Related Experiment Video
Updated: Jul 4, 2026
07:36
Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
Published on: May 1, 2015
Lymphangioleiomyomatosis: A review.
Donald W Hohman1, Dena Noghrehkar, Saman Ratnayake
1Department of Medical Education, Kern Medical Center, Bakersfield, CA 93305, United States. hohdon@sgu.edu
European Journal of Internal Medicine
|June 14, 2008
Summary
Lymphangioleiomyomatosis (LAM) is a rare smooth muscle cell disorder affecting women. Increased awareness and data registries are crucial for diagnosis and developing treatments for this progressive disease.
Area of Science:
- Rare diseases
- Pulmonology
- Genetics
Background:
- Lymphangioleiomyomatosis (LAM) is a rare disease of unknown cause, primarily affecting women.
- It involves a diffuse proliferation of smooth muscle cells and can occur sporadically or with tuberous sclerosis complex (TSC).
- LAM often presents with respiratory symptoms like breathlessness or pneumothorax, and can also manifest as abdominal issues.
Purpose of the Study:
- To summarize the current understanding of Lymphangioleiomyomatosis (LAM).
- To highlight the diagnostic features and clinical presentation of LAM.
- To emphasize the need for increased awareness and improved diagnostic strategies for LAM.
Main Methods:
- Review of clinical and pathological features of LAM.
- Analysis of imaging findings, including CT scans.
- Utilization of international LAM data registries to understand disease evolution.
Main Results:
- LAM is characterized by smooth muscle cell proliferation in the lungs and abdomen.
- CT scans reveal characteristic lung cysts and abdominal tumors (angiomyolipomas, lymphangioleiomyomas).
- The disease is likely underdiagnosed due to clinical latency and lack of specific tests.
Conclusions:
- No effective treatment currently exists for LAM.
- International data registries are evolving the understanding of LAM's presentation.
- Increased awareness and accurate diagnosis are vital for advancing therapeutic strategies and reducing misdiagnosis.
Related Concept Videos
01:15Development of the Lymphatic System
The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
01:22Myasthenia Gravis ll: Pathophysiology
The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...