在HbSS中急性脊髓封存:来自牙买加出生队列的观察
Graham Serjeant1, Karlene Mason2, Ian Hambleton3
1Sickle Cell Trust (Jamaica), The Sickle Cell Trust, Kingston, Jamaica grserjeant@gmail.com.
Archives of disease in childhood
|November 2, 2023
概括
在同卵性状细胞病 (HbSS) 中,急性脊髓封存 (ASS) 影响了35%的患者,导致显著的发病率和死亡率. 较高的胎儿血红蛋白水平可能会对ASS提供保护,ASS是一种与凝结酶负型葡萄球菌相关的疾病.
科学领域:
- 血液学 血液学 血液学
- 儿科 儿科 儿科
- 遗传学 是一个遗传学.
背景情况:
- 同卵性状细胞疾病 (HbSS) 是一种具有重大健康影响的遗传血液疾病.
- 急性脊髓封存 (ASS) 是HbSS的一个严重并发症,特别是在幼儿时期.
- 了解ASS的患病率和结果对于管理HbSS患者至关重要.
研究的目的:
- 为了记录患病率,临床特征,血液学和急性脊髓封存 (ASS) 的结果在同卵性状细胞疾病 (HbSS) 患者中.
主要方法:
- 从出生开始进行了前性队列研究.
- 从1973年至1981年间发现的311例HBSS病例中收集了数据.
- 长期随访和患者护理重点是ASS事件.
主要成果:
- 在105名患者 (35%的队列) 中发生了183次ASS发作,首次发作的中位数年龄为1.07岁.
- 在ASS期间,血红蛋白水平显著下降,而网状细胞和核细胞增加.
- 切除术与没有死亡相关,而保守治疗的死亡率为6%;在某些情况下,确诊了凝结酶负型葡萄球菌.
结论:
- ASS是HbSS患者发病率和死亡率的主要原因之一,特别是在发展中国家.
- ASS可能是对各种风险因素的非特异性反应,包括细菌感染,如凝结酶阴性葡萄球菌.
- 更高的胎儿血红蛋白水平与ASS的敏感性降低和晚期发病有关.
相关概念视频
Multipotency of Hematopoietic Stem Cells
The hematopoietic stem cells or HSCs are multipotent, meaning they can differentiate and give rise to all blood and immune cells. HSCs are maintained in the quiescent stage until an external stimulus initiates their differentiation. The multipotent HSCs exist as two heterogeneous populations, long-term repopulating cells (LTRC) and short-term repopulating cells (STRC). The two HSC populations have different surface markers or receptors and are classified based on quiescence and long-term...
Bone Marrow Sampling and Transplants
Bone marrow transplant is a potential cure for several diseases, including cancer and specific genetic disorders. Notably, this procedure is applicable for patients suffering from aplastic anemia, certain types of leukemia, severe combined immunodeficiency disease (SCID), Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma, thalassemia, sickle-cell disease, and certain cancers.
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy the...
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy the...


