Epstein-barr virus-related hemophagocytic lymphohistiocytosis: hematologic emergency in the critical care setting

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic.

Figure 1

Images of the bone marrow. (a) Bone marrow aspirate smear, 1000x magnification, shows a hemophagocyte which engulfed 7 separate red blood cells (red arrows) and 2 platelets (green arrow). Yellow arrow points to a nuclei of histiocyte; (b) bone marrow clot section, EBV-encoded RNA (EBER) staining of the bone marrow revealing extensive EBV infected lymphocytes in the background (dark nuclear stain).

Figure 2

Trend of liver enzymes, temperature (a), total bilirubin, and ferritin (b) during the initial hospital stay. Dotted line indicates the start of therapy on day 3 (ALT: alanine aminotransferase; AST: aspartate aminotransferase; T bili: total bilirubin).