In this issue of Lymphatic Research and Biology, we are pleased to include several manuscripts focusing on the common theme of lymphangiomatosis. This was partially motivated by the continued frustration in the management with patients having disorders dominated by the sequelae of lymphangiomatosis. This rare and often progressive disorder often requires the expertise of multiple specialists to manage the orthopedic, pulmonary, gastrointestinal, biochemical and/or pain-related symptoms. The authors in this issue provide a clinical overview, discuss common secondary problems, and provide insights into potential mechanisms mediating these sequelae. A review of the status of animal models is also presented, with exciting new research utilizing zebrafish models.
The authors elucidate the differences between lymphangiomatosis and other lymphatic diseases and discuss molecular pathways modulating lymphangiogenesis and osteoclast activity. These articles underscore the impediments patients and physicians encounter when dealing with lymphangiomatosis. Treatment is often empirical and sequential based on published single case reports and small series. At present there is no laboratory model that directly correlates with this disorder, making disease-specific therapies difficult to study.
We are grateful to the contributors of this series of articles and anticipate that this information will foster further basic research in this topic.
