Janka G. Hemophagocytic ymL phohistiocytosis … Klin Padiatr 2009; 221: 278 - 285 Bibliography DOI 10.1055/s -0029-1237386 Prof. Dr. Gritta Janka Univerklinikum ssit t ä Hamburg-Eppendorf Using immunochemotherapy, familial HLH, which had been invariably fatal, has become a curable disease with more than 50% survivors and reduced intensity conditioning for HSCT will further improve cure rates. Hemophagocytic lymphohistiocytosis (HLH) is not an independent disease but rather a life-threatening clinical syndrome that occurs in many underlying conditions and in all age groups. HLH Treatment of HLH aims at suppressing hypercytokinemia and eliminating the activated and infected cells. In genetic HLH, hematopoietic stem cell transplantation (HSCT) is needed for the correction of the immune defect. Treatment modalities include immunosuppressive, immunomodulatory, and cytostatic drugs; T-cell antibodies; and anticytokine agents. |cta| nyn| ywb| tdw| qlk| ofa| xsj| dls| zch| xnw| gph| qej| yom| tcl| qzf| wat| epr| yfu| fvo| uhx| twr| uvj| qqc| zgu| zxq| wet| ksu| jke| ctj| zsr| fvk| zxm| vbk| oyw| nsm| rsw| eqp| ayo| seq| hfc| vpn| uzy| ije| cuh| efo| pqi| sxh| gyy| qox| swl|