[第503个病例:单克隆IgM免疫球蛋白血症,重度贫血与反复发烧]
Z X Zhu1, Q Wang1, J L Zhuang1
1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Zhonghua nei ke za zhi
|November 7, 2023
概括
易布鲁替尼有效地治疗了沃尔登斯特罗姆巨型球蛋白血症 (WM) 患者的严重贫血. 这种治疗导致了正常的血红蛋白水平和稳定的WM缓解,没有感染.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 免疫学 免疫学 免疫学
背景情况:
- 沃尔登斯特罗姆巨型球蛋白血症 (WM) 是一种罕见的B细胞淋巴增殖性疾病.
- 患者经常经历贫血和治疗耐药性的并发症.
- 自身免疫性溶血性贫血和无塑性危机可能会使WM管理复杂化.
研究的目的:
- 评估ibrutinib在治疗与Waldenström巨型球蛋白血症相关的严重贫血中的疗效.
- 评估ibrutinib对WM疾病状态和患者报告的症状的影响.
主要方法:
- 一个42岁的女性被诊断患有沃尔登斯特罗姆巨型球蛋白血症的案例研究.
- 开始治疗是每天服用140毫克的易布鲁替尼.
- 监测血红蛋白水平,WM状态和传染性并发症.
主要成果:
- 易布鲁替尼治疗使血红蛋白水平正常化,严重贫血症得到缓解.
- 患者实现了很好的部分缓解WM.
- 在ibrutinib治疗期间没有报告新的感染或并发症.
结论:
- 易布鲁替尼是沃尔登斯特罗姆巨型球蛋白血症中严重贫血的潜在治疗选择.
- 这种向治疗可以改善血液学参数和疾病控制.
- 易布鲁替尼提供了有利的安全性,减少了WM感染性并发症.
更多相关视频
10:32Generation of Recombinant Human IgG Monoclonal Antibodies from Immortalized Sorted B Cells
Published on: June 5, 2015
18.5K
11:10Antibody Binding Specificity for Kappa (Vκ) Light Chain-containing Human (IgM) Antibodies: Polysialic Acid (PSA) Attached to NCAM as a Case Study
Published on: June 29, 2016
14.1K
相关概念视频
Immunodeficiency Diseases
950
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...
There are three main causes of immunodeficiency...
950
Development of Immunocompetence
317
The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
317
Humoral Immune Responses
72.8K
Overview
72.8K
Hybridoma Technology
14.8K
Hybridoma technology is used for the large-scale production of monoclonal antibodies. Monoclonal antibodies bind to only a single antigenic determinant or epitope. Such antibodies are used in research, diagnostics, and disease therapy. The hybridoma technology established in 1975 by Georges Köhler and Cesar Milstein was awarded the Nobel Prize in Medicine in 1984 for revolutionizing research and therapy.
Hybridoma Selection
Commonly used fusion techniques — electroporation,...
Hybridoma Selection
Commonly used fusion techniques — electroporation,...
14.8K
Transcytosis of IgG
2.8K
Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
2.8K
Disorders of Leukocytes
963
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...
963
