What pathological finding is associated with Gaucher's Disease?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Gaucher's Disease is characterized by the accumulation of glucocerebrosides due to a deficiency in the enzyme glucocerebrosidase. This accumulation primarily affects macrophages, leading to the classic histological finding of crinkled or “wrinkled tissue paper” appearance in these macrophages. This distinct appearance is due to the engorgement of these cells with lipid-laden material, which is a hallmark of the disease.

The presence of crinkled-appearing macrophages is significant as it helps pathologists identify Gaucher's Disease. These macrophages are often referred to as Gaucher cells and can be found in various organs, including the spleen, liver, and bone marrow, where they contribute to the symptoms of the disease, such as splenomegaly and bone pain.

In this context, other options do not correctly describe the pathological findings seen in Gaucher's Disease. For instance, large globoid bodies are associated with Krabbe’s disease, while increased VLDL synthesis and the presence of ApoB lipoproteins are more related to lipid metabolism disorders but are not characteristic of Gaucher’s Disease. Thus, the key understanding here centers on recognizing the unique appearance of macrophages in Gaucher

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