Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis
نویسندگان
چکیده
We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.
منابع مشابه
Secondary sea-blue histiocytosis derived from Niemann-Pick disease.
Sea-blue histiocytosis is a rare disorder seen in patients with lipid metabolic or ceroid storage diseases. Sea-blue histiocytes are ceroid-laden macrophages detectable by May-Giemsa staining. We report a case of a 28-year-old woman diagnosed with Niemann-Pick disease at 2 or 3 years of age. To confirm this diagnosis, we examined her bone marrow, which revealed scattered foci containing aggrega...
متن کاملSea blue histiocytosis 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, and cultured skin fibroblasts showed the patient to be deficient in sphingomyelinase activity....
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متن کاملIs it a sea-blue histiocyte?
In the “Images in Hematology’’ section of the Journal, And›ç et al. [1] briefly presented sea-blue histiocytes in a 45-year-old woman with type B Niemann-Pick disease [2,3]. I believe the cell photographed by the authors is fairly typical for Niemann-Pick disease with few nuclear remnants and superimposed lymphocytes. In my opinion, it is not typical for sea-blue histiocytes. We have seen sea-b...
متن کاملnon-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, and cultured skin fibroblasts showed the patient to be deficient in sphingomyelinase activity....
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