Characteristics of primary splenic diffuse large B-cell lymphoma and role of splenectomy in improving survival.
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.
['Bairey|Osnat|O|', 'Shvidel|Lev|L|', 'Perry|Chava|C|', 'Dann|Eldad J|EJ|', 'Ruchlemer|Rosa|R|', 'Tadmor|Tamar|T|', 'Goldschmidt|Neta|N|']
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 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
diffuse large B-cell lymphoma splenectomy non-Hodgkin lymphoma progression-free survival splenic lymphoma