The histopathology of Erdheim-Chester disease: a comprehensive review of a molecularly characterized cohort.

ABSTRACT: Erdheim-Chester disease is a rare, non-Langerhans cell histiocytosis histologically characterized by multi-systemic proliferation of mature histiocytes in a background of inflammatory stroma. The disease can involve virtually any organ system; most commonly the bones, skin, retroperitoneum, heart, orbit, lung, and brain are affected. Although a histiocytic proliferation is the histological hallmark of the disease, a wide range of morphological appearances have been described as part of case studies or small series. A comprehensive review of histopathological features in clinically and molecularly defined Erdheim-Chester disease has yet to be characterized. To address this issue and help guide clinical practice, we comprehensively analyzed the pathological spectrum of Erdheim-Chester diseas

SUBMITTER: Ozkaya N 

PROVIDER: S-EPMC6718953 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature