Versiti Blood Research Institute and Medical College of Wisconsin, Milwaukee, WI.
Blood. 2023 Jun 8;141(23):2797-2812. doi: 10.1182/blood.2022017152.
Chronic or recurrent episodes of acute inflammation cause attrition of normal hematopoietic stem cells (HSCs) that can lead to hematopoietic failure but they drive progression in myeloid malignancies and their precursor clonal hematopoiesis. Mechanistic parallels exist between hematopoiesis in chronic inflammation and the continuously increased proliferation of myeloid malignancies, particularly myeloproliferative neoplasms (MPNs). The ability to enter dormancy, a state of deep quiescence characterized by low oxidative phosphorylation, low glycolysis, reduced protein synthesis, and increased autophagy is central to the preservation of long-term HSCs and likely MPN SCs. The metabolic features of dormancy resemble those of diapause, a state of arrested embryonic development triggered by adverse environmental conditions. To outcompete their normal counterparts in the inflammatory MPN environment, MPN SCs co-opt mechanisms used by HSCs to avoid exhaustion, including signal attenuation by negative regulators, insulation from activating cytokine signals, anti-inflammatory signaling, and epigenetic reprogramming. We propose that new therapeutic strategies may be derived from conceptualizing myeloid malignancies as an ecosystem out of balance, in which residual normal and malignant hematopoietic cells interact in multiple ways, only few of which have been characterized in detail. Disrupting MPN SC insulation to overcome dormancy, interfering with aberrant cytokine circuits that favor MPN cells, and directly boosting residual normal HSCs are potential strategies to tip the balance in favor of normal hematopoiesis. Although eradicating the malignant cell clones remains the goal of therapy, rebalancing the ecosystem may be a more attainable objective in the short term.
慢性或复发性急性炎症发作会导致正常造血干细胞(HSCs)耗竭,从而导致造血衰竭,但也会促使髓系恶性肿瘤及其前体细胞克隆性造血进展。慢性炎症中的造血与髓系恶性肿瘤(尤其是骨髓增殖性肿瘤)的持续过度增殖之间存在机制相似性。进入休眠状态的能力是保存长期 HSCs 并可能保存骨髓增殖性肿瘤干细胞(MPN SCs)的关键,休眠状态是一种深度静止的状态,其特征是氧化磷酸化水平低、糖酵解水平低、蛋白质合成减少和自噬增加。休眠的代谢特征类似于休眠,休眠是由不利的环境条件引发的胚胎发育停滞状态。为了在炎症性 MPN 环境中与正常对照竞争,MPN SC 会采用 HSCs 避免衰竭所使用的机制,包括负调节因子的信号衰减、与激活细胞因子信号、抗炎信号和表观遗传重编程的隔离。我们提出,新的治疗策略可能源于将髓系恶性肿瘤视为失衡的生态系统,其中残留的正常和恶性造血细胞以多种方式相互作用,其中只有少数已被详细描述。打破 MPN SC 隔离以克服休眠、干扰有利于 MPN 细胞的异常细胞因子回路以及直接增强残留的正常 HSCs 是有利于正常造血的平衡的潜在策略。虽然消除恶性细胞克隆仍然是治疗的目标,但在短期内重新平衡生态系统可能是一个更可实现的目标。