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World J Hematol. Aug 6, 2014; 3(3): 71-84
Published online Aug 6, 2014. doi: 10.5315/wjh.v3.i3.71
Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment
Shinsaku Imashuku
Shinsaku Imashuku, the Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Kyoto 611-0042, Japan
Author contributions: Imashuku S contributed to this paper.
Correspondence to: Shinsaku Imashuku, MD, PhD, Consultant to the Department of Laboratory Medicine, Uji-Tokushukai Medical Center, 86 Kasugamori, Ogura-cho, Uji, Kyoto 611-0042, Japan.
Telephone: +81-774-201111 Fax: +81-774-202336
Received: March 9, 2014
Revised: May 9, 2014
Accepted: June 18, 2014
Published online: August 6, 2014
Core Tip

Core tip: This review discusses the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), the algorithms used to identify the underlying immune defects at the molecular level, and the optimal therapeutic approaches. For any HLH cases, a screening for primary HLH should be made following the diagnostic algorithm. During the process, immunosuppressive therapy should be started to control the hypercytokinemia with steroids, cyclosporine A, or intravenous immune globulin, and if primary HLH is confirmed, immunochemotherapy with a regimen containing etoposide or anti-thymocyte globulin should be given. Supportive measures to control hemorrhage/organ dysfunction are also required. In cases of primary HLH or secondary/refractory HLH, timely allogeneic hematopoietic stem cell transplantation is recommended.