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Table 1 Common differential diagnosis for Rosai Dorfman disease

From: ROSE in Rosai–Dorfman–Destombes (RDD) disease: a cytological diagnosis

Disease Clinical features Cytology IHC
Rosai Dorfman disease Children’s and young adults, M > F, painless lymphadenopathy, extra-nodal presentation seen Histiocytes with vesicular nucleus and abundant clear cytoplasm, with fine vacuoles and lymphocytes, reactive background of lymphocytes, plasma cells, neutrophils S100, CD68 positive, CD1a negative
Reactive lymph node hyperplasia Malaise, painless lymphadenopathies, self-limited disease Neutrophils, histocytes may or may not be present, Histiocytes negative for S100
LCH Localized or multiple lesions with disseminated disease. Nodal involvement may be sole manifestation, bone lesion may be seen Polymorphic infiltrate with eosinophils and histiocytes with cleaved nucleus CD1a positive
Hemophagocytic lymphohistiocytosis May be associated with malignancy of hematological origin, multi organ failure, pancytopenia, Hepatosplenomegaly Benign histiocytes with engulfed platelets and RBCs CD68 positive
Non-Hodgkin's lymphoma Lymphadenopathy, B symptoms weight loss, fever, loss of appetite Monotonous population of lymphoid cells Depends of cell of origin
Hodgkin lymphoma Lymphadenopathy with B symptoms Polymorphic population with small lymphocytes, eosinophils, plasma cells, and RS cells RS cell positive for CD15 and CD30
Metastatic carcinomas Primary in any organ Lymphoid population with metastatic tumor cells resemblance to primary organ morphology Depends on organ of origin