Abstract
IntroductionSinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy or extranodal lesions consisting of tissue infiltrates composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, sometimes in patients with a variety of treated and untreated neoplastic diseases. However, the literature contains reports of only 19 cases of Rosai-Dorfman Disease in association with lymphomas, Hodgkin's or non-Hodgkin's. The majority of these cases have the two diagnoses, malignant lymphoma and Rosai-Dorfman Disease, separated in time. Interestingly, infradiaphragmatic lymphadenopathy was a feature in the majority of previously reported cases of Rosai-Dorfman Disease and non-Hodgkin's lymphoma.Case presentationThis report provides details of a case with co-existing sinus histiocytosis with massive lymphadenopathy and diffuse large B cell non-Hodgkin's lymphoma. This case is the fifth described case of simultaneous Rosai-Dorfman Disease and concurrent non-Hodgkin's lymphoma. Unfortunately, the diagnosis of a clinically aggressive diffuse large B cell lymphoma was made at autopsy. The aggressive biological behavior of the diffuse large B cell lymphoma in this patient may have been related to the underlying immune dysregulation believed to be part of the pathophysiology of Rosai-Dorfman Disease.ConclusionTaken together this report and the preceding reports of Rosai-Dorfman Disease and non-Hodgkin's lymphoma suggests that in cases with a diagnosis of Rosai-Dorfman Disease in the setting of prominent infradiaphragmatic lymphadenopathy, clinicians should maintain a high index of suspicion for the presence of occult non-Hodgkin's lymphoma especially if the clinical course is atypical for classic Rosai-Dorfman Disease.
Highlights
Sinus histiocytosis with massive lymphadenopathy, known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes
Taken together this report and the preceding reports of Rosai-Dorfman Disease and non-Hodgkin's lymphoma suggests that in cases with a diagnosis of Rosai-Dorfman Disease in the setting of prominent infradiaphragmatic lymphadenopathy, clinicians should maintain a high index of suspicion for the presence of occult non-Hodgkin's lymphoma especially if the clinical course is atypical for classic Rosai-Dorfman Disease
Sinus histiocytosis with massive lymphadenopathy (SHML), known as Rosai-Dorfman Disease (RDD), is a rare entity first described in 1969 [1] that belongs to a group of non-malignant histiocytic disorders in which there is a pathologic increase in the number of histiocytes, mainly mononuclear phagocytic cells and the antigenpresenting cells of bone marrow origin [2], in nodal or extra-nodal sites
Summary
Simultaneous RDD and lymphoma remains a rare entity. The diagnosis of RDD in the presence of prominent subdiaphragmatic lymphadenopathy should raise the index of suspicion for concomitant lymphoma especially in the setting of limited response to steroid therapy. AFP: Alpha Fetal Protein; BAL: Bronchial Alveolar Lavage; bpm: beats per minute; CD: Cluster determinant; cm: centimeter; CMV: cytomegalovirus; CT: Computed Tomography; dl: deciliter; DCBCL: Diffuse Large B Cell Lymphoma; EBV: Epstein Barr Virus; g: gram; HD: Hodgkin's disease; HIV: Human Immunodeficiency Virus; HSV: Herpes Simplex Virus; ICU: Intensive Care Unit; Ig: immunoglobulin; IV: intravenous; L: liter; mg: milligram; mm: millimeter; mo: month; NHL: non-Hodgkin's Lymphoma; NOS: not otherwise specified; PO: per oral; SHML: Sinus Histiocytosis with Massive Lymphadenopathy; RDD: Rosai-Dorfman Disease; yr: year(s)
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