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Case Reports in Hematology
Volume 2014, Article ID 287479, 4 pages
Case Report

Secondary Hemophagocytic Syndrome Associated with Richter’s Transformation in Chronic Lymphocytic Leukemia

1Trinity College, Dublin, Ireland
2Department of Hematology, Mayo Clinic Rochester, 200 1st Street SW, Rochester, MN 55905, USA

Received 21 November 2013; Accepted 11 December 2013; Published 16 January 2014

Academic Editors: D. J. Allsup and S. Tauro

Copyright © 2014 Nura El-Haj et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one’s own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency.