Hemophagocytic Lymphohistiocytosis (HLH) is a state of exaggerated immune response that manifests with multiorgan failure and decreased peripheral counts. Primary HLH is secondary to a genetic defect that leads to a dysregulated immune response and usually manifests in infancy. Secondary HLH is more common in adults and is usually associated with an underlying trigger such as infections (such as Ebstein Bar Virus) or malignancy. HLH has been described with T-cell associated malignancies whereas its association with B-cell malignancies and chronic lymphocytic leukemia (CLL) is rare. We hereby present the case of a patient who presented with HLH secondary to untreated CLL and where no infectious cause was identified. We then review the literature and discuss the diagnosis, prognosis and treatment of HLH most likely triggered by progression of an underlying CLL.
Keywords: Hemophagocytic Lymphohistiocytosis, HLH, CLL