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. 2014:2014:287479.
doi: 10.1155/2014/287479. Epub 2014 Jan 16.

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

Nura El-Haj  1 , Wilson I Gonsalves  2 , Vinay Gupta  2 , Jacob P Smeltzer  2 , Sameer A Parikh  2 , Preet P Singh  2 , Naseema Gangat  2
Affiliations

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

Nura El-Haj et al. Case Rep Hematol. 2014.

Abstract

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.

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Figures

Figure 1
Figure 1
(a) CT imaging of the chest demonstrating bilateral pleural effusions (arrows) and mediastinal adenopathy (circles) and (b) CT imaging of the abdomen demonstrating bulky mesenteric adenopathy (circle).
Figure 2
Figure 2
Bone marrow aspirate demonstrating a blue foamy macrophage engulfing erythroid and lymphoid precursors (arrow).
Figure 3
Figure 3
Bone marrow biopsy demonstrating a cluster of large cells positive for the EBV-encoded RNA (EBER) stain suggestive of an EBV driven large cell lymphoma (arrow).
See this image and copyright information in PMC

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