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.
Children and infants are the persons most likely to be affected, with two thirds of the patients younger than three months. The presenting symptoms of FHL are pallor, irritability, anorexia, diarrhea, failure to thrive, and a maculopapular rash. Approximately one third of the patients have central nervous system involvement. Pathology of the liver shows focal fatty changes, necrosis, and infiltration by lymphocytes and histiocytes. Erythrophagocytosis and hemophagocytosis are prominent findings essential to the diagnosis.