Primary Splenic Lymphoma Research Articles

Primary splenic diffuse large B-cell lymphoma (PS-DLBCL) presenting in an asymptomatic patient after sports trauma

Primary splenic diffuse large cell lymphoma is unusual and accounts for 2% of all splenic lymphomas. The symptoms included left upper abdominal pain, indigestion, loss of appetite, generalized weakness, fever, anemia, and thrombocytopenia or elevated C Reactive Protein. The current case report, to our knowledge the first of its kind, presents unusual clinicopathologic findings, where the patient remained asymptomatic until the time of diagnosis but showed extensive splenic involvement with stage IV disease. Alternatively, the morphologic findings in this case were like to those seen in other Primary Splenic Diffuse Large B Cell Lymphoma reported in the literature. In conclusion, the present report emphasizes the need for additional case studies and research on the biology of this unusual subset of lymphoma.

Laparoscopic Partial Splenectomy May Be Valuable for the Diagnosis of Malignant Lymphoma: A Case Report.

Diagnosing primary splenic malignant lymphoma (PSML) is challenging due to the non-specific nature of splenomegaly, necessitating splenic biopsy for confirmation. However, performing partial splenic resection for diagnostic purposes is an elective procedure due to the risk of major hemorrhage. Despite the longstanding practice of splenectomy over the past few decades, it remains invasive and may result in severe early or late complications. Hence, we present laparoscopic partial splenectomy (LPS) in a patient suspicious of PSML for diagnostic purposes in this study. An 81-year-old woman presented to our hospital with a one-month history of fever and dry cough. Atypical cells had been detected in her peripheral blood nine months ago. However, at that time, a bone marrow examination did not reveal any atypical cells. The laboratory tests revealed a soluble interleukin receptor-2 levels of 4,667 U/dl and atypical cells were also found in peripheral blood. Abdominal computed tomography showed splenomegaly without any other relevant findings. These findings are suspicious of PSML and LPS without vessel ligation was performed and a small fraction of the spleen from the inferior pole measuring 1.8×1.0 cm was resected. The operation lasted for 63 min with minimal estimated blood loss. Histopathological findings were compatible with the diagnosis of diffuse B-cell lymphoma. The postoperative clinical course was uneventful, and splenomegaly demonstrated improvement six months after the operation. LPS without vessel ligation for biopsy may be valuable for the diagnosis of malignant lymphoma, particularly when there are no swollen lymph nodes, as it offers a less invasive approach.

Primary Splenic Hodgkin Lymphoma and its Prognosis: A Report of Two Cases

Primary Splenic Lymphomas (PSL) constitute an extremely uncommon variety of splenic neoplasm and can present with grave complications such as splenic rupture. Hodgkin Lymphomas (HL), a type of hematopoietic neoplasm, are typically diagnosed between the ages of 20 and 30 years and present with supra-diaphragmatic lymphadenopathy, often accompanied by systemic B symptoms. A histopathological diagnosis involving Reed-Sternberg (RS) cells in an inflammatory background is crucial. However, other differentials such as reactive hyperplasia, infectious mononucleosis, anaplastic large cell lymphoma, or various other lymphomas may mimic Hodgkin disease both clinically and histologically. Therefore, accurate diagnostic evaluation of Hodgkin lymphoma is crucial, especially as it is highly curable with combination chemotherapy, even in higher stage disease. In this report, the authors present two cases of primary splenic HL diagnosed through histopathological and immunohistochemical examination of splenectomy specimens from January 2020 to December 2021 at the Department of Pathology, IPGME&R, Kolkata, India. While reporting the histopathological sections, the authors also considered other differentials such as reactive changes, tuberculosis, and other non-Hodgkin lymphomas. Both patients were male, aged 10 years and 18 years, and presented with abdominal distension and fever, without any palpable peripheral lymph nodes, for the last six months to one year. After chemotherapy, the patients were regularly monitored to identify any signs of recurrence or relapse. Therefore, the initial recognition and proper diagnosis of Hodgkin lymphoma presenting in the spleen may vary in clinical presentation and morphology, but early accurate diagnosis carries a good prognosis, as survival is best determined by its histopathological type.

An Unusual Case of a Central Pontine Myelinolysis Associated with Primary Splenic Diffuse Large B-cell Lymphoma.

