title: Characteristics of primary splenic diffuse large B-cell lymphoma and role of splenectomy in improving survival.
abstract: BACKGROUND
  Primary splenic diffuse large B-cell lymphoma (PS-DLBCL), an uncommon type of non-Hodgkin lymphoma, has been investigated only in small patient series before the rituximab era. The therapeutic role of splenectomy in addition to immunochemotherapy is unknown.
  
  
  METHODS
  The databases of 7 medical centers in Israel were searched for patients diagnosed with PS-DLBCL in 1982-2013, and clinical, treatment, and outcome data were collected for 87 patients. The mean patient age was 59.6 years; 57.5% were male.
  
  
  RESULTS
  Patients presented with abdominal pain (81%), B symptoms (59%), splenomegaly (84%), splenic masses (97%), and high lactate dehydrogenase (LDH) levels (84%); 61% had stage I or II disease. The diagnosis was made with core-needle biopsy in 46 patients and with diagnostic splenectomy in 39 patients. Eighty patients (92%) were treated with cyclophosphamide, doxorubicin, vincristine, and prednisone; 68 (78%) received rituximab. A complete response was achieved in 67 patients (77%), and a partial response was achieved in 8 (9%). At 5 years, the overall survival (OS) rate was 77%, and the progression-free survival (PFS) rate was 67%. When patients were stratified by splenectomy at diagnosis, the OS rates were 91% for splenectomized patients and 68% for nonsplenectomized patients (P = .08), and the PFS rates were 85% and 55%, respectively (P = .02). The respective values for the subgroup with early-stage disease were 96% and 63% for OS (P = .009) and 90% and 51% for PFS (P = .01). In a multivariate analysis, a low Eastern Cooperative Oncology Group performance status and splenectomy independently predicted better PFS (P < .03).
  
  
  CONCLUSIONS
  Patients with PS-DLBCL usually present with abdominal pain, high LDH levels, and a splenic mass. This study shows for the first time that splenectomy at diagnosis improves survival, specifically in patients with early-stage disease.
authors.affiliation: Hematology Institute, Rabin Medical Center, Petach, Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Osnat
authors.initials: O
authors.lastname: Bairey
authors.affiliation: Hematology Institute, Kaplan Medical Center, Rehovot, Israel.|Faculty of Medicine, Hebrew University, Jerusalem, Israel.
authors.forename: Lev
authors.initials: L
authors.lastname: Shvidel
authors.affiliation: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.|Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Chava
authors.initials: C
authors.lastname: Perry
authors.affiliation: Blood Bank and Hematology Institute, Rambam Medical Center, Haifa, Israel.|Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
authors.forename: Eldad J
authors.initials: EJ
authors.lastname: Dann
authors.affiliation: Hematology Department, Shaare Zedek Medical Center, Jerusalem, Israel.
authors.forename: Rosa
authors.initials: R
authors.lastname: Ruchlemer
authors.affiliation: Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.|Hematology Institute, Bnai-Zion Medical Center, Haifa, Israel.
authors.forename: Tamar
authors.initials: T
authors.lastname: Tadmor
authors.affiliation: Faculty of Medicine, Hebrew University, Jerusalem, Israel.|Hematology Department, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Neta
authors.initials: N
authors.lastname: Goldschmidt
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D013156:Splenectomy; D016896:Treatment Outcome; D055815:Young Adult
keywords: diffuse large B-cell lymphoma; non-Hodgkin lymphoma; progression-free survival; splenectomy; splenic lymphoma