Superior outcome for splenectomised patients in a population‐based study of splenic marginal zone lymphoma in Sweden

Abstract

Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma where associations with viral hepatitis and autoimmune and inflammatory diseases (AID) have been indicated. We aimed at assessing the prevalence of viral hepatitis and AID at SMZL diagnosis and outcome by treatment in a Swedish population-based study. A total of 277 SMZL patients registered in the Swedish Lymphoma Register in 2007-2017 were included. A history of viral hepatitis was reported in five (2%) patients and AID prior to SMZL in 72/240 (30%) patients. Treatment was given up front for 207 (75%) patients. Splenectomy with or without systemic treatment was performed in 119 (57%) and was associated with statistically significantly better overall survival [hazard ratio, HR = 0Á47 (95% confidence interval, CI: 0Á23-0Á93), P = 0Á03] and progression-free survival (HR = 0Á55, 95% CI: 0Á35-0Á86, P = 0Á008) compared to non-splenectomised patients in multivariable analyses. The up-front splenectomised group was younger and generally had a lower Ann Arbor stage, but also more frequently B symptoms and high lactate dehydrogenase than the non-splenectomised group. Viral hepatitis and AID history did not affect SMZL outcome. We report high incidence of AIDs and low incidence of viral hepatitis in this population-based study of SMZL. Splenectomy up front was associated with a favourable outcome.

Splenic marginal zone lymphoma in Sweden 2000–2020: Increasing rituximab use and better survival in the elderly

“…Herein probably lies part of the explanation why our study shows no survival difference between rituximab-treated and splenectomized patients. However, also in the report by Sima et al, overall survival times with rituximab monotherapy and splenectomy appear very similar; their main finding was different outcomes between splenectomy versus all other modalities combined (including systemic treatment without rituximab) [13]. As in their material, an overall survival analysis of our material in which splenectomy is compared with all other modalities combined (including systemic treat-ment without rituximab) would show superiority for splenectomy with p = 0⋅022.…”

“…Modern first-line treatments include splenectomy [3][4][5][6], rituximab monotherapy [7][8][9][10] and rituximab combined with chemotherapy [9][10][11][12][13]. Splenectomy is preferable from a diagnostic perspective and gives a rapid relief of symptoms (i.e., abdominal discomfort and cytopaenias related to splenic sequestration) [13]. However, perioperative complications may occur (i.e., infections, bleeding and thrombosis), and patients with splenic marginal zone lymphoma are often elderly and at risk for surgical complications [14].…”

“…There is no consensus on first-line therapy of splenic marginal zone lymphoma. Modern first-line treatments include splenectomy [3][4][5][6], rituximab monotherapy [7][8][9][10] and rituximab combined with chemotherapy [9][10][11][12][13]. Splenectomy is preferable from a diagnostic perspective and gives a rapid relief of symptoms (i.e., abdominal discomfort and cytopaenias related to splenic sequestration) [13].…”

New Insights into the Biology and Diagnosis of Splenic Marginal Zone Lymphomas

“…In these cases, therapeutic options are splenectomy and rituximab, or conventional chemotherapy such as CHOP (rituximab, cyclophosphamide, doxorubicin, and vincristine). Response to splenectomy with correction of cytopenia occurs in approximately 90% of patients [ 10 ] and has been associated with statistically significantly better overall survival and progression-free survival than non-splenectomized patients in a recent study [ 11 ].…”

“…Ibrutinib has proved its efficacy in CLL compared to placebo [102], and CLL is characterized by an immunosuppressive environment [103]. As previously shown, ibrutinib has also shown interesting results in relapsed or refractory SMZL, with an overall response rate of 62% [11].…”