Prevalence and clinical significance of Küpffer cell hyperplasia with hemophagocytosis in liver biopsies.
Prendki V, Stirnemann J, Lemoine M, Lohez M, Aras N, Ganne-Carrié N, Larroche C, Roulot D, Tengher-Barna I, Fain O, Ziol M. Am J Surg Pathol. 2011 Mar;35(3):337-45. (CUMC Full Text PDF)
Prendki V, Stirnemann J, Lemoine M, Lohez M, Aras N, Ganne-Carrié N, Larroche C, Roulot D, Tengher-Barna I, Fain O, Ziol M. Am J Surg Pathol. 2011 Mar;35(3):337-45. (CUMC Full Text PDF)
Prendki and colleagues discuss the prevalence and clinical significance of Kupffer cell hyperplasia with accompanying hemophagocytosis in liver biopsy samples. The authors identified hemophagocytosis in 69 of 5194 liver biopsies (1.3%) obtained over a 6-year period. Complete hemophagocytic syndrome (fever, splenomegaly, bicytopenia, hypertriglyceridemia, hyperferritinemia, and/or hypofibrinogenemia) was seen in 36% of patients in which hemophagocytosis was identified. Almost all patients had at least one of the following conditions: HIV infection, hematologic malignancy, autoimmune diseases, or an ongoing infectious process, including tuberculosis, CMV, or EBV. A decreased perforin to CD3+ lymphocyte ratio was associated with hemophagocytosis in this study.
This is a case we had in our service a couple of weeks ago. This young female presented with complete hemophagocytic syndrome and underwent liver biopsy for severe hepatic dysfunction. Decreased perforin to CD3 lymphocytes is seen in this case, as described in the study above.
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