[与骨髓质疏松性瘤和慢性骨髓单细胞白血病相关的免疫障碍表现]
Vincent Jachiet1, Jérôme Hadjadj1, Lin-Pierre Zhao2
1Sorbonne université, Assistance publique-Hôpitaux de Paris, centre hospitalo-universitaire Saint-Antoine, service de médecine interne et Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France.
Bulletin du cancer
|July 6, 2023
概括
系统性炎症性疾病发生在四分之一的骨髓质疏松症候群患者中. 用诸如阿扎西提丁之类的药物向底层克隆显示出管理这些具有挑战性的条件的前景.
科学领域:
- 血液学 血液学 血液学
- 类风湿病学 类风湿病学
- 在瘤学瘤学.
背景情况:
- 系统性炎症性或自身免疫性疾病 (SIAD) 影响高达25%的骨髓发育综合征 (MDS) 或慢性骨髓单细胞白血病 (CMML) 患者.
- 在MDS/CMML中,SIAD具有广泛的范围,从无症状异常到明显的系统性疾病.
- 病理生理学联系正在出现,特别是维克萨斯综合征 (UBA1突变) 和切割性骨髓形成症.
研究的目的:
- 审查MDS/CMML中SIAD的临床谱和病理生理基础.
- 讨论这些患者SIAD管理的挑战,包括皮质类固醇依赖和常规免疫抑制剂的局限性.
- 突出针对病态克隆的新兴治疗策略.
主要方法:
- 关于MDS/CMML中SIAD理解的最新进展的文献综述.
- 对关联炎症和髓状血病的分子机制的分析.
- 评估当前和新的治疗方法.
主要成果:
- 在MDS中,SIAD的存在不会显著影响整体存活率或AML转变风险.
- 在MDS/CMML中治疗SIAD是具有挑战性的,原因是皮质类固醇的依赖性和标准免疫抑制剂的耐受性/有效性差.
- 最近的数据表明脱甲基剂,特别是阿扎丁,是一个有前途的战略.
结论:
- SIAD是MDS/CMML中具有复杂管理需求的显著并发症.
- 用像阿扎西提丁这样的药物准潜在的骨髓克隆提供了一个新的治疗途径.
- 需要进一步研究炎症和骨髓瘤恶性瘤之间的相互作用.
相关概念视频
01:27Disorders of Leukocytes
984
Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
984
01:24Chronic Kidney Disease II: Clinical Manifestations
36
Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
36
01:15Differentiation of Common Myeloid Progenitor Cells
3.2K
Common myeloid progenitors (CMPs) are oligopotent cells that can differentiate into granulocytes and macrophages. Granulocytes and macrophages are essential for protecting the body against bacterial, viral, or fungal infections. They migrate from the bone marrow into the circulating blood to reach specific tissue sites where they differentiate and help in immune surveillance. However, they survive only for a few days and must be continuously made available to the organism to maintain a robust...
3.2K
01:17Chronic Bowel Disorders: Introduction
489
Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
489