This fascinating disorder is also known as Sinus Histiocytosis with Massive Lymphadenopathy . It commonly presents as massive, painless, bilateral lymph node enlargement in the neck with fevers.
Most cases occur in the first or second decade of life and have a predilection for blacks.
Rarely sites other than the lymph nodes can be involved including the central nervous system, eyes, upper respiratory tract, skin, and head and neck region. Interestingly, the spleen and bone marrow have been spared. The association is dependent upon the site of the extranodal disease. Some cases have responded to chemotherapy but many times the disease undergoes spontaneous resolution. In others, an insidious course develops for years or decades.