骨髓坏死综合征:研究发展近况

Current status of research on bone marrow necrosis syndrome

背景:骨髓坏死早期临床特征无特异性,医生对该病认识不足,常常误诊和漏诊。目的:提高对骨髓坏死综合征的认识和警惕性,进一步探讨其临床、血液学特点及发病机制、治疗等。方法:以"Bone marrow necrosis,Hematologic neoplasms,Solid tumor,Bone marrow puncture,Bone marrow pathology"为英文检索词,以"骨髓坏死,血液系统肿瘤,实体肿瘤,骨髓穿刺,骨髓病理"为中文检索词,由第一作者检索Pub Med、万方数据库1941至2016年骨髓坏死相关文献,经过筛选最终获得43篇文献进行综述。结果与结论:骨髓坏死为临床少见多种疾病所致的并发症,临床以骨痛、发热、幼红-幼粒细胞贫血、骨髓穿刺和/或骨髓活检显示坏死为特征,发病机制复杂,但是目前对其仍知之甚少,值得进一步研究。目前对骨髓坏死尚无较好的治疗方法,预后较差。早期发现、早期诊断、早期积极治疗原发病对骨髓坏死的预后尤为重要。随着对疾病认识的提高,骨髓细胞学、基因学、MRI等检测手段的应用及造血干细胞移植等治疗措施的发展,骨髓坏死患者有望获得更好的预后。

BACKGROUND:Bone marrow necrosis has unspecific clinical features,which is often misdiagnosed or missed due to a lack of the knowledge of the disease.OBJECTIVE:To improve the awareness and vigilance to bone marrow necrosis,and to further explore the clinical manifestations,hematological characteristics,pathogenesis and treatment of bone marrow necrosis.METHODS:The“bone marrow necrosis,hematologic neoplasms,solid tumor,bone marrow puncture,bone marrow pathology”in Chinese and English served as the search terms to search articles related to bone marrow necrosis in PubMed and Wanfang databases,published from1941to2016.Totally43articles were selected for review.RESULTS AND CONCLUSION:Bone marrow necrosis is a rare complication caused by various diseases,clinically characterized by bone pain,fever,anemia,and nucleated red cells and immature neutrophilic leukocytes in the blood smear.Bone marrow aspiration and/or bone marrow biopsy show(s)necrotic features.Its pathogenesis is complex,and it is still poorly understood and needs further research.There is no good treatment for bone marrow necrosis,and the prognosis is poor.Early correct diagnosis and etiological treatment are crucial for the prognosis of bone marrow necrosis.With the improvement of disease awareness,bone marrow cytology,genetics,MRI and hematopoietic stem cell transplantation,bone marrow necrosis is expected to get a better prognosis.