title: [Clinical analysis of 10 cases of secondary hemophagocytic lymphohistiocytosis treated with HLH-2004 chemotherapy].
abstract: This study was aimed to investigate the therapeutic efficacy of HLH-2004 chemotherapy in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). 10 cases of sHLH treated with HLH-2004 regimen at our department were analyzed retrospectively. The results showed that 7 patients had clinical response to HLH-2004 regimen, while other 3 patients had no clinical response. 5 cases did not complete initial therapy of 8 weeks. Out of 5 cases, 4 died in the process of chemotherapy, 1 patient abandoned for serious side effects but finally acquired remission following 4 cycles of CHOP regimen. 5 cases underwent the whole courses of initial therapy. Out of 5 cases, 3 patients acquired remission, and other 2 were not well controlled. Out of the 3 patients who had achieved remission, one died of relapse, and other 2 patients kept remission. Out of the 2 patients who were not well controlled, one patient died, but another patient acquired remission after being discharged. It is concluded that patients with infection-associated hemophagocytic syndrome (IAHS) have high rates of remission after receiving HLH-2004 regimen combining with effective antibiotics. However, patients with HLH secondary to EBV (EBV-HLH) or lymphoma (LAHS) have low rates of remission or are easy to get relapse after remission.
authors.affiliation: Department of Hematology, Jiangsu Provincial Hospital, Nanjing 210029, Jiangsu Province, China.
authors.forename: Li-Juan
authors.initials: LJ
authors.lastname: Zhang
authors.forename: Hong-Xia
authors.initials: HX
authors.lastname: Qiu
authors.forename: Jian-Yong
authors.initials: JY
authors.lastname: Li
authors.forename: Ji
authors.initials: J
authors.lastname: Xu
authors.forename: Ling-Ling
authors.initials: LL
authors.lastname: Wang
authors.forename: Yi-Xin
authors.initials: YX
authors.lastname: Hu
authors.forename: Lei
authors.initials: L
authors.lastname: Fan
authors.forename: Su-Jiang
authors.initials: SJ
authors.lastname: Zhang
authors.forename: Wei
authors.initials: W
authors.lastname: Xu
authors.forename: Han-Xin
authors.initials: HX
authors.lastname: Wu
authors.forename: Si-Xuan
authors.initials: SX
authors.lastname: Qian
authors.forename: Hua
authors.initials: H
authors.lastname: Lu
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone