Hemophagocytic Lymphohistiocytosis (HLH): A Case Series and Review

Christine A. Garcia^1, , Kester Haye² and Theodore Gabig³

¹Department of Internal Medicine, Stony Brook University, Stony Brook, NY, US

²Department of Pathology, Stony Brook University, Stony Brook, NY, US

³Department of Medicine, Division of Hematology and Oncology, Stony Brook University, Stony Brook, NY, US

Cite this paper:
Christine A. Garcia, Kester Haye and Theodore Gabig. Hemophagocytic Lymphohistiocytosis (HLH): A Case Series and Review. American Journal of Medical Case Reports. 2016; 4(3):74-79. doi: 10.12691/ajmcr-4-3-1

Abstract

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 non-specific 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.

Keywords:
hemophagocytic lymphohistiocytosis hematology malignancy lymphoma infections

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