Nodal Langerhans cell histiocytosis with prominent eosinophilic emperipolesis

Abstract

Multinucleated giant cells of osteoclastic type are known to occur in nodal and extranodal lesions of Langerhans cell histiocytosis (LCH). These giant cells are thought to be derived from phagocytic histiocytes, which correlate with the degree of necrosis in LCH lesions. Emperipolesis commonly seen in Rosai-Dorfman disease is a distinct phenomenon characterized by intact phagocytosed cells in an intracytoplasmic vacuole protected from proteolytic digestion. We present a case of emperipolesis of inflammatory cells especially of eosinophils by multinucleated giant cells of Langhans type in a lymph node involved by LCH--a finding that has not been described previously in the literature.

Rosai–Dorfman‐type histiocytes occur in eosinophilic chronic rhinosinusitis and potentially play a role in disease initiation and persistence

“…The presence of eosinophils and in conjunction with RDD has been reported in association with idiopathic hypereosinophilic syndrome at autopsy in multiple sites, and in a patient with known RDD who later developed cutaneous lesions . Eosinophilic emperipolesis has been described recently in a case of nodal Langerhans cell histiocytosis . No reports were found documenting the presence of eosinophilic emperipolesis in RDD, which was a constant feature in all of our cases.…”

“…25 Eosinophilic emperipolesis has been described recently in a case of nodal Langerhans cell histiocytosis. 26 No reports were found documenting the presence of eosinophilic emperipolesis in RDD, which was a constant feature in all of our cases. This appears to be a unique finding in this context.…”

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

“…In rare cases of RDD, nuclear atypia, eosinophil infiltrate, and patients synchronously diagnosed with CHL were reported. [16][17][18] Nevertheless, to the best of our knowledge no study has specifically reported CD30 expression in RDD cells. BRAF V600E mutations were described in various histiocytic/dendritic cell lesions, including LCH 6 ; however, they were not found in either of the previous or present patients.…”

“…Emperipolesis is commonly observed in RDD, although it is not a prerequisite for the diagnosis. In rare cases of RDD, nuclear atypia, eosinophil infiltrate, and patients synchronously diagnosed with CHL were reported 16–18 . Nevertheless, to the best of our knowledge no study has specifically reported CD30 expression in RDD cells.…”

Infantile Rosai-Dorfman Disease With Isolated Brain Lesions Disseminated to the Parenchyma and Intraventricular Ependyma, Alteration of Leukocytes as a Promotion Factor in Immune Defense, and New Proposals: A Case Report and Literature Review

“…Although it is required for the diagnosis of RDD, it is not specific to this condition. Emperipolesis can be observed in various physiological and pathological conditions, including sarcoidosis [22], LCH [23], ECD [24], Hodgkin and non-Hodgkin lymphoma [25,26], neuroblastoma [21,27], epithelioid glioblastoma [28], and others [21].…”