Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by multisystemic involvement. We report a case of a 51-year-old male patient treated with pegylated IFN-α in a monthly schedule with low-dose prednisolone and achieved 34 months of control with minimal side effects.
Highlights
Histiocytoses constitute a heterogeneous group of rare disorders, characterized by
Open Access infiltration of myeloid cells to almost any organ with diverse macrophage or dendritic cell phenotypes. They are divided into Langerhans cells and non-Langerhans histiocytoses [1]
Erdheim-Chester disease (ECD) is a non-Langerhans variant of histiocytosis that often manifests as multisystemic involvement in adults aged 40 - 60, with a male predominance [5]
Summary
Histiocytoses constitute a heterogeneous group of rare disorders, characterized by. Open Access infiltration of myeloid cells to almost any organ with diverse macrophage or dendritic cell phenotypes. They are divided into Langerhans cells and non-Langerhans histiocytoses [1]. Non-Langerhans cell histiocytoses include numerous benign or malignant, localized or systemic, adult or pediatric diseases [2]. Some cases have an excellent prognosis after resection, and some even disappear spontaneously, but on the other hand, some progress rapidly and require intensive systemic therapies [2]
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