A 54-year-old woman with persistent fatigue and a high fever presented with central pontine myelinolysis (CPM) on brain imaging, despite the absence of neurological symptoms. The patient had a slightly low serum sodium concentration. Further investigation led to a diagnosis of primary splenic diffuse large B-cell lymphoma (PS-DLBCL). Brainstem auditory-evoked potentials (BAEP) indicated minor abnormalities. CPM was successfully resolved after splenectomy and immunochemotherapy. This is the first reported case of CPM in a patient with PS-DLBCL confirmed by a pathological diagnosis following splenectomy and BAEP-detected abnormalities that improved with immunochemotherapy.

FRI694 Hypercalcemia And Renal Failure As Initial Presentation Of A Rare Case Of Primary Splenic Large B Cell Lymphoma

Abstract Disclosure: N. Ding: None. S.K. Majumdar: None. Background: Hypercalcemia can be seen in patients with malignancy. However, primary splenic large B cell lymphoma presenting with hypercalcemia and renal failure is rare. Clinical Case: A 75-year-old male with PMH of migraine headaches and hearing loss was sent to the hospital by his primary care doctor due to lab abnormalities, including hypercalcemia and renal failure. He also had abdominal pain, weakness, polyuria, and weight loss for several weeks. He took calcium 1000 mg and vitamin D 5000 units daily. Laboratory tests showed creatinine 2.8 (0.4-1.3) mg/dL, calcium 13.8 (8.8-10.2) mg/dL, ionized calcium 6.75 (4.6-5.08) mg/dL, phosphorus 3.6 (2.2-4.5) mg/dL, PTH 12.6 (15-65) pg/mL, PTHrP 23 (14-27) pg/mL, 25-hydroxy vitamin D 53 (30-100) ng/mL, 1,25-dihydroxy vitamin D 171 (18-72) ng/L, CTX collagen C-telopeptide 1137 pg/ml, SPEP without significant abnormality, UPEP with Bence-Jones proteinuria. Skeletal series showed no suspicious lesions, but MRI abdomen revealed an 11 cm splenic mass. Patient underwent splenectomy and pathology revealed diffuse large B-cell lymphoma. Following surgery, his hypercalcemia resolved without additional interventions. 1,25-dihydroxy vitamin D reduced to 25 ng/l and CTX reduced to 451 pg/ml. Further workup, including bone marrow biopsy and imaging, were negative. Patient was subsequently diagnosed with primary splenic large B cell lymphoma. He completed R-CHOP therapy and had no hypercalcemia in follow-up labs. Conclusion: Calcitriol-mediated hypercalcemia occurs in less than 1% of lymphomas, and primary splenic lymphoma constitutes less than 1% of non-Hodgkin lymphomas. The case represents an extremely rare etiology for hypercalcemia. Interestingly, elevated 1,25-dihydroxy vitamin D and hypercalcemia resolved after splenectomy. Clinicians should consider lymphoma as a potential cause of hypercalcemia and renal failure in elderly patients. Presentation: Friday, June 16, 2023

ABCL-451 Unusual Presentation of Primary Splenic Lymphoma: A Case Report

ABCL-451 Unusual Presentation of Primary Splenic Lymphoma: A Case Report

Primary Splenic Diffuse Large B-Cell Lymphoma: A Case Report.

The differential diagnosis of splenic nodules or infiltrates is vast and challenging, and it includes haematological diseases, systemic infectious diseases but also non-malignant infiltrative diseases.Although some lymphomas frequently present with splenomegaly, this is not the case of DLBCL, with the exception of PS-DLBCL.PS-DLBCL is a very rare pathology, accounting for 1% of all DLBCL and less than 1% of all NHL.

