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. 2015 Aug;100(8):997-1004.
doi: 10.3324/haematol.2015.123562.

Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies

Kai Lehmberg  1 , Kim E Nichols  2 , Jan-Inge Henter  3 , Michael Girschikofsky  4 , Tatiana Greenwood  3 , Michael Jordan  5 , Ashish Kumar  5 , Milen Minkov  6 , Paul La Rosée  7 , Sheila Weitzman  8 ; Study Group on Hemophagocytic Lymphohistiocytosis Subtypes of the Histiocyte Society
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Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies

Kai Lehmberg et al. Haematologica. 2015 Aug.

Abstract

The hyperinflammatory syndrome hemophagocytic lymphohistiocytosis can occur in the context of malignancies. Malignancy-triggered hemophagocytic lymphohistiocytosis should be regarded separately 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.

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Figures

Figure 1.
Figure 1.
Flow chart for diagnosis and management of malignancy-associated hemophagocytic lymphohistiocytosis.
See this image and copyright information in PMC

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