Coexistence of Langerhans cell histiocytosis, Rosai–Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver

Abstract

The coexistence of skin-limited Langerhans cell histiocytosis (LCH) and Rosai-Dorfman disease (RDD) is an exceptional finding. The association of lymphomas and histiocytosis is also infrequent. We report the case of a 68-year-old man which presented an exceptional association of cutaneous LCH and RDD and splenic marginal zone lymphoma. He was stable for few years. Suddenly, the patient was admitted into Hematology Department with a remarkable enlargement of spleen and liver without enlargement of lymphadenopathies or skin lesions flare. He died 24 h later despite treatment with systemic chemotherapy combined with prednisone. Pre-mortem biopsy showed infiltration with histiocytic sarcoma. We think that a transdifferentiation phenomenon could explain our case, although we could not show a clonal relationship between the cutaneous and the liver diseases. We also want to pay attention to the fact that a fast transformation to a more aggressive disease can occur long time after the presentation of the first lesion, a problem that stresses the importance of performing a close and permanent follow-up of these patients.

Rosai-Dorfman disease: a report of eight cases in a tertiary care center and a review of the literature

“…The exact relation between these entities is not known, but the relatively frequent association among these rare diseases suggests that there may be a pathophysiological relationship among them and not only a simple coincidence. This is the case of the association between RDD and LCH, which suggests a common monocytic-macrophage lineage origin ( 42 - 45 ). RDD associated with different types of lymphoma can be simultaneously diagnosed and can occur in the same organ ( 46 - 48 ).…”

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

“…Further information: HS has been described as a secondary malignancy after several conditions, such as LCH, RDD, and lymphomas [123]. In several patients, a clonal relationship between HS or dendritic cell sarcoma and CLL with loss of PAX5 was observed [171,197].…”

Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

“…Llamas-Velasco et al 11 reported the first case with an aggressive clinical presentation. They identified a patient with concurrent RDD, LCH, and splenic marginal zone lymphoma.…”