Not all lymphadenopathy is lymphoma: A case report of Rosai-Dorfman disease

Abstract

Background: Rosai-Dorfman Disease (RDD) is a rare non-Langerhans cell histiocytic disorder most commonly presenting as bilateral cervical lymphadenopathy in children and young adults. Although a heterogeneous entity with a range of clinical phenotypes, here we describe a presentation at one of the oldest ages found to date in the available literature and its spontaneous resolution in the absence of treatment. Case presentation: A 75-year-old man presented with new-onset right-sided cervical lymphadenopathy and fever, weight loss, fatigue, and anorexia. Physical exam revealed cervical and axillary lymphadenopathy. CT chest/abdomen/pelvis showed extensive adenopathy in the axillary regions, groin, mediastinum, retroperitoneum, splenic hilum, gastrohepatic ligament and porta hepatis, and celiac chains. Left axillary lymph node excisional biopsy revealed sinus histiocytosis with massive lymphadenopathy, consistent with a diagnosis of RDD. Flow cytometry showed mixed polytypic B-cell and T-cell subsets with polyclonal plasma cells. Immunofixation was negative for monoclonal immunoglobulins. On seeing a hematologist one month later, the patient’s symptoms had resolved and he had gained 10+ pounds. Discussion: Rosai-Dorfman Disease is a rare non–Langerhans cell histiocytosis most often seen in children and young adults, although it has been reported up to age 79 years. It typically presents with markedly enlarged, non-tender cervical lymphadenopathy; however, other nodal and extranodal sites can be involved. Due to the rarity of the disease there is a lack of robust data regarding specific treatment modalities, although consensus guidelines were published in 2018 recommending various treatment options, including corticosteroids, chemotherapy, radiation, and surgery. Spontaneous resolution may be observed but can take many months to years. Keywords: sRosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Non–langerhans cell histiocytosis.