Abstract
Abstract 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
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a state of uncontrolled cytokine production secondary to an underlying defective natural killer (NK) cell removal of antigen stimulation, resulting in persistent activation and proliferation of cytotoxic T-cell and antigen presenting cells
Primary HLH is secondary to a genetic defect that leads to a dysregulated immune response and usually manifests in infancy
Hemophagocytic lymphohistiocytosis is a dysregulated immune response characterized by multiorgan failure with peripheral cytopenias
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a state of uncontrolled cytokine production secondary to an underlying defective natural killer (NK) cell removal of antigen stimulation, resulting in persistent activation and proliferation of cytotoxic T-cell and antigen presenting cells (macrophages, histiocytes). Abnormal liver function tests with an aspartate aminotransferase (AST) level of 700 IU/L (normal range 10-40), alanine aminotransferase level (ALT) of 856 IU/L (normal range 7-56), and an alkaline phosphatase level of 389 IU/L (normal range 44-147) She was found to have hyperferritenemia (1800ug/L) and an elevated creatinine level of 4.3 mg/dL reflecting acute kidney injury as she was normal prior to admission. The patient’s family decided to withdraw care and did not want further treatment for either CLL or HLH
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