Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies
Kai Lehmberg,
Kim E. Nichols,
Jan-Inge Henter,
Michael Girschikofsky,
Tatiana Greenwood,
Michael Jordan,
Ashish Kumar,
Milen Minkov,
Paul La Rosée,
Sheila Weitzman
Affiliations
Kai Lehmberg
Department of Pediatric Hematology and Oncology, University Medical Center Eppendorf, Hamburg, Germany
Kim E. Nichols
Department of Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA
Jan-Inge Henter
Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden
Michael Girschikofsky
Department of Medicine I, Hematology and Stem Cell Transplantation, Hemostasis and Medical Oncology, Elisabethinen Hospital, Linz, Austria
Tatiana Greenwood
Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden
Michael Jordan
Department of Immunology, Cincinnati Children’s Hospital, OH, USA
Ashish Kumar
Department of Immunology, Cincinnati Children’s Hospital, OH, USA
Milen Minkov
Department of Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Vienna, Austria
Paul La Rosée
Klinik für Innere Medizin II, Abt. Hämatologie und internistische Onkologie, Universitätsklinikum Jena, Germany
Sheila Weitzman
Department of Hematology and Oncology, Sick Kids Hospital, Toronto, Canada
The hyperinflammatory syndrome hemophagocytic lymphohistiocytosis can occur in the context of malignancies. Malignancy-triggered hemophagocytic lymphohistiocytosis should be regarded seperately from hemophagocytic lymphohistiocytosis during chemotherapeutic treatment, which is frequently associated with an infectious trigger. The substantial overlap between the features of hemophagocytic lymphohistiocytosis with features of neoplasms makes its identification difficult when it occurs in malignant conditions. To facilitate recognition and diagnostic workup, and provide guidance regarding the treatment of malignancy-associated hemophagocytic lymphohistiocytosis, consensus recommendations were developed by the Study Group on Hemophagocytic Lymphohistiocytosis Subtypes of the Histiocyte Society, an interdisciplinary group consisting of pediatric and adult hemato-oncologists and immunologists.
