Articles

DYSPLASIA OF THE LYMPHATICS WITH LYMPHOEDEMA, GENERALIZED LYMPHANGIECTASIS, CHYLOTHORAX AND "PSEUDO-STORAGE-DISEASE"

Authors
  • H Heimpel
  • JR Bierich
  • JM Herrmann
  • H Meister
  • J Vollmar

Abstract

A patient howing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in
early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous
angle. Chylous effusion and subcutaneous ehyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence
of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The " pseudo"-storage disease I regarded as secondary to the backflow of chylus into the tissues.

How to Cite:

Heimpel, H. & Bierich, J. & Herrmann, J. & Meister, H. & Vollmar, J., (1979) “DYSPLASIA OF THE LYMPHATICS WITH LYMPHOEDEMA, GENERALIZED LYMPHANGIECTASIS, CHYLOTHORAX AND "PSEUDO-STORAGE-DISEASE"”, Lymphology 12(4), 228-240.

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Published on
26 Sep 1979
Peer Reviewed