4. Clinical Manifestations


We have had a very macrophage lysosome-centric view of the disease, which holds that the lipid accumulates in the macrophages hence we see these very prominent and conspicuous macrophages that are abnormal under the light microscope because the cells are engorged with lysosomes containing this lipid. This view holds that the macrophages accumulate and get filled with this lipid, and wherever these macrophages build up you get disease manifestations, hence splenomegaly, lung infiltration, wide spectrum of bone disease, hepatomegaly as well as marrow infiltration in cytopenia.





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