Hemophagocytic Lymphohistiocytosis (HLH): A Case Series and Review

Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening emergency and any delay in diagnosis and/or treatment is associated with high mortality. It is rarely observed in adult patients. HLH has multifaceted clinical presentations with often non-specific signs and symptoms that are often found in other clinical conditions. Classical manifestations suggestive of HLH include fever, cytopenia (thrombocytopenia), liver dysfunction, presence of CNS symptoms and coagulopathy. Hepatic dysfunction and failure can also be a presenting manifestation of HLH and may greatly complicate the clinical course. Case Presentation: We present four cases of secondary HLH in adults with varying presentations with a variety of underlying triggers including EBV infection, T cell lymphoma and blinatumomab therapy. Conclusion: HLH has multifaceted clinical presentations with often nonspecific signs and symptoms that are often found in other clinical conditions. Early recognition of HLH is critical in initiating therapy early on and preventing high mortality resulting from multi-organ failure.