Weathering a Cytokine Storm: A Case of EBV-Induced Hemophagocytic Lymphohistiocytosis.

Tiffany Y Shaw,Michael Schivo

doi:10.1177/2324709616647409

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by excessive immune activation. Acquired HLH is seen in adults and is often caused by infection or malignancy. Diagnosis is difficult and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Thus, in patients presenting with presumed severe sepsis or septic shock complicated by multiorgan failure without a clear infectious source, HLH should be considered. A disproportionately high ferritin may be one of the earlier laboratory findings to suggest HLH. We discuss a case of a young male who presented with presumed septic shock with multiorgan failure who was eventually found to have Epstein-Barr virus–induced HLH.

Highlights

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by uncontrolled immune activation leading to excessive macrophage activity and cytokine release.[1]
Hemophagocytic lymphohistiocytosis is a rare, life-threatening clinical syndrome that results from excessive activation of the immune system leading to uncontrolled cytokine release, tissue infiltration of histiocytes and lymphocytes, and multiorgan failure
In cases of primary HLH, relapsed HLH, or HLH from an unknown cause, hematopoietic stem cell transplant is recommended to improve longterm prognosis.[2,13]

Summary

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease caused by uncontrolled immune activation leading to excessive macrophage activity and cytokine release.[1] Tissue infiltration by overactive macrophages and cytotoxic lymphocytes leads to multiorgan dysfunction.[1] There are 2 types of HLH: primary or familial HLH, most commonly seen in infants and young children and caused by genetic defects,[2] and secondary or acquired HLH, seen in adults and often triggered by infection, malignancy, or rheumatologic disease.[1,2]. HLH has a variable presentation with nonspecific clinical symptoms and laboratory findings.[2] Because of probable underdiagnosis, the true incidence and prevalence of HLH in adults are unknown given insufficient epidemiologic data.[2,3] If left untreated the disease is frequently fatal, with median survival estimated to be less than 2 months.[4] With this case presentation, our aim is to increase awareness and help facilitate early recognition of HLH

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Conclusion