It was initially recognized in children, where it occurs primarily as an inherited syndrome related to homozygous null mutations in immune response genes involved in cytotoxic T cell and NK cell function. A minority of pediatric patients develop "secondary" HLH as a consequence of infection or ...
噬血性淋巴组织细胞增生症的诊疗建议(2010)(讨论稿)中华医学会儿科学分会血液学组,汤永民王天友噬血性淋巴组织细胞增生症(hemophagocyticlymphohistiocytosis,HLH),又称噬血细胞综合征(hemophagocyticsyndrome,HPS),是一组较为少见、由活化的淋巴细胞和组织细胞增生引起的多器官高炎症反应而又免疫无效的临床综合征,起病急...
THU0557鈥匟aemophagosytic Limphohistiosytosis (HLH) in Adults. Diseases Triggers, Course, Treatment and Pronostic Factors. Differences Between Patients with Macrophage Activation Syndrome (MAS) and Other Causes of Secundary Heamophagosytic Limphohistiosytosis (SHLH). report Of Thirty-Two Cases...
or with an immunodeficiency state such as Chediak-Higashi syndrome. Acquired forms of HLH can occur at any age – but often affect adults (although some adults are later found to have a predisposing mutation
Successful use of Infliximab in macrophage activation syndrome with severe CNS affection Macrophage activation syndrome (MAS) is a secondary form of haemophagocytic lymphohistiocytosis (HLH) seen in childhood in the context of rheumatic diseas... A Sellmer,B Stausbølgrøn,T Herlin - 《Pediatric ...
噬血性淋巴组织细胞增生症(hemophagocytic lymphohistiocytosis,HLH),又称噬血细胞综合征(hemophagocytic syndrome,HPS),是一组较为少见、由活化的淋巴细胞和组织细胞增生引起的多器官高炎症反应而又免疫无效的临床综合征,起病急、病情进展迅速、病死率高。主要特征为发热、肝脾肿大、血细胞减少和组织细胞噬血现象(主要见于...
(in non-SCT patients) 22 Reactivation therapy 22 Macrophage activation syndrome 23 Salvage therapy 23 Ending therapy 23 DEFINITION OF DISEASE STATES 24 STEM CELL TRANSPLANTATION 25 Suggestion for SCT regimen 25 DRUG INFORMATION AND TOXICITY 27 Therapy modifications 27 DATA COLLECTION AND EVALUATION 28 ...
Introduction: HLH syndrome is a rare but life-threatening condition of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages secreting high amounts of inflammatory cytokines. It can present in adults as a Sepsis mimic. Case 71-year-old Caucasian male with...
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of uncontrolled immune activation that has gained increasing attention during the last decade. The diagnosis of HLH is based on a constellation of clinical and laboratory abnormalities, including elevated serum ferritin levels. In the pediatric ...
In adults, HLH has been associated with a number of infectious etiologies, particularly viral infections. Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to a global pandemic and has been associated with acute respiratory ...