Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Friday, August 15, 2008

Successful Bilateral Lung Transplantation for Lymphangiomatosis.

Successful Bilateral Lung Transplantation for Lymphangiomatosis.
Am J Transplant. 2008 Jul 28

Kinnier CV, Eu JP, Davis RD, Howell DN, Sheets J, Palmer SM.
Division of Pulmonary Medicine, Duke University Medical Center, Durham, NC, USA.

Lymphangiomatosis is a rare disease of lymphatic proliferation for which no adequate treatment is known. We report the first successful case of bilateral lung transplantation for the treatment of end-stage pulmonary lymphangiomatosis. A successful outcome was achieved with continued survival beyond 4 years posttransplant and stable lung function. The primary obstacles to significant gains in pulmonary function were thoracic, skeletal and abdominal lymphangiomatosis, which led to pulmonary restriction. Our report demonstrates that pulmonary lymphangiomatosis should be included among those diseases for which lung transplantation is considered potentially beneficial treatment but also emphasizes the importance of screening patients carefully for chest wall and abdominal lymphangiomas that may impede recovery.

PMID: 18671675 [PubMed - as supplied by publisher]


Lymphangiomatosis: a differential diagnosis of lytic lesions of the temporal bone.

Lymphangiomatosis: a differential diagnosis of lytic lesions of the temporal bone.
J Laryngol Otol. 2008 Jul
Naguib MB, Al-Jazan N, Hashem T.
Department of Otolaryngology, King Faisal University, Al-Khobar, Saudi Arabia.

Objective: We report a histologically proved case of lymphangiomatosis of the skull, involving the temporal bone and presenting as multiple lytic bone lesions.Method:A case report and a review of the literature concerning lymphangiomatosis are presented.

Results: Lymphangiomatosis affecting bones is a rare disorder caused by a congenital malformation of the lymphatic system, resulting in diffuse proliferation of the lymphatic channels and involving bones, parenchymal organs and soft tissue. Involved bones show massive osteolysis and progressive, localised bone resorption.

Conclusion: Lymphangiomatosis should be kept in mind in the differential diagnosis of lytic lesions of the skull.

PMID: 18667106 [PubMed - as supplied by publisher]


Monday, August 04, 2008

Organization and developmental aspects of lymphatic vessels

Organization and developmental aspects of lymphatic vessels

Arch Histol Cytol. 2008 Mar
Ohtani O, Ohtani Y.
Department of Anatomy, Faculty of Medicine and Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama.

The lymphatic system plays important roles in maintaining tissue fluid homeostasis, immune surveillance of the body, and the taking up dietary fat and fat-soluble vitamins A, D, E and K. The lymphatic system is involved in many pathological conditions, including lymphedema, inflammatory diseases, and tumor dissemination. A clear understanding of the organization of the lymphatic vessels in normal conditions would be critically important to develop new treatments for diseases involving the lymphatic vascular system. Therefore, the present paper reviews the organization of the lymphatic vascular system of a variety of organs, including the thyroid gland, lung and pleura, small intestine, cecum and colon in the rat, the diaphragm in the rat, monkey, and human, Peyer's patches and the appendix in the rabbit, and human tonsils.

Methods employed include scanning electron microscopy of lymphatic corrosion casts and tissues with or without treatment of alkali maceration technique, transmission electron microscopy of intact tissues, confocal microscopy in conjunction with immunohistochemistry to some lymphatic-specific markers (i.e., LYVE-1 and VEGFR-3), and light microscopy in conjunction with enzyme-histochemistry to 5'-nucleotidase. Some developmental aspects of the lymphatic vessels and lymphedema are also discussed.

Archives of Histology and Cytology