Hemophagocytic Lymphohistiocytosis After Inactivated Influenza Vaccination in a Young Man Complicated by Severe Rhabdomyolysis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune response disorder that is usually fatal despite treatment. It is characterized by a dysregulation in natural killer (NK) T-cell function, causing activation of lymphocytes and histiocytes, resulting in a cytokine storm, end-organ damage, and eventually death. In this report, we describe the case of a previously healthy 38-year-old Caucasian man who presented with fever, nausea, vomiting, abdominal pain, myalgias, and weight loss for one week after inactivated influenza vaccination. The initial evaluation showed leukocytosis, lactic acidosis, and a severely elevated creatine kinase level (19,639 IU/L). The presentation was consistent with a diagnosis of sepsis, likely secondary to viral etiology and rhabdomyolysis. Subsequently, he rapidly deteriorated, requiring mechanical ventilation and developed refractory shock requiring pressor support and continuous veno-venous hemofiltration for acute kidney injury due to rhabdomyolysis. Later, he developed bicytopenia, hyperferritinemia, hypertriglyceridemia, and elevated inflammatory markers, raising the possibility of underlying HLH. Further tests showed low NK cell cytotoxicity and elevated sCD25. The H-score, which is a clinical tool to estimate the probability of HLH, showed an 88-93% probability of that potentially fatal disorder. The patient was treated with pulse-dose corticosteroids, intravenous immunoglobulins (IVIGs), and anakinra. He had a prolonged and complicated hospital stay for about two months. However, he was able to slowly recover. We believe that he developed secondary HLH in the setting of vaccination. Although rare, an early suspicion of HLH leads to the early initiation of directed therapy with immunosuppressant that would limit morbidity and mortality.

Keywords: anakinra; hemophagocytic lymphohistiocytosis (hlh); high ferritin; influenza vaccine; rhabdomyolysis.

Figure 1. Trend of creatine kinase levels during hospitalization.

Figure 2. Trends of CRP and ferritin levels during hospitalization.

CRP: C-reactive protein.