Quero-Hernández A, Echeverría RA, Zárate AR, Escárraga VY, Garzón SE, Reyes MBM, Álvarez SRM, Vargas VM

Language: Spanish
References: 15
Page: 222-225
PDF size: 572.68 Kb.

ABSTRACT

The Erdheim-Chester disease is classified as non-Langerhans cell histiocytosis, whose histopathologic feature is the infiltration of foamy lipid-laden histiocytes.
Case report: Female two years, with peripheral pancytopenia, exophthalmos and limitation of motion of the right hip. Radiologically with lytic lesions in skull; lytic and sclerotic long bones. Bone biopsy was CD68+, CD1a-, MPO + and reticulin fibrosis. She was treated with interferon-alpha with lesion progression, so that systemic chemotherapy is applied, with appropriate response.
Discussion: There is no consensus treatment for this disease, so rare diagnosis. Different results have been reported with the use of systemic steroids, interferon, cytotoxic drugs, radiotherapy, chemotherapy and hematopoietic cell transplantation.
Conclusion: Apparently, this case represents the first to be reported in which the lesions are lytic and sclerotic type, prone to symmetry, with immune phenotype of Erdheim-Chester (CD68+, CD1a-) and appropriate response to the scheme designed to the Langerhans cell histiocytosis.

REFERENCES

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