Pediatr Blood Cancer. 2007;48:124-131 [3] Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford). 2019;58(1):5-17. [4] Ramos-Casals M, Brito-Zer贸n P, L贸pez-Guillermo A, Khamas...
ranges between 0.36 and 0.9 per 100,000, compared to 3.0 per 100,000 in adults [Zhu, 2013]. The incidence of SLE in a Caucasian male under age 10 is extremely rare, with one study documenting lupus nephritis in Caucasian boys with an estimated prevalence of 0.20% and an annual incidence...
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with familial and acquired forms. The familial form is associated with mutations in the perfori... MB Jordan,D Hildeman,J Kappler,... - 《Blood》 被引量: 759发表: 2004年 Hemophagocytic lymphohistiocytosis (HLH) and related disorders He...
Introduction:HLH is a rare, life-threatening disorder, characterized by hyperstimulation of immune system leading to systemic inflammation and multi-organ failure. It is categorized as primary and secondary HLH. Secondary HLH usually affects adolescents and adults. It results from acquired immune dysregul...
However, in adults, the 3 specialized tests (gene mutations, NK cell function, and sIL-2r) are rarely performed.7 Among these 3 tests, sIL-2r is the most easily implemented in most adult centers. Primary HLH is highly penetrant at a young age, so genetic testing in adults is ...
Objective: To describe a case of adult onset, secondary hemophagocytic lymphohistiocytosis (HLH), with neurological manifestations.Background: HLH is a rare immune disorder, most often observed in children, involving macrophage hyperactivation and subsequent systemic inflammation. In adults, secondary HLH ...
Hemophagocytic Lymphohistiocytosis (HLH) is a severe and rapidly progressive immune disorder that can be life threatening if not diagnosed and treated promptly. The HLH2004 guidelines, originally devised for the diagnosis of HLH in pediatric subjects, lack due utility in the adult population. Despite ...
Ten of 100 patients who received transplantations received a cord blood transplantation (missing information on source, n = 24), and 8 are alive with a median follow-up after transplantation of 4.9 years. In children with active disease at HSCT (n = 43), 5-year cumulative survival after ...
Most patients responded well to discontinuation of anti-epileptics and HLH-directed therapy. Further research into immunological mechanisms of these complications is warranted.doi:10.1182/blood-2024-202475Yaswanta K Gummadi MDLuke Y. C. Chen MDMDBlood...
Familial hemophagocytic lymphohistiocytosis (HLH) is a rare primary immunodeficiency disorder characterized by defects in cell-mediated cytotoxicity that results in fever, hepatosplenomegaly, and cytopenias. Familial HLH is well recognized in children but rarely diagnosed in adults. We conducted a ...