FDG PET/CT in Aseptic Splenic Abscess

HomeRadiologyRecently Published PreviousNext Reviews and CommentaryFree AccessImages in RadiologyFDG PET/CT in Aseptic Splenic AbscessWeiting Liu, Xiaohong Ou Weiting Liu, Xiaohong Ou Author AffiliationsFrom the Department of Nuclear Medicine, West China Hospital of Sichuan University, No. 37 Guoxue Alley, Chengdu 610041, People’s Republic of China.Address correspondence to X.O. (email: [email protected]).Weiting LiuXiaohong Ou Published Online:Mar 28 2023https://doi.org/10.1148/radiol.222955MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In A 32-year-old woman presented with fever and arthralgia for 1 month. She had a past medical history of rheumatoid arthritis with 2 years of glucocorticoid treatment. At physical examination, splenomegaly was found. Her laboratory data showed leukocytosis (15.86 × 109/L; reference range, 3.5–9.5 × 109/L) and a high erythrocyte sedimentation rate. Serial blood cultures were negative. Intravenous antibiotics and corticosteroid therapy did not relieve her symptoms. Fluorodeoxyglucose (FDG)-PET/CT examination was performed, showing abnormal standardized uptake values in the spleen (Figure). Primary splenic lymphoma was suspected, and the patient underwent laparoscopic splenectomy. At pathologic examination (Figure), the diagnosis of aseptic splenic abscesses as a complication of rheumatoid arthritis was made. Corticosteroids and supportive treatment were then initiated, and her clinical condition gradually improved.Images in a 32-year-old woman presenting with fever and arthralgia for 1 month. She had a past medical history of rheumatoid arthritis with 2 years of glucocorticoid treatment. A fluorodeoxyglucose (FDG)-PET examination was performed. (A) Maximum intensity projection whole body image shows intense diffuse splenic uptake and moderate bone marrow uptake. (B) Axial-fused PET/CT abdominal image shows an enlarged spleen with heterogeneously increased FDG standardized maximum uptake value (standardized maximum uptake value,13.94; liver standardized maximum uptake value, 3.76). At pathologic examination, macroscopic examination of the spleen showed many nonconfluent white nodules (not shown). (C) With use of hematoxylin and eosin, a photomicrograph with 400× magnification shows numerous areas of necrosis surrounded by intense polymorphonuclear cell infiltration (arrows) and fibroblastic proliferation at the periphery of the splenic nodules.Download as PowerPointOpen in Image Viewer Splenic abscesses generally occur in patients with immune dysfunction (1), presenting as solitary or multifocal lesions. Aseptic abscesses are most commonly associated with nonspecific inflammatory bowel disease and other autoimmune diseases (spondyloarthropathy, rheumatoid arthritis, lupus erythematosus, and nodular arteritis), or autoinflammatory (synovitis, acne, pustulosis, hyperostosis, and osteitis) and neoplastic ones (myeloma, monoclonal gammopathy) (2). Histopathologic examination helps to exclude other conditions. The cornerstones of aseptic abscesses are mature polymorphonuclear neutrophils in the center of the splenic nodules, with histiocytes and single giant cells present at the periphery (2). It is unclear whether glucocorticoids could be a factor that promotes the formation of aseptic splenic abscesses. The presence of disseminated sterile splenic microabscesses with intense FDG uptake related to rheumatoid arthritis is rare.Disclosures of conflicts of interest: W.L. No relevant relationships. X.O. No relevant relationships.

Carcinogenesis Observed in the Spleens of Balb/c Mice After Head-neutron Irradiation.

To investigate the long-term influence of head-neutron irradiation on mice spleens, post-radiation late effects were examined in three types of mice: Balb/c and severe combined immunodeficiency (SCID) mice, which have high radio-sensitivities, and C3H mice. Neutron irradiation was performed with the neutron beam of the Kyoto University Research Reactor. Survival fractions and the change in spleen size after head-neutron irradiation were investigated in three different types of mice. Physical condition after neutron irradiation was observed for eighteen months. The onset of primary splenic malignant lymphoma was recognized in many of the Balb/c mice 18 months after head-neutron irradiation. Eight months after head-neutron irradiation, many SCID mice developed an abscess in the part exposed to radiation and spleen swelling. The swollen spleen of SCID mice had hematopoiesis from the marrow. Low energy head-neutron irradiation damages immune organs in radiosensitive SCID and Balb/c mice. A combination of boron neutron capture therapy and immunotherapy may be less toxic than low-energy neutron-irradiation alone.

Splenic Lymphomas: A Tertiary Care Center Experience and Review of Literature.

