title: Clinical analysis and prognostic significance of lymphoma-associated hemophagocytosis in peripheral T cell lymphoma.
abstract: This study aims to retrospectively analyze the clinical characteristics, treatments, and prognosis of aggressive peripheral T cell lymphoma (PTCL) patients with a lymphoma-associated hemophagocytosis syndrome (LAHS). We compared the clinical features and the overall survival (OS) rates of 159 PTCL patients with and without LAHS as well as the treatment outcomes of these patients with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or intensive chemotherapy regimens. We observed that in 23 % (36/159) patients PTCL was associated with LAHS. Different subtypes of PTCL in LAHS patients were diagnosed and peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) was the main subtype (78 %). The median survival rates of the LAHS and non-LAHS groups were 3 and 16 months, respectively. The elevated rates of serum β2-microglobulin, ferritin, fasting triglycerides, and hypofibrinogen levels were higher in the LAHS group, so were bone marrow involvement, liver dysfunction, hepatosplenomegaly, and B symptoms. Three patients who were treated with a plasma exchange had a longer survival time. There was no statistically significant difference in the OS rates between the intensive chemotherapy and CHOP regimen groups (P > 0.05). PTCL patients with LAHS had a poorer prognosis. Awareness of the clinical symptoms and laboratory findings are crucial in order to diagnose LAHS in an early stage and repeated biopsies of multiple bone marrows from different locations in those patients without enlargement of superficial lymph nodes are necessary to improve the diagnosis. Intensive chemotherapy due to its severe toxicity was not obviously advantageous for the OS rate compared to the CHOP regimen.
authors.affiliation: Department of Hematology, Bone Marrow Transplant Center, The First Affiliated Hospital of Medical School of Zhejiang University, No. 79 Qingchun Road Hangzhou, 310003, Zhejiang, China.
authors.forename: Wanzhuo
authors.initials: W
authors.lastname: Xie
authors.forename: Keyue
authors.initials: K
authors.lastname: Hu
authors.forename: Fan
authors.initials: F
authors.lastname: Xu
authors.forename: De
authors.initials: D
authors.lastname: Zhou
authors.forename: Jingsong
authors.initials: J
authors.lastname: He
authors.forename: Jimin
authors.initials: J
authors.lastname: Shi
authors.forename: Yi
authors.initials: Y
authors.lastname: Luo
authors.forename: Jingjing
authors.initials: J
authors.lastname: Zhu
authors.forename: Jie
authors.initials: J
authors.lastname: Zhang
authors.forename: Maofang
authors.initials: M
authors.lastname: Lin
authors.forename: Xiujin
authors.initials: X
authors.lastname: Ye
authors.forename: He
authors.initials: H
authors.lastname: Huang
authors.forename: Zhen
authors.initials: Z
authors.lastname: Cai
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001773:Blood Cells / Q000473:pathology* / Q000502:physiology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010587:Phagocytosis / Q000502:physiology*; D011237:Predictive Value of Tests; D011379:Prognosis; D016019:Survival Analysis; D055815:Young Adult