The disease is usually benign, but significant morbidity and even death may result if massive tissue invasion of the liver, kidneys, lungs, and other critical structures occurs. Although 80 percent of patients are diagnosed in the first or second decade of life, this disorder can also affect the elderly. Patients are usually of African descent, and the incidence of this disease is highest in Africa and the West Indies. In patients with sinus histiocytosis with massive lymphadenopathy, the involved lymph nodes show marked sinusoidal dilatation and follicular hyperplasia with proliferation of foamy histiocytes and multinucleated giant cells in the sinuses. The etiology of this disorder is unknown and may be related to abnormal immune regulation.
Treatment is usually both unnecessary and ineffective.
As the disease resolves, extranodal disease regresses before nodal disease. Attempts at treatment should be reserved for special circumstances that are potentially life threatening. Irradiation, corticosteroids, vinblastine, and cyclophosphamide have all been administered with varying degrees of success. Familial Hemophagocytic Lymphohistiocytosis FHL is a rapidly fatal inherited disorder that is characterized by peripheral pancytopenia with bone marrow hyperplasia, systemic lymphadenopathy, fever, severe hepatic dysfunction, and hepatosplenomegaly. Patients have low fibrinogen levels with no evidence of disseminated intravascular coagulation.