Hemophagocytic lymphohistiocytosis in adults. Leuk Lymphoma 2019 Jan;60(1):19-28
Date
07/04/2018Pubmed ID
29966474DOI
10.1080/10428194.2018.1482543Scopus ID
2-s2.0-85061376634 (requires institutional sign-in at Scopus site) 24 CitationsAbstract
Hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening condition characterized by uncontrolled inflammation, is increasingly recognized in adults. The management of adult onset HLH is challenging, in part due to gaps in current state of knowledge on etiology, clinical presentation, diagnosis, and management. HLH secondary to triggers such as infections, autoimmune disorders, and malignancy are more commonly seen in adults although cases of familial form have also been reported. Underlying conditions such as sepsis, or malignancy could pose as major confounders while applying universal diagnostic criteria, and therefore could lead to delay in diagnosis. Despite advent of newer therapeutic agents, outcomes of adults continue to remain poor. Future efforts need to be orchestrated to develop evidence-based tailored therapies to improve outcomes of this under recognized heterogeneous entity.
Author List
Bhatt NS, Oshrine B, An Talano JAuthor
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultAge of Onset
Anti-Infective Agents
Clinical Trials as Topic
Disease-Free Survival
Drug Therapy, Combination
Evidence-Based Medicine
Hematopoietic Stem Cell Transplantation
Humans
Immunosuppressive Agents
Lymphohistiocytosis, Hemophagocytic
Myeloablative Agonists
Neoplasm Recurrence, Local
Prognosis
Remission Induction
Salvage Therapy
Transplantation Conditioning
Transplantation, Homologous
