2011
DOI: 10.1182/asheducation-2011.1.178
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Approach to Hemophagocytic Syndromes

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory condition. It may occur as a primary (genetic) condition due to mutations in genes important in the cytolytic secretory pathway that cause perforin and granzymes to induce apoptosis in target cells. Primary HLH is divided into familial HLH (FHLH1-5), in which HLH is the only manifestation of disease, and other genetic causes in which HLH is one of several clinical manifestations. The identical clinical findings may arise secondar… Show more

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Cited by 176 publications
(217 citation statements)
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“…The disease is characterized by: 1) hyperactivation of CD8 + T lymphocytes and macrophages; 2) the proliferation, ectopic migration and infiltration of these cells into various organs; and 3) hypercytokinemia with elevated levels of various cytokines, resulting in progressive organ dysfunction (7). Clinically, HLH primarily involves unremitting fever, hepatosplenomegaly, disseminated intravascular coagulation and cytopenia (at least two lineages).…”
Section: Discussionmentioning
confidence: 99%
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“…The disease is characterized by: 1) hyperactivation of CD8 + T lymphocytes and macrophages; 2) the proliferation, ectopic migration and infiltration of these cells into various organs; and 3) hypercytokinemia with elevated levels of various cytokines, resulting in progressive organ dysfunction (7). Clinically, HLH primarily involves unremitting fever, hepatosplenomegaly, disseminated intravascular coagulation and cytopenia (at least two lineages).…”
Section: Discussionmentioning
confidence: 99%
“…Acquired HLH is primarily observed in adults; however, both types can occur in all age groups (7-9). Acquired HLH is a very heterogeneous disorder that can be triggered by various infections, malignancies and metabolic and autoimmune conditions (7)(8)(9). It is difficult and challenging for clinicians to diagnose HLH in the acute phase, as the presentation of the syndrome is nonspecific and there are no gold standard diagnostic criteria.…”
Section: Discussionmentioning
confidence: 99%
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“…Desde la primera descripción en 1939 por Scott y Robb Smith (2), se han descrito mejor sus mecanismos, clasificándolos como primarios o forma genética, predominante en población pediátrica y el secundario, asociado a infecciones, procesos autoinmunes o neoplasias (1,3), y con criterios diagnósticos propuestos por la Histiocyte Society (4). Los mecanismos fisiopatológicos en la etiología primaria, involucran defectos genéticos que conllevan a un estado de inmunodeficiencia, activación inmune significativa o inmunopatología anormal (5).…”
Section: Introductionunclassified