title: The significance of pre-therapeutic F-18-FDG PET-CT in lymphoma-associated hemophagocytic lymphohistiocytosis when pathological evidence is unavailable.
abstract: OBJECTIVE
  The significance of positron emission tomography/computed tomography (PET-CT) in identifying patients with lymphoma-associated hemophagocytic lymphohistiocytosis (LAHLH) when pathological evidence is unavailable remains uncertain.
  
  
  METHODS
  In this retrospective study, 44 HLH patients who underwent PET-CT before clinical treatment were enrolled, and 18 of them were highly suspected as LAHLH by PET-CT. We compared the PET-CT parameters between confirmed LAHLH and non-LAHLH patients. The efficacy of initial therapies for highly suspected LAHLH patients was analyzed as well.
  
  
  RESULTS
  We found that the SUVSp, SUVBM, SUVLN, SUVmax, SUVLN/Li, and SUVmax/Li in LAHLH group were significantly higher than those in non-LAHLH group (p = 0.003, p = 0.034, p = 0.003, p < 0.001, p = 0.039, and p = 0.035, respectively). HLH patients with an SUVmax value >5.5, an SUVLN value >3.3, and an SUVSp value >4.8 were more likely to be LAHLH (p < 0.001, p = 0.003, and p = 0.003, respectively). And the incidence of multiple lymphadenopathy with increased FDG uptake or the incidence of multiple bone lesions in LAHLH patients was significantly higher than those in non-LAHLH group (92.9 vs. 35.7 %, p = 0.004; 42.9 vs. 0 %, p = 0.016, respectively). Furthermore, by comparing the efficacy of initial therapies for highly suspected LAHLH patients (n = 18), we indicated that the CR rate was significantly higher in lymphoma-chemotherapy group than in immunosuppressive therapy group (90 and 25 %, respectively; p = 0.013). OS analysis revealed that highly suspected LAHLH patients treated with lymphoma-chemotherapy had better prognosis (264 days) than those treated with immunosuppressive therapy (15 days) (p < 0.0001).
  
  
  CONCLUSIONS
  When pathological evidence is absent, PET-CT may play an important role in identifying HLH patients underlying lymphoma. Once highly suspected as LAHLH by PET-CT, lymphoma-chemotherapies that directly treat the underling lymphoma may have a relatively favorable effect and better clinical outcomes than immunosuppressive therapy.
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Jujuan
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Dongjiao
authors.initials: D
authors.lastname: Wang
authors.affiliation: Department of PET-CT Centre, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Qingbo
authors.initials: Q
authors.lastname: Zhang
authors.affiliation: Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Limin
authors.initials: L
authors.lastname: Duan
authors.affiliation: Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Tian
authors.initials: T
authors.lastname: Tian
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Xiaoyan
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
authors.forename: Jianyong
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China. qiuhongxia@njmu.edu.cn.|Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China. qiuhongxia@njmu.edu.cn.
authors.forename: Hongxia
authors.initials: H
authors.lastname: Qiu
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000008:administration & dosage; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D053208:Kaplan-Meier Estimate; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D008223:Lymphoma / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging / Q000379:methods; D049268:Positron-Emission Tomography / Q000379:methods; D011241:Prednisone / Q000008:administration & dosage; D012372:ROC Curve; D019275:Radiopharmaceuticals / Q000008:administration & dosage; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Chemotherapy; Differential diagnosis; Lymphoma-associated hemophagocytic lymphohistiocytosis (LAHLH); PET–CT; Prognosis