Sea-blue histiocytes

Type E is also known as the sea-blue histiocytes syndrome and is considered to be a special form of Niemann-Pick disease found in adults. The stored, ceroid-like, acid-proof and PAS-positive pigment shows a sea-blue colour on Giemsa staining. Hepatosplenomegaly is present thrombopenia is likewise in evidence due to bone marrow involvement. Cirrhosis may develop. The prognosis is considered to be good. (243)... 

Putterman, C, Zelingher, X, Shouval, D. Liver failure and the sea-blue histiocyte/adult Niemann-Pick disease. Case report and review of the literature. J. Clin. Gastroenterol. 1992 15 146-149... 

When one young woman on chronic parenteral nutrition for short bowel syndrome presented with hepato-splenomegaly and pancytopenia (78), bone marrow examination showed the sea-blue histiocytes to be lipidladen macrophages. The total amount of fat in the regimen was subsequently reduced, and there was partial hematological improvement. The condition observed in this patient is analogous to that which occurs in Gaucher s disease and Niemann-Pick disease. 

Maier-Redelsperger M, Girot R. Sea-blue histiocytes in bone-marrow due to a long-term total parenteral nutrition including fat-emulsions. Br J Haematol 1997 97(3) 689. 

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