hematology and oncology
Hemophagocytic Lymphohistiocytosis (HLH)
Treatment of HLH
Chemotherapy, cytokine inhibitors, immune suppression, and sometimes hematopoietic stem cell transplantation Treatment for hemophagocytic lymphohistiocytosis should be started if the disorder is suspected, even if not all diagnostic criteria are fulfilled. Patients are usually treated by a pediatric hematologist and in a referral center experienced in treating patients with HLH. Treatment depends on the presence of factors such as a family history of HLH, coexisting infections, and demonstrated immune system defects. Treatment for HLH may include cytokine inhibitors, immune therapy, chemotherapy, some combination of these, and possibly Hematopoietic Stem Cell Transplantation . (IL1 antagonist) in patients with secondary HLH has been reported and may allow for avoidance of intensive chemotherapy but requires further investigation ( Treatment reference ). Emapalumab, an interferon gamma–blocking monoclonal antibody, is available for patients with refractory, recurrent, or progressive HLH or with an intolerance to conventional therapy. 1.Bami S, Vagrecha A, Soberman D, et al: The use ofanakinrain the treatment of secondary hemophagocytic lymphohistiocytosis.Pediatr Blood Cancer67(11):e28581, 2020. doi: 10.1002/pbc.28581 