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Erdheim-Chester disease and knee pain in a dialysis patient.

Rademacher S, Anagnostopoulos J, Luft FC, Kettritz R - Clin Kidney J (2014)

Bottom Line: Positron emission tomography shows areas of involvement.We initiated interferon-α therapy although other options remain open.In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Intensive Care Medicine, Virchow Klinikum , Charité Medical Faculty , Berlin , Germany.

ABSTRACT
Erdheim-Chester disease is a rare inflammatory condition characterized by a non-Langerhans histiocytic infiltration, involving the skeleton, nervous system, viscera, retroperitoneum and elsewhere. The aetiology is unknown. Positron emission tomography shows areas of involvement. We managed a dialysis patient with knee pain; a bone marrow specimen showed typical CD68 positive, but CD1a negative cells. We initiated interferon-α therapy although other options remain open. In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.

No MeSH data available.


Related in: MedlinePlus

(A) Sclerosis of both distal and proximal femurs is evident. Note is also made of the tumorous soft-tissue calcification adjacent to both trochanters and at other sites. (B) Total-body PET-CT shows markedly increased glucose metabolism at numerous skeletal sites, particularly proximal and distal long bones and pelvis.
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SFU031F2: (A) Sclerosis of both distal and proximal femurs is evident. Note is also made of the tumorous soft-tissue calcification adjacent to both trochanters and at other sites. (B) Total-body PET-CT shows markedly increased glucose metabolism at numerous skeletal sites, particularly proximal and distal long bones and pelvis.

Mentions: Additional imaging of our patient revealed sclerotic changes of both proximal and distal femurs bilaterally (Figure 2A), whereas additional tumorous calcifications are also visible. We performed whole-body positron emission tomography (CT-PET) scanning with 18F deoxyglucose (Figure 2B). The study revealed osteolytic and osteoplastic changes with increased activity at numerous sites including proximal and distal long bones, clavicles, sacrum, acetabula and glenoids. Note was also made of tumorous calcifications at various sites. We performed a bone marrow biopsy (Figure 3A). That study showed, in addition to changes of secondary hyperparathyroidism (not shown), intense infiltration with lipid-laden histiocytic cells. The cells were CD1a negative, but CD68 positive (Figure 3B). Long-term high-dose interferon-α is considered the treatment of choice for ECD and was initiated by our consultants [4]. We are currently observing our patient, who seems to be improving.Fig. 2.


Erdheim-Chester disease and knee pain in a dialysis patient.

Rademacher S, Anagnostopoulos J, Luft FC, Kettritz R - Clin Kidney J (2014)

(A) Sclerosis of both distal and proximal femurs is evident. Note is also made of the tumorous soft-tissue calcification adjacent to both trochanters and at other sites. (B) Total-body PET-CT shows markedly increased glucose metabolism at numerous skeletal sites, particularly proximal and distal long bones and pelvis.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4377794&req=5

SFU031F2: (A) Sclerosis of both distal and proximal femurs is evident. Note is also made of the tumorous soft-tissue calcification adjacent to both trochanters and at other sites. (B) Total-body PET-CT shows markedly increased glucose metabolism at numerous skeletal sites, particularly proximal and distal long bones and pelvis.
Mentions: Additional imaging of our patient revealed sclerotic changes of both proximal and distal femurs bilaterally (Figure 2A), whereas additional tumorous calcifications are also visible. We performed whole-body positron emission tomography (CT-PET) scanning with 18F deoxyglucose (Figure 2B). The study revealed osteolytic and osteoplastic changes with increased activity at numerous sites including proximal and distal long bones, clavicles, sacrum, acetabula and glenoids. Note was also made of tumorous calcifications at various sites. We performed a bone marrow biopsy (Figure 3A). That study showed, in addition to changes of secondary hyperparathyroidism (not shown), intense infiltration with lipid-laden histiocytic cells. The cells were CD1a negative, but CD68 positive (Figure 3B). Long-term high-dose interferon-α is considered the treatment of choice for ECD and was initiated by our consultants [4]. We are currently observing our patient, who seems to be improving.Fig. 2.

Bottom Line: Positron emission tomography shows areas of involvement.We initiated interferon-α therapy although other options remain open.In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.

View Article: PubMed Central - PubMed

Affiliation: Department of Nephrology and Intensive Care Medicine, Virchow Klinikum , Charité Medical Faculty , Berlin , Germany.

ABSTRACT
Erdheim-Chester disease is a rare inflammatory condition characterized by a non-Langerhans histiocytic infiltration, involving the skeleton, nervous system, viscera, retroperitoneum and elsewhere. The aetiology is unknown. Positron emission tomography shows areas of involvement. We managed a dialysis patient with knee pain; a bone marrow specimen showed typical CD68 positive, but CD1a negative cells. We initiated interferon-α therapy although other options remain open. In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.

No MeSH data available.


Related in: MedlinePlus