Hodgkinoid histiocytosis: an atypical nodal CD30 and S100-positive histiocytosis with eosinophilia

Histiocytes and dendritic cells may display cytological atypia and an aberrant immunophenotype even in reactive processes. Herein, we describe two cases of Hodgkinoid histiocytosis that show distinctive clinicopathological features, mimicking morphologically classic Hodgkin lymphoma (CHL), but suggesting reactive histiocytic/dendritic cell proliferation in lymph nodes. Both the patients presented with peripheral lymphadenopathy and blood eosinophilia with skin manifestations. Lymph node biopsy revealed scattered large histiocytes resembling Hodgkin cells with a round or stellate shape, abundant cytoplasm, and distinct nucleoli admixed in a predominant inflammatory background. The Hodgkinoid histiocytes occasionally showed emperipolesis. They expressed CD30, S100, and PD-L1 proteins but lacked PAX5 and CD1a expressions, Epstein-Barr association, BRAF V600E mutation, and PD-L1 gene amplification. Neither of the patients showed overt progression to malignant haematopoietic neoplasms during the disease course. An identical case series of four patients has been reported to date. Both these series highlight the potential of being interpreted as CHL due to the presence of Hodgkinoid histiocytes with CD30 positivity.

Automated summary

Hodgkinoid histiocytosis is a rare condition that mimics classic Hodgkin lymphoma but suggests reactive histiocytic/dendritic cell proliferation in lymph nodes. It is characterized by peripheral lymphadenopathy, blood eosinophilia, and skin manifestations. Pathological examination reveals large histiocytes resembling Hodgkin cells in an inflammatory background. These histiocytes express CD30 but lack other markers, viral association, and genetic mutations commonly seen in Hodgkin lymphoma.