分子注释的骨髓增殖性肿瘤患者的MPN10评分与生存情况
MPN10 score and survival of molecularly annotated myeloproliferative neoplasm patients.
作者信息
ElNahass Yasser H, Mahmoud Hossam K, Mattar Mervat M, Fahmy Omar A, Samra Mohamed A, Abdelfattah Raafat M, ElRefaey Fatma A, Fahmy Hala M, Fathy Gamal M, Abdulgawad Ahmed, AbdelKader Mohamed, Elleithy Hend N, Gamil Mona, Talaat Mohamed, Nader Heba A, ElMetnawy Wafaa H
机构信息
a National Cancer Institute, Cairo University , Cairo , Egypt.
b Faculty of Medicine, Cairo University , Cairo , Egypt.
出版信息
Leuk Lymphoma. 2018 Apr;59(4):844-854. doi: 10.1080/10428194.2017.1365852. Epub 2017 Aug 22.
JAK2, CALR, MPL and triple-negative mutational status has a direct impact on symptom severity and disease burden assessed by MPN10 score in myeloproliferative neoplasms (MPNs). Among 93 patients; median MPN10 score was 48 (5-76) in JAK2 mutants versus 25 (4-80) in JAK2 negative (p < .001); 22.5 (4-65) in CALR mutants versus 35 (5-80) in CALR negative (p < .050) and 21 (10-48) in triple negative versus 40 (4-80) in JAK2/CALR/MPL mutants (p < .001). At three years, progression free and overall survival of JAK2-positive versus JAK2-negative patients were 62% versus 100% (p < .001); 85% versus 100% (p = .011) and were 100% versus 78% (p = .067); 100% versus 92% (p = .197) in CALR-positive versus CALR-negative patients and 100% versus 75% (p = .004); 100% versus 90% (p = .015) in triple negative versus mutant patients, respectively. MPN10 score in association with driver gene mutations can be used as a predictor of survival in MPN patients.
JAK2、CALR、MPL及三阴性突变状态对骨髓增殖性肿瘤（MPN）中通过MPN10评分评估的症状严重程度和疾病负担有直接影响。在93例患者中，JAK2突变体的MPN10评分中位数为48（5 - 76），而JAK2阴性患者为25（4 - 80）（p < 0.001）；CALR突变体为22.5（4 - 65），而CALR阴性患者为35（5 - 80）（p < 0.050）；三阴性患者为21（10 - 48），而JAK2/CALR/MPL突变体为40（4 - 80）（p < 0.001）。三年时，JAK2阳性与JAK2阴性患者的无进展生存率和总生存率分别为62%对100%（p < 0.001）；85%对100%（p = 0.011）；CALR阳性与CALR阴性患者分别为100%对78%（p = 0.067）；100%对92%（p = 0.197）；三阴性与突变患者分别为100%对75%（p = 0.004）；100%对90%（p = 0.015）。与驱动基因突变相关的MPN10评分可作为MPN患者生存的预测指标。
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