Prolonged Survival of a 79-Year Old Man with Acute Myeloid Leukemia M2, Normal Karyotype, NPM1 and FLT3-ITD Mutations, WBC 33.7 × 109/L, and Involving only Granulocyte-Macrophage Line on 53 Cycles of Low-Dose Cytarabine

P. Lemež, H. Dignum, M. Ganczakowski, L. Chiecchio, R. Ayto, K. Baker, S. Singh, T. Cranfield, G. Matthias, C. James, R. Corser
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Abstract

The prognosis of older patients with de novo Acute Myeloid Leukemia (AML) is usually dismal. Palliative therapy with LDAC is one of the treatment options with a median survival of less than one year. Several reported older cases with AML with a survival of 25-51 months on therapy with LDAC lack details of the AML type, clinical characteristics, and treatment. This case report describes a 79-year old man with AML M2, normal karyotype, leukocytosis 33.7 x 10 9 /L, and involving only Granulocyte-Macrophage Line (GM-AML) who survived 84 months on 53 repeated cycles of LDAC, the longest described survival on LDAC. His leukemic cells exhibited Nucleophosmin 1 ( NPM1 ) mutation and Fms-Like Tyrosine-kinase 3 gene ( FLT3 ) Internal Tandem Duplication (ITD) with a high FLT3 -ITD to FLT3 WT allelic ratio, typical immunophenotype, morphology and no dysplastic features. We propose that older patients with de novo GM-AML with these characteristics may benefit from prolonged LDAC therapy. Changes; EMD: Erythroblastic and/or Megakaryocytic Dysplasia; BM: Bone Marrow; FBC: Full Blood Cell count; FLT3- ITD: Fms-like Tyrosine kinase-3 gene ( FLT3 ) Internal Tandem Duplication (ITD); FLT3 WT: FLT3 Wild Type; GM-AML: AML involving only cells of Granulocytic-Macrophage line; CR: Complete Remission; HC: Hydroxycarbamide; LDAC: Low-Dose Cytarabine; NK: normal karyotype; NPM1 : Nucleophosmin 1 gene; PML/RARA : Fusion gene of Promyelocytic Leukemia gene/Retinoic Acid Receptor-Alpha gene; PS: Performance Status; RBC: Red Blood Cells; SICT: Standard-Dose Induction Chemotherapy; WBC: White Blood Cells
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79岁急性髓系白血病患者M2,核型正常,NPM1和FLT3-ITD突变,WBC 33.7 × 109/L,仅涉及粒细胞-巨噬细胞系,低剂量阿糖胞苷53周期延长生存期
老年新发急性髓细胞白血病(AML)患者的预后通常是令人沮丧的。LDAC的姑息治疗是中位生存期不到一年的治疗选择之一。一些报告的老年AML患者在接受LDAC治疗后存活25-51个月,缺乏AML类型、临床特征和治疗的详细信息。本病例报告描述了一名79岁男性,患有AML M2,核型正常,白细胞增多症33.7 x 10 9/L,仅涉及粒细胞巨噬细胞系(GM-AML),他在53个重复周期的LDAC中存活了84个月,是LDAC中所描述的存活时间最长的。他的白血病细胞表现出核磷酸蛋白1(NPM1)突变和Fms样酪氨酸激酶3基因(FLT3)内部串联重复(ITD),具有高的FLT3-ITD与FLT3 WT等位基因比例、典型的免疫表型、形态和无发育异常特征。我们提出,具有这些特征的新发GM-AML老年患者可能受益于长期LDAC治疗。变更;EMD:红细胞和/或巨核细胞发育不良;BM:骨髓;FBC:全血细胞计数;FLT3-ITD:Fms样酪氨酸激酶-3基因(FLT3)内部串联复制(ITD);FLT3 WT:FLT3野生型;GM-AML:AML仅涉及粒细胞巨噬细胞系的细胞;CR:完全缓解;HC:羟基脲;LDAC:低剂量阿糖胞苷;NK:正常核型;NPM1:核磷酸蛋白1基因;PML/RARA:早幼粒细胞白血病基因/维甲酸受体α基因的融合基因;PS:性能状态;RBC:红细胞;SICT:标准剂量诱导化疗;WBC:白细胞
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