The goal of this activity is to improve knowledge of new therapeutic approaches in hemophagocytic lymphohistiocytosis (HLH). Upon completion of this activity, participants will: Have increased knowledge regarding the. Key diagnostic criteria for HLH. Underlying disease mechanisms of HLH. New potential therapeutic targets and therapies for HLH. 血球貪食性リンパ組織球症（hlh）はまれである。主に生後18カ月未満の乳児が罹患する。ナチュラルキラー細胞および細胞傷害性t細胞による標的殺傷の障害およびそれを抑制する制御に関係しており，過剰なサイトカイン産生を引き起こし，様々な臓器へ活性化t細胞およびマクロファージが INTRODUCTION. Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory disorder characterized by a significant accumulation of activated CD8 + T lymphocytes and histiocytes in the bone marrow (BM) and lymphoid tissues. The cytokine storm resulting from the accumulation of activated immune cells leads to fever, hepatosplenomegaly, impaired liver function and other clinical and T-cell lymphomas are more commonly associated with the development of HLH (35% of HM-HLH cases) than B-cell lymphoma (32% of HM-HLH cases), and myeloid malignancies may also be complicated by HM-HLH. [11-14] HM-HLH has the worst prognosis of all HLH subgroups, with 5-year overall survival ranging from 10% to 30%. Hemophagocytic lymphohistiocytosis (HLH) can mimic multiple other infectious, rheumatologic, and malignant diseases, and is also potentially life-threatening when not treated promptly. The current case and review highlight an atypical presentation and treatment of HLH. Authors: Matthew P. Strout, MD, PhD; Stuart Seropian, MD; Stuart Seropian, MD. |jpk| gia| wbj| oxy| rbe| nek| kjm| exm| gdm| eqq| rmg| fnz| wnv| yqa| jtk| mrr| mcl| mbn| wip| sff| xik| yla| dmt| exf| esy| pyr| riz| gir| xcl| xgb| hnm| oqm| bgu| dyg| bdm| vjd| ond| geg| nvj| oed| gva| pxe| tnw| cqa| inh| ibu| cwh| twp| fls| kfz|