Children sea-blue histiocytosis (2 cases) compared with phospholipidosis induced by 4-4' DET (4-4' p (diethylamino-2-ethoxy phenyl) 3-4 hexane) in rat.

Abstract

Two cases of children with liver and spleen enlargement are reported. Sea-blue histiocytes and Pick cells were found in both cases in liver, spleen, bone marrow and blood. Further more, lysobisphodphatidic acids were identified in phospholipid analysis of liver biopsies and cultived liver cells. Absence of neurologic involvement at 14 and 18 years fo age suggest a Crocker type C of Niemann-Pick disease, i.e. a not yet well defined entity. Resemblance of these morphological and biochemical abnormalities with certain cases of drug poisoning (especially the well-known intoxication by 4-4' DET) is discussed on the basis of results from experimental studies with this drug in the rat.

• Authors: M Gautier, J Raulin, D Lapous, C Loriette, J P Carreau, J Scotto
• Journal: Biomedicine / [publiee pour l'A.A.I.C.I.G.] (Biomedicine) Vol. 26 Issue 1 Pg. 52-60 (Feb 1977) ISSN: 0300-0893 [Print] France
• PMID: 193589 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Phosphatidic Acids
• Phospholipids
• Sphingomyelins
• Hexestrol

Topics

• Adolescent
• Adult
• Animals
• Bone Marrow (pathology)
• Cells, Cultured
• Color
• Female
• Hexestrol (adverse effects, analogs & derivatives, metabolism)
• Histiocytes (pathology)
• Humans
• Liver (metabolism, pathology, ultrastructure)
• Lymphocytes (pathology)
• Male
• Microscopy, Electron
• Niemann-Pick Diseases (metabolism, pathology)
• Phosphatidic Acids (metabolism)
• Phospholipids (metabolism)
• Rats
• Sphingomyelins (metabolism)