Primary splenic lymphomas are rare with the majority of lymphomas in spleen being secondary to an extra-splenic lymphoma. We aimed to analyze the epidemiological profile of the splenic lymphoma and review the literature. This was a retrospective study including all splenectomies and splenic biopsies from 2015 to September 2021. All the cases were retrieved from Department of Pathology. Detailed histopathological, clinical and demographic evaluation was done. All the lymphomas were classified according to WHO 2016 classification. A total of 714 splenectomies were performed for a variety of benign causes, as part of tumor resections and for the diagnosis of lymphoma. Few core biopsies were also included. A total of 33 lymphomasdiagnosed in the spleen, primary splenic lymphomas constituted 84.84% (n = 28) of the cohort with 5 (15.15%) having the primary site elsewhere. The primary splenic lymphomas constituted 0.28% of all the lymphomas arising at various sites. Adult population (19-65years) formed the bulk (78.78%) with a slight male preponderance. Splenic marginal zone lymphomas (n = 15, 45.45%) comprised of major proportion of cases followed by primary splenic diffuse large B-cell lymphoma (n = 4, 12.12%). Splenectomy was the main course of treatment for SMZL with a good overall outcome, with chemotherapy ± radiotherapy forming the mainstay in other lymphomas. Lymphomas in spleen can be infiltrative or a primary, hence proper clinic-radiological and pathological evaluation is required. Appropriate management is guided by the precise and detailed evaluation by the pathologist, requiring understanding of the same.

Unusual Rapid Growth of Primary Splenic Diffuse Large B-Cell Lymphoma with Extensive Necrosis.

The primary splenic lymphoma is extremely uncommon with an incidence rate of <1% of all the lymphomas under the strict criteria for diagnosis expounded by Das Gupta et al. Clinical presentations of nonspecific symptoms are weight loss, weakness, fever, and left upper quadrant pain or discomfort due to enlarged spleen. Abdominal ultrasound and CT are the most widely used imaging modality for the assessment of lymphoma. The imaged features of splenic lymphoma are nonspecific; typical lymphoma presents as a diffusely enlarged spleen. The abdominal CT scan in our case showed a large cystic splenic mass measuring 14 cm without enhancement after contrast medium. Lymphoma is often described as an aggressive tumor because its rapid doubling time can quickly increase the size of a tumor. In our case, the tumor grew to more than 100 times its original size in 4 months. So, we present this unusual rapid growth of primary splenic lymphoma.

An Unusual Differential Diagnosis of Gastric Haemorrhage: A Rare Case of Gastrosplenic Fistula.

A gastrosplenic fistula is a rare complication of primary splenic lymphoma and a rare cause of massive upper gastrointestinal haemorrhage. We report a case of a spontaneous gastrosplenic fistula secondary to splenic large B-cell lymphoma.The patient was admitted to the emergency department with haematemesis. Oesophagogastroduodenoscopy showed a deep gastric ulcer, and a subsequent CT scan revealed a gastrosplenic fistula. Gastric biopsy demonstrated gastric mucosa with infiltration by large lymphoid cells. A multidisciplinary discussion on the management of this case was conducted.Primary surgical treatment of the fistula was not deemed indicated because the bleeding had stopped. The patient was stabilized, transfused, and then transferred to the oncology unit for chemotherapy.During hospitalization, lung metastases were found but the progressive worsening of the patient’s general condition contraindicated chemotherapy. She was transferred to a hospice and died 2 months later of neoplastic cachexia.Gastrosplenic fistula is a rare condition. Prompt recognition of the underlying pathology can save the patient’s life. We aim to highlight this rare complication of splenic lymphoma, discuss the presenting signs and symptoms, and explore the management options.LEARNING POINTSA gastrosplenic fistula is a rare complication of primary splenic lymphoma.It can cause massive upper gastrointestinal haemorrhage.Our patient was managed without surgery but died 2 months later from neoplastic cachexia.

Primary Splenic Lymphoma with Splenic Hilar Lymphadenopathy

Splenic lymphoma can occur as a primary or secondary malignancy, but the latter form is more common. Primary splenic lymphoma is defined as lymphomatous involvement of the spleen with or without splenic hilar lymphadenopathy. It can present as splenomegaly with homogenous echogenicity without a focal lesion, a diffuse infiltrative lesion with miliary lesions, multiple focal nodular lesions, or as a large solitary mass. A 71-year-old woman presented with mild upper abdominal pain and a splenic mass. We visualized a hypoechoic splenic mass with splenic hilar lymphadenopathy on abdominal ultrasound. The patient underwent surgery and was diagnosed with splenic diffuse large B-cell lymphoma.

