Similar but not the same: Differential diagnosis of HLH and sepsis

• Published in: Critical reviews in oncology/hematology Vol. 114; pp. 1 - 12
• Main Authors: Machowicz, Rafał, Janka, Gritta, Wiktor-Jedrzejczak, Wieslaw
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.06.2017
• Subjects: Diagnosis, Differential; Hematology, Oncology and Palliative Medicine; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; HLH; Humans; Hyperferritinemia; Inflammation; Lymphohistiocytosis, Hemophagocytic - diagnosis; Macrophage activation syndrome; MAS; Sepsis; Sepsis - diagnosis; IAHS; TNFα; Hyperferritinemia; C-reactive protein; Human Immunodeficiency Virus; PT; Central nervous system; Familial Hemophagocytic Lymphohistiocytosis; ALT; Multi-Organ Failure; HS; Multiple Organ Dysfunction Syndrome; Activated partial thromboplastin time; Systemic inflammatory response syndrome; Prothrombin time; MODS; sCD; Aspartate transaminase; Hemoglobin; Hb; FHLH; Macrophage activation syndrome; IFNγ; patients; IVIG; SIRS; ANC; IPEX; NK-cell; Sepsis; sJIA; MAHS; MAS; Hemophagocytic Syndrome; Interferon gamma; CRP; Fever of Unknown Origin; LPI; HLH; Hemophagocytic Lymphohistiocytosis; CNS; Disseminated intravascular coagulation; FHL; Absolute neutrophil count; Malignancy-Associated Hemophagocytic Syndrome; LDH; Intensive Care Unit; sIL-2R; tumor necrosis factor alpha; EBV; PLT; WBC; Immune dysregulationPolyendocrinopathy Enteropathy X-linked; Epstein–Barr virus; DIC; FUO; aPTT; AST; LAHS; white blood cell; MOF; soluble Cluster of Differentiation; Intravenous immunoglobulin; Lymphoma-Associated Hemophagocytic Syndrome; Lactate dehydrogenase; Macrophage Activation Syndorme; Inflammation; and systemic juvenile idiopathic arthritis; pts; HIV; Alanine transaminase; Infeciton-Associated Hemophagocytic Syndrome; ICU; Natural Killer cell; Platelets; soluble interleukin 2 receptor; lysinuric protein intolerance; Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome

Highlights • In many patients HLH can be differentiated from sepsis. • Comparison of multiple parameters in HLH and sepsis is provided. • HLH-2004 criteria should be assessed in unresponsive patients with presumed sepsis.