成年患者中CRLF2阳性B细胞急性淋巴细胞白血病：单中心经验
CRLF2-Positive B-Cell Acute Lymphoblastic Leukemia in Adult Patients: A Single-Institution Experience.
作者信息
Konoplev Sergej, Lu Xinyan, Konopleva Marina, Jain Nitin, Ouyang Juan, Goswami Maitrayee, Roberts Kathryn G, Valentine Marc, Mullighan Charles G, Bueso-Ramos Carlos, Zweidler-McKay Patrick A, Jorgensen Jeffrey L, Wang Sa A
机构信息
From the Departments of Hematopathology and Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Department of Pathology, St Jude Children's Research Hospital, Memphis, TN.
出版信息
Am J Clin Pathol. 2017 Apr 1;147(4):357-363. doi: 10.1093/ajcp/aqx005.
OBJECTIVES
Cytokine receptor-like factor 2 ( CRLF2 ) rearrangement is found in approximately 50% of pediatric Ph-like B-cell acute lymphoblastic leukemia (B-ALL), and around 50% of CRLF2 + cases harbor JAK mutations. We analyzed CRLF2 expression and studied its correlation with CRLF2 rearrangement in adult patients with B-ALL.
METHODS
Multiparameter flow cytometry (MFC) was performed consecutively in 126 patients.
RESULTS
CRLF2 overexpression was detected in 30 (27%) patients, 28 (41%) of 69 patients with B-ALL not otherwise specified, 14 (21%) of 67 untreated patients, and 16 (27%) of 59 patients with relapsed B-ALL, with the highest among Hispanic patients (25/55, 45%). Of CRLF2+ cases, 21 (100%) of 21 cases showed CRLF2 rearrangement by fluorescence in situ hybridization, preferentially involving IGH@CRLF2 (15/15). The entire coding region of JAK2 was sequenced in 14 patients with CRLF2+ B-ALL, and nine (64%) were positive for JAK2 mutations.
CONCLUSIONS
MFC allows a rapid, inexpensive, and reliable detection of B-ALL with CRLF2 rearrangement that would further facilitate testing for JAK2 mutations for targetable therapy.
目的
细胞因子受体样因子2（CRLF2）重排在约50%的儿童Ph样B细胞急性淋巴细胞白血病（B-ALL）中被发现，并且约50%的CRLF2阳性病例存在JAK突变。我们分析了成人B-ALL患者中CRLF2的表达，并研究其与CRLF2重排的相关性。
方法
对126例患者连续进行多参数流式细胞术（MFC）检测。
结果
在30例（27%）患者中检测到CRLF2过表达，在69例未另行分类的B-ALL患者中有28例（41%），在67例未经治疗的患者中有14例（21%），在59例复发B-ALL患者中有16例（27%），其中西班牙裔患者中CRLF2过表达率最高（25/55，45%）。在CRLF2阳性病例中，21例中的21例（100%）通过荧光原位杂交显示CRLF2重排，优先涉及IGH@CRLF2（15/15）。对14例CRLF2阳性B-ALL患者的JAK2整个编码区进行测序，9例（64%）JAK2突变呈阳性。
结论
MFC能够快速、廉价且可靠地检测出存在CRLF2重排的B-ALL，这将进一步促进针对可靶向治疗的JAK2突变检测。
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