Primary splenic lymphoma discovered on massive splenomegaly: A case report.

Introduction and importanceMalignant lymphoma occurs in all the systemic organs. Rarely, large B-cell lymphoma is located in the spleen, making the diagnosis difficult.Herein, we report a patient presenting with massive splenomegaly due to LBCL. Splenectomy was essential to assess the diagnosis and to guide postoperative therapeutics.Presentation of a caseA 47-year-old woman, with no comorbidities, complained of weight loss and abdominal pain. She had a palpable spleen that extended below the navel.CT scan revealed massive splenomegaly and lymph nodes in the spleen hilum. Splenectomy was performed.Histopathological examination confirmed the diagnosis of large B-cell lymphoma.The postoperative course was uneventful. Three courses of chemotherapy were given. The patient was in remission after a follow-up of 8 months.DiscussionMassive splenomegaly can be one of the circumstances of the discovery of large B-cell lymphoma.Splenectomy was then essential to confirm the diagnosis and to guide postoperative therapeutics. It also permits reducing hypersplenism and preventing spleen rupture.In patients with high operative risk, splenic needle biopsy should be taken into consideration.Splenic artery embolization before surgery can also be performed in patients having massive splenomegaly to reduce the spleen volume.We highlight the importance of splenectomy to confirm the diagnosis and to relieve the symptoms. Postoperative chemotherapy is essential to prevent relapses.ConclusionSplenectomy is essential in spleen localized large B-cell lymphoma. It permits to confirm the diagnosis, relieve symptoms, and treatment of underlying hematologic malignancies. Postoperative chemotherapy is essential to prevent relapses.

Frequency of Paroxysmal Nocturnal Hemoglobinuria in Patients with Lymphoma

Objective: To investigate the frequency of paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal hematopoietic stem cell disease resulting in complement-mediated hemolysis, in patients with lymphoma by flow cytometry.Methods: Fifty patients with lymphoma who were admitted to the hematology clinic, newly diagnosed and not yet treated were included in this study conducted in 2014. The presence of PNH clones was checked by FLAER flow cytometry method in peripheral blood samples. FLAER is a non-hemolytic fluorescently labeled inactive toxin aerolysin method that can detect up to 0.5% of PNH cells instead of bacterial toxin aerolysin, which binds to RBCs via the GPI anchor and initiates hemolysis for PNH screening or PNH clone detection. With this technique, PNH clones in all hematopoietic cell lines can be detected in an assay.Results: PNH clone was observed over 10% in two patients, one male and the other female. However, no hemolysis was found in patients with PNH clones. The lymphoma subtypes of the patients with positive PNH clone were B-cell small-cell lymphocytic lymphoma in the male patient and primary splenic lymphoma in the female patient.Conclusion: PNH or PNH-like disorders accompanying hematological malignancies, especially lymphomas, are not very common in the literature. There is a need to elucidate the relationship between hematological malignancies and PNH with the help of more advanced molecular techniques.

IGH/BCL2 Status Better Predicts Clinico-Pathological Behavior in Primary Splenic Follicular Lymphoma than Histological Grade and Other Molecular Markers.

Splenic lymphoma may be primary or secondary. Primary splenic lymphoma’s are rare and usually of follicular cell origin representing <1% of Non-Hodgkin’s Lymphoma’s. Most are secondary with 35% representing Marginal Cell sub-type with the rest being Diffuse Large B-Cell Lymphoma’s. Unlike the uniformly aggressive clinical course of Diffuse Large B-Cell Lymphoma’s, biological behavior of Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma remains less well defined. We present here a solitary splenic mass confirmed as Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma after a diagnostic splenectomy. Biopsy revealed monomorphic small lymphoid cells with low grade mitotic activity. Flow cytometry showed a lambda restricted population of B-Cells displaying dim CD19 and CD10. The cells were negative for CD5, CD11c, and CD103. FISH was negative for IGH/BCL2 fusion unlike nodal Follicular Lymphoma’s which are usually positive for this translocation. Evidence from this case and a review of literature support the finding that Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma is less likely to have the classic IGH-BCL2 fusion and the associated chromosomal 14;18 translocation. This profile is associated with less aggressive clinical behavior even when histopathology represents a high-grade pattern. In such cases splenectomy alone is adequate for localized disease when negative for IGH/BCL2 fusion regardless of histological grade.

