports and review of the literature.
Acute lymphoblastic leukemia (ALL) with erythropoietin receptor (EPOR) gene rearrangement is rarely reported. These patients test negative for the Philadelphia chromosome but exhibit a gene expression profile similar to that of Philadelphia chromosome-positive acute lymphoblastic leukemia, leading to their designation as Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL). EPOR-rearranged ALL exhibits aggressive clinical course and dismal prognosis with a short remission duration and high level of minimal residual disease (MRD). The addition of tyrosine kinase inhibitors or Janus kinases inhibitors to conventional chemotherapy is an effective treatment approach for patients with Ph-like ALL. Here, we present two pediatric cases of Ph-like ALL with EPOR rearrangement that were treated with ruxolitinib and provide a literature review to assess the effectiveness of this treatment.
期刊介绍:
Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.