Division of Cancer Medicine, Medical Oncology Fellowship, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Leuk Lymphoma. 2021 Dec;62(14):3402-3410. doi: 10.1080/10428194.2021.1961235. Epub 2021 Aug 12.
Myeloid sarcoma (MS) in the setting of concomitant medullary AML is relatively well described, while much less is known about patients presenting with MS with <20% bone marrow blasts. We conducted a retrospective analysis of 56 patients with MS with <20% marrow blasts seen at MD Anderson between 2005 and 2020. The prevalence of MS without medullary AML was 1.4% among all newly diagnosed AML patients. The majority (75%) of patients had a single known anatomic site involved, with the skin (34%) being the most frequent. The most common histologic subtype was monocytic, and 11% of patients had a known history of an antecedent hematologic disorder. The majority of patients (70%) received frontline intensive chemotherapy induction, with 75% of those evaluable attaining complete or partial responses. The median overall survival (OS) and event-free survival (EFS) were 3.41 and 3.07 years, respectively. Patients with bone marrow blasts of ≥5% or medullary relapse had inferior outcomes, while age (>60 years) was not associated with outcomes. There was a suggestion that patients with isolated leukemia cutis may have had better outcomes compared to patients with other organ involvement, but this did not reach statistical significance. Most patients who had cytogenetic analysis had a diploid karyotype within their MS and bone marrow pathway mutations were enriched in MS at diagnosis, and at time of medullary relapse. Our study provides a large dataset summarizing the clinical and molecular analysis of patients with MS with <20% BM blasts and suggests that monitoring for medullary leukemia is important for early detection of relapse.
在同时伴有骨髓性急性髓细胞白血病（AML）的情况下，骨髓性肉瘤（MS）的描述相对较为详细，而对于骨髓性原始细胞 <20%的 MS 患者的了解则要少得多。我们对 2005 年至 2020 年间在 MD 安德森癌症中心就诊的 56 例骨髓性原始细胞 <20%的 MS 患者进行了回顾性分析。在所有新诊断的 AML 患者中，无骨髓性 AML 的 MS 患病率为 1.4%。大多数（75%）患者仅存在一个已知的解剖部位受累，其中皮肤（34%）是最常见的受累部位。最常见的组织学亚型是单核细胞型，11%的患者有已知的前驱血液病病史。大多数患者（70%）接受了一线强化化疗诱导，其中 75%的可评估患者获得了完全或部分缓解。中位总生存期（OS）和无事件生存期（EFS）分别为 3.41 年和 3.07 年。骨髓性原始细胞≥5%或骨髓性复发的患者预后较差，而年龄（>60 岁）与预后无关。有迹象表明，与其他器官受累的患者相比，孤立性白血病皮肤的患者可能有更好的结局，但这并未达到统计学意义。大多数接受细胞遗传学分析的患者在 MS 和骨髓途径中具有二倍体核型，在 MS 诊断时和骨髓性复发时，MS 中存在基因突变。我们的研究提供了一个大型数据集，总结了骨髓性原始细胞 <20%的 MS 患者的临床和分子分析，表明监测骨髓性白血病对于早期发现复发非常重要。
