نتایج جستجو برای: hlh
تعداد نتایج: 1249 فیلتر نتایج به سال:
The familial form of hemophagocytic lymphohistiocytosis (HLH) is a lethal disorder. Although the prognosis for Epstein-Barr virus-associated HLH (EBV-HLH) remains uncertain, numerous reports indicate that it can also be fatal in a substantial proportion of cases. We therefore assessed the potential of immunochemotherapy with a core combination of steroids and etoposide to control EBV-HLH in 17 ...
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation that mimics and occurs with other systemic diseases. A 35-year-old female presented with signs of viral illness at 13 weeks of pregnancy and progressed to acute liver failure (ALF). We discuss the diagnosis of HLH and Kikuchi-Fujimoto (KF) lymphadenitis in the context of pregnancy and ALF. HLH may respond to c...
Hemophagocytic lymphohistiocytosis (HLH) is a result of uncontrolled macrophage activation with prominent phagocytosis of platelets, erythrocytes, and lymphocytes and hematopoietic precursors. Attempt should be made to establish or rule out HLH in patients presenting with fever, multisystem inflammation, and varying degrees of unexplained pancytopenia. Owing to the guarded prognosis associated ...
We report two cases of Herpes Simplex Virus (HSV) induced hemophagocytic lymphohistiocytosis (HLH). These cases highlight that neonates with HSV may have concurrent secondary HLH, a life threatening condition in which prompt diagnosis and treatment can drastically improve survival. Therefore, a low index of suspicion is needed to send, cost effective, sensitive screening blood tests for HLH in ...
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primar...
Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease, presenting with T cell-induced dysregulated cytokine production, systemic inflammation, and frequently intractable coagulopathy. We have administered recombinant human soluble recombinant thrombomodulin (rTM) for four cases of severe childhood Epstein-Barr virus-associated HLH (EBV-HLH) in combination with anti-inflammatory therapy an...
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory/hypercytokinemia syndrome clinicopathologically manifested by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hemophagocytosis. METHODS We searched the medical literature for English-written articles and analyzed data regarding the diagnosis, pathoetiology, prognosis, and managem...
Mondo complexes regulate TFEB via TOR inhibition to promote longevity in response to gonadal signals
Germline removal provokes longevity in several species and shifts resources towards survival and repair. Several Caenorhabditis elegans transcription factors regulate longevity arising from germline removal; yet, how they work together is unknown. Here we identify a Myc-like HLH transcription factor network comprised of Mondo/Max-like complex (MML-1/MXL-2) to be required for longevity induced b...
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare, underdiagnosed, fatal and devastating hyperinflammatory syndrome that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of uncontrolled inflammation. Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. In this case series,...
Evaluation of the Plasma Micro RNA Expression Levels in Secondary Hemophagocytic Lymphohistiocytosis
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. Micro RNAs (miRNA) are about 22 nucleotide-long, small RNAs encoded with genes, and they have regulatory functions in immune response. OBJECTIVE To determine the miRNA expression levels of 11 secondary HLH patients, we evaluated the associations of miRNA levels with pathogenesis, clinical pre...
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