title: Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome.
abstract: T-cell lymphoma-associated hemophagocytic syndrome (T-LAHS) has been frequently reported in Asian countries and is considered with extremely poor prognosis. To summarize its clinical characteristics and explore its early diagnosis and treatment, we retrospectively analyzed the records of 113 patients with aggressive T cell lymphoma, of which 28 were associated with LAHS. According to WHO classification (2001), 22 cases were classified into peripheral T-cell lymphoma (unspecified), 2 into extranodal NK/T-cell lymphoma, and 4 into systemic anaplastic large cell lymphoma. The median survivals of the LAHS and no-LAHS groups were 40 days and 8 months, respectively. The elevating rates of serum lactate dehydrogenase (LDH) (100% vs. 55%), ferritin (100% vs. 64%), fasting triglycerides (79% vs. 43%), and hypofibrinogen (43% vs. 14%) levels were higher in the LAHS group than in the no-LAHS group (P < 0.05), so were bone marrow involvement (57% vs. 32%, P < 0.05) and liver dysfunction (40% vs. 13%, P < 0.05). Eleven of the 28 LAHS patients did not receive any chemotherapy, and 14 received CHOP regimen as initial chemotherapy. Three patients in critical conditions were given plasma exchange and gained the chance of initial chemotherapy. We suggest that in patients presenting with fever, hepatosplenomegaly, cytopenia, and constantly increasing levels of serum LDH, CA125, ferritin, transglutaminase, and beta2-microglobulin, T-LAHS should be taken into account. Repeating biopsies of multiple parts of bone marrow may help diagnosis. The therapeutic result of chemotherapy alone or combined for T-LAHS was discouraging and the survival time of most cases was no more than 1 year. Plasmapheresis as initial therapy is worth considering in critical cases.
authors.affiliation: Department of Hematology, the First Affiliated Hospital, Zhejiang University College of Medicine, Hangzhou, Zhejiang, People's Republic of China.
authors.forename: Hongyan
authors.initials: H
authors.lastname: Tong
authors.forename: Yanling
authors.initials: Y
authors.lastname: Ren
authors.forename: Hui
authors.initials: H
authors.lastname: Liu
authors.forename: Feng
authors.initials: F
authors.lastname: Xiao
authors.forename: Wenyuan
authors.initials: W
authors.lastname: Mai
authors.forename: Haitao
authors.initials: H
authors.lastname: Meng
authors.forename: Wenbin
authors.initials: W
authors.lastname: Qian
authors.forename: Jian
authors.initials: J
authors.lastname: Huang
authors.forename: Lingping
authors.initials: L
authors.lastname: Mao
authors.forename: Ying
authors.initials: Y
authors.lastname: Tong
authors.forename: Lei
authors.initials: L
authors.lastname: Wang
authors.forename: Jiejing
authors.initials: J
authors.lastname: Qian
authors.forename: Jie
authors.initials: J
authors.lastname: Jin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001856:Bone Marrow Examination; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000401:mortality / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000145:classification / Q000150:complications* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis