以骨损伤为特征的POEMS综合征:一个病例报告
Yang Wang1, Yalong Liu1, Xiaoli Huang2
1Wenjiang District People's Hospital, Chengdu, China.
Medicine
|December 20, 2023
概括
POEMS综合征通常会影响神经和皮肤,可能会出现骨损伤. 这一案例凸显了考虑POEMS综合征的重要性,即使有不寻常的主要症状,如骨病.
科学领域:
- 罕见的临床综合征
- 血液学 血液学 血液学
- 神经学 神经学
背景情况:
- POEMS综合征 (多神经病变,有机巨变,内分泌病变,单克隆性偏蛋白血症,皮肤变化) 是一种罕见的疾病.
- 通常表现为神经病变和皮肤变化;骨病不常见.
研究的目的:
- 报告一个独特的POEMS综合征病例,主要表现为骨损伤.
- 强调POEMS综合征非典型表现的诊断考虑因素.
主要方法:
- 一名患者出现肝功能不全,导致调查.
- 增强型CT显示了骨质变化;超声波显示脊髓巨变.
- 骨髓检查和免疫固定电泳证实IgA (λ) 单克隆蛋白.
主要成果:
- 该患者的诊断是基于骨损伤,器官壮大以及皮肤变化的病史,诊断出POEMS综合征.
- 用普得尼松酸盐和莱纳利多米德治疗导致肝功能改善,肝硬度降低.
结论:
- 骨损伤可能是POEMS综合征的主要表现.
- 彻底的病史和考虑非典型的表现对于准确的诊断至关重要.
相关概念视频
Bone Disorders
3.5K
Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
3.5K
Osteoclasts in Bone Remodeling
3.0K
Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during...
3.0K
Disorders of Leukocytes
959
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...
959
Disorders of the Skeletal Muscle
962
The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
962
The Proteasome
8.7K
Eukaryotic cells can degrade proteins through several pathways. One of the most important amongst these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
8.7K
Bone Marrow Sampling and Transplants
333
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 transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy...
333


