Peripheral T-Cell Lymphoma, Hemophagocytic Lymphohistiocytosis, and XIAP Gene Mutations: Getting the Treatment Right!

Clinical Practice Points

• •Hemophagocytic lymphohistiocytosis (HLH) is known to occur in the setting of malignancies, especially non-hodgkin lymphomas (NHL). Therapeutic management in such scenarios is usually targeted at treating the underlying lymphoma.
• •The occurrence of genetic mutations linked to familial HLH may add a considerable degree of uncertainty regarding optimal management, which may necessitate guiding therapy based on the patient's clinical condition.
• •Allogeneic hematopoietic cell transplantation (HCT) may be considered in the management of lymphomas presenting with HLH, in the setting of genetic mutations suggestive of a familial HLH predisposition.