Very low levels of high density lipoprotein cholesterol in four sibs of a family with non-neuropathic Niemann-Pick disease and sea-bluehistiocytosis. Very low serum levels of high density lipoprotein cholesterol ranging from 8.6 to 13.9 mg/dl were detected in four out of 12 sibs of a Brazilian kindred with the non-neuropathic form of Niemann-Pick disease. Hepatosplenomegaly, interstitial
Seabluehistiocytosis: a common abnormality of the bone marrow in myelodysplastic syndromes. To determine whether myelodysplastic syndromes (MDS) are associated with seabluehistiocytosis in the bone marrow. A retrospective review of bone marrow aspirates from 35 patients presenting consecutively with MDS and from 20 patients with each of the following: normal marrow appearance (routine
Sea-bluehistiocytosis associated with hyperlipidaemia. A patient with sea-bluehistiocytosis in spleen and bone marrow with an accompanying hyperlipidaemia is described. The hyperlipidaemia was due to an increase in "free" cholesterol, lecithin, and triglycerides. Despite these findings lecithin-cholesterol acyl transferase activity was normal. Although the precise biochemical defect
Seabluehistiocytosis in a patient with chronic non-neuropathic Niemann-Pick disease. A patient with Niemann-Pick disease is reported together with family studies. Her liver and bone marrow were shown to be infiltrated with sea blue histiocytes. Other organs, spleen and lung, were presumably also involved but histological proof was not obtained. Enzyme assay of leucocytes, lymphocytes was slightly more labile than that of the controls. It seems clear that chronic Niemann-Pick disease without neurological involvement is associated with seabluehistiocytosis.