Hassan Naglaa M, Abdellateif Mona S, Radwan Enas M, Hameed Sayed Abed, Desouky Eman D El, Kamel Mahmoud M, Gameel Abdallah M
Clinical Pathology Department, National Cancer Institute, Cairo University, Cairo, Egypt.
Cancer Biology Department, National Cancer Institute, Cairo University, Cairo, Egypt.
Clin Lymphoma Myeloma Leuk. 2022 Jun;22(6):e376-e385. doi: 10.1016/j.clml.2021.12.006. Epub 2021 Dec 11.
The prognostic significance of cytokine receptor-like factor 2 (CRLF2) overexpression in pediatric B-cell precursor (BCP) acute lymphoblastic leukemia (ALL) is still controversial. We aimed to investigate the role of CRLF2 overexpression and JAK2 mutation in the diagnosis and prognosis of newly diagnosed pediatric B-ALL patients.
CRLF2 expression was assessed by real-time quantitative polymerase chain reaction (PCR) in 115 pediatric patients newly diagnosed with precursor B-ALL patients compared with 24 age- and sex-matched controls. JAK2 R683G mutation status was performed by the qBiomarker Somatic Mutation PCR Assay.
CRLF2 overexpression was identified in 21 patients (18.3%), while the JAK2 R683G mutant type was found in only in 7 patients (6.1%). There was a significant CRLF2 overexpression in patients with high initial TLC, high blast count in blood, and organomegaly (P .04, 0.005 & 0.05 respectively). No patients with CRLF2 overexpression expressed any recurrent cytogenetic translocations. 4 patients with CRLF2 overexpression showed JAK2 R683G mutation. CRLF2 levels and JAK2 R683G mutation status did not have a significant impact on either overall survival or disease-free survival.
CRLF2 expression was significantly higher in Egyptian precursor B-ALL pediatric patients. CRLF2 overexpression was associated with a number of unfavorable prognostic factors with high tumor load, but was not an adverse independent parameter in pediatric BCP-ALL patients. Some patients with CRLF2 overexpression display JAK2 mutation, which may benefit from targeted therapy by kinase inhibitors.
细胞因子受体样因子2(CRLF2)在小儿B细胞前体(BCP)急性淋巴细胞白血病(ALL)中过表达的预后意义仍存在争议。我们旨在研究CRLF2过表达和JAK2突变在新诊断的小儿B-ALL患者诊断和预后中的作用。
通过实时定量聚合酶链反应(PCR)评估115例新诊断的前体B-ALL小儿患者的CRLF2表达,并与24例年龄和性别匹配的对照进行比较。通过qBiomarker体细胞突变PCR检测法检测JAK2 R683G突变状态。
21例患者(18.3%)检测到CRLF2过表达,而仅7例患者(6.1%)发现JAK2 R683G突变型。初始TLC高、血液中原始细胞计数高和器官肿大的患者中存在显著的CRLF2过表达(分别为P<0.04、0.005和0.05)。没有CRLF2过表达的患者表现出任何复发性细胞遗传学易位。4例CRLF2过表达的患者显示JAK2 R683G突变。CRLF2水平和JAK2 R683G突变状态对总生存期或无病生存期均无显著影响。
埃及前体B-ALL小儿患者中CRLF2表达显著更高。CRLF2过表达与一些肿瘤负荷高的不良预后因素相关,但不是小儿BCP-ALL患者的不良独立参数。一些CRLF2过表达的患者表现出JAK2突变,可能从激酶抑制剂的靶向治疗中获益。