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 lymphohistio-
cytosis, 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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