Erdheim-Chester disease: A case report
2017
Introduction. Erdheim-Chester disease (ECD) is a rare non-Langerhans-cell
histiocytosis. About 500 cases are published so far. It is multisystemic
disease characterised by bilateral symmetric long bones sclerosis. Main
histopathological finding is accumulation of big foamy histiocytes,
immunohistochemically positive to CD68, and negative to S-100 and CD1a. There
are no guidelines that reliably identify population that requires therapy,
but symptomatic ECD, organ failure and central nervous system involvement
re-quire treatment. Case report. We described a patient with a multisystemic
form of ECD affecting long bones, the hypophysis, abdomen, and the peripheral
nerves. Five years after initial symptoms ECD was suspected. Prednison was
initiated, 60 mg once a day. After obtaining the diagnosis of ECD, interferon
alpha 2A was introduced, but soon after stopped due to severe side effects.
Considering that histiocytes were positive to platelet derived growth factor
receptor alpha (PDGFR alpha) imatinib mesylate was started, but after two
months stopped due to no clinical and radiological improvement. The disease
was worsening and the patient died. Conclusion. We described the patient with
intraperitoneal form of ECD, without cardiac and pulmonary involvement. There
are several important issues: the diagnosis of ECD could be difficult to
make, three treatment regimens were included and the patient died nine years
after the initial symptoms due to indolent course of the disease and
unsuccessful treatment.
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