Some patients with Hodgkin's Lymphoma will develop a hemophagocytic syndrome (HPS).


Features of HPS:

(1) proliferation of benign macrophages

(2) extensive phagocytosis of hematopoietic cells with cytopenias

(3) fever

(4) hepatosplenomegaly

(5) abnormal liver function tests and acute phase reaction


Typical findings in a patient with Hodgkin Lymphoma associated HPS:

(1) often male gender

(2) mixed cellularity or lymphocyte depleted Hodgkin's disease

(3) usually the presence of Epstein-Barr virus in tumor cells (100% if HIV positive, 92% if not HIV positive)

(4) disseminated Hodgkin's disease at diagnosis of HPS


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