Pathology
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Atlas of Bone Marrow pathology
Mojmir Moulis, Josef Feit et al.
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Gaucher's Disease
Etiology, pathogenesis

A congenital deficiency in lysosomal glucocerebrosid-beta-glucosidase causing glukocerebroside accumulation in macrophages in liver, spleen and bone marrow.

Clinical signs
  • pancytopenia caused by suppression of hematopoiesis in the marrow
  • other symptoms vary, depending other affected organs
Histology

Bone marrow is hypercellular with suppressed hematopoiesis and an increased number of large macrophages. These so-called Gaucher cells have an eccentric nucleus and abundant cytoplasm with a striated appearance which resembles folded cigarette rolling paper.