PMC8276182_01
Radiological and biopsy findings.. (a) MRI (T2-blade-sag-FS) of the right humerus showing mottled signal changes. (b) Clavicular bone (H&E): The marrow space is infiltrated by histiocytes with bluish transparent bubbles. (c) Bone marrow (H&E): Massive infiltration of histiocytes and scarce remaining hematopoietic tissue. (d) Gastric mucosa (H&E): Infiltration of vacuolated histiocytes in an extended lamina propria. All scale-bars 50 mum.
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Magnetic resonance imaging (MRI) of the clavicular region and the upper arm showed mottled signal changes with a high signal intensity in the lateral clavicle, the humeral bone and the acromion ( Figure 1a). The fibrotic tissue contained the multivacuolated histiocytes as singular cells, small groups or sheets of cells ( Figure 1b). The bone marrow biopsy showed massive histiocytic infiltrates ( Figure 1c). The gastric biopsy showed antrum mucosa with elongated gastric pits and aggregates of multivacuolated histiocytes in both the superficial and deep lamina propria ( Figure 1d).
Staining properties.. All micrographs are from the same bone marrow biopsy. (a) CD 68/PGM1: The vacuolated cells are CD68-positive histiocytes. (b): Congo red stain: Vacuole content stains faintly red. (b) PASM: Vacuole content stains grey or black. All scale bars 50 mum.
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The cells were positive for CD68/PGM1 confirming the histiocytic nature ( Figure 2a). The vacuoles stained red in Congo red stain and black in the periodic acid methenamine silver (PASM) stain ( Figure 2b and c).
Pathological fractures and bone destruction: radiological and biopsy findings.. (a) Pelvic radiograph. Left hip: The hip prosthesis has been removed due to loosening and replaced by a Girdlestone hip. Right hip: Extensive lytic and sclerotic changes in the proximal femur and acetabulum leading to medial dislocation of the femoral head. (b) Biopsy from the right greater trochanter (H&E): The marrow space is filled with histiocytes with the bluish vacuoles characteristic of PVP-deposition. The bone trabecula has empty lacunar spaces and contains a bluish material. Scale bar 50 mum.
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In the right hip, he developed severe bone destruction of the acetabulum with dislocation of the femoral head and a subsequent femoral neck fracture ( Figure 3a). Biopsies from the greater trochanter region showed extensive histiocytic infiltrates ( Figure 3b).
Autopsy findings (H&E).. Infiltrates of histiocytes with the bluish vacuoles characteristic of PVP in all organs. (a) Myocardium: Interstitial fibrosis and histiocytic infiltrates. Scale bar 50 mum. (b) Pleura: Nodular lesion composed of fibrotic tissue and histiocytes. We found similar lesions in the pericardium and peritoneum. These were macroscopically visible. Scale bar 200 mum. (c) Pancreas: Pronounced fibrosis with histiocytic infiltrates. Poorly preserved ductal structures. Scale bar 50 mum. (d) Kidney: Cortical tissue showing interstitial fibrosis, tubular atrophy, and an infiltrate of histiocytes in the extended interstitium. Scale bar 50 mum.
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Microscopically, we observed PVP-deposition in all sampled organs and tissues ( Figure 4). PVP-containing histiocytes were partly scattered in the interstitium, e.g. in the myocardium ( Figure 4a), and partly organised like larger, nodular lesions, e.g. in the pleura ( Figure 4b). Peri- and intra-neural distribution was seen in several organs including the heart. The pancreatic tissue was dominated by dense fibrosis interspersed by heavy infiltrations of histiocytes ( Figure 4c). There were infiltrates of PVP-containing histiocytes in the interstitium, mostly in atrophic areas ( Figure 4d).