Question
How to Treat Hemophagocytic Lymphohistiocytosis?
Answer
Hemophagocytic lymphohistiocytosis, also known as hemophagocytic histiocytic lymphadenopathy, is a type of histiocytic disorder classified as Type II of histiocytosis. It includes both familial and secondary types. Familial hemophagocytic lymphohistiocytosis is an autosomal dominant genetic disease. Infants appear normal at birth but may suddenly develop high fever, jaundice, bleeding, and liver and spleen enlargement between 6 months and 1 year of age, with some experiencing seizures. About 50% have a positive family history, and the clinical course is often fatal, with a median survival of 2 months without treatment. It is caused by a 10th chromosome gene defect. Treatment and prognosis depend on the type of disease. Current treatments mainly include chemotherapy, immunotherapy, hematopoietic stem cell transplantation, and plasma exchange. Chemotherapy can use cytotoxic drugs such as vincristine or vinblastine combined with adrenal cortex hormones, or repeated plasma exchange can be applied along with VP16 or VM26 combined with adrenal cortex hormones. Immunotherapy has achieved satisfactory results using cyclosporine A in the treatment of familial HPS, and similarly, the use of antithymocyte globulin (ATG) can also induce remission. Hematopoietic stem cell transplantation can cure familial HPS patients, but chemotherapy cannot cure familial HPS.