A Case Report on Splenic Marginal Zone Lymphoma

Background: Out of the various malignant tumours originating from the lymphatic hematopoietic system, lymphoma is one such important entity. It is divided into Non-Hodgkin’s Lymphoma (NHL) and Hodgkin Lymphoma (HL) depending on its cell source. A very rare type of malignant variant of lymphoma is the primary splenic lymphoma, involving exclusively the spleen and splenic hilar lymph nodes. Moreover, splenic marginal zone lymphoma (SMZL) is even more infrequent. SMZL is an uncommon chronic B lymphocyte proliferative disease, which only accounts for about 1–2% of all non-Hodgkin’s lymphoma. The mean age of SMZL incidence is about 65 years. There is no known significant gender predominance. A quarter of patients with early diagnosed SMZL have known to have vague symptoms like abdominal pain and distention; and other patients may be accompanied by loss of weight, malaise, cachexia, splenomegaly, or other manifestations.&#x0D; Conclusion: Although, a good prognostic outcome is what is usually expected from most patients of Splenic Marginal Zone Lymphoma who undergo splenectomise, an aggressive transformation leading to a worse direction cannot be ruled out. SMZL is very challenging to be diagnosed pre-operatively due to the lack of specificity in clinical presentation.

Low-Grade Primary Splenic CD10-Positive Small B-Cell Lymphoma/Follicular Lymphoma.

Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas (FL), lymphoplasmacytic lymphoma and chronic lymphocytic leukemia/ small lymphocytic lymphoma, particularly as part of advanced stage disease, are more common. Indolent B cell lymphomas expressing CD10 almost always represent FL, which in its primary splenic form is the focus of this review. Primary splenic follicular lymphoma (PSFL) is exceedingly infrequent. This type of lymphoproliferative disorder is understudied and, in most cases, clinically characterized by splenomegaly or cytopenias related to hypersplenism. The diagnosis requires correlation of histopathology of spleen, blood and/or bone marrow with the correct immunophenotype (determined by flow cytometry and/or immunohistochemistry) and if necessary, additional molecular profiling. Management of this incurable disease is evolving, and splenectomy remains the mainstream treatment for stage I PSFL.

S2361 Primary Splenic Lymphoma Complicated by Spontaneous Gastro-Splenic Fistula Presenting as Upper GI Bleeding: A Case Report

S2361 Primary Splenic Lymphoma Complicated by Spontaneous Gastro-Splenic Fistula Presenting as Upper GI Bleeding: A Case Report

The effect of surgery on primary splenic lymphoma: A study based on SEER database.

BackgroundAlthough primary splenic lymphoma (PSL) is rare, it ranks first among splenic primary malignant cancers, and the incidence of lymphoma of spleen has gradually increased in recent years. However, the efficacy of surgery for PSL has not been clinically verified by large sample data, which has affected the formulation of relevant guidelines.AimTo assess whether surgery can enhance the prognosis PSL patients.MethodsExtracted the data of patients with PSL from The Surveillance, Epidemiology, and End Results (SEER) database, and divided the patients into surgery and non‐surgery group. Kaplan–Meier curves and log‐rank tests were used to compare the overall survival (OS) and cancer‐specific survival (CSS). The propensity score matching (PSM) was used to match the data, then compared the OS and CSS again. The COX proportional hazard regression model was used for univariate and multivariate analysis. Finally, we performed subgroup analysis in different Ahmann stages.ResultsA sum of 2207 patients with PSL were enrolled, of which 1062 (48.1%) patients received surgery, and 1145 (51.9%) patients did not undergo surgery. Overall, patients in the surgery group had better OS and CSS. After the propensity scores matching, surgery was not statistically significant in OS and CSS. In the subgroup analysis, surgery was a protective factor for the OS and CSS in Ahmann I/II. However, surgery was no statistical significance in OS and CSS in Ahmann III. In patients with Ahmann Ⅰ/Ⅱ SMZL, surgery was a protective factor for OS and CSS. In patients with Ahmann Ⅲ SMZL, surgery was also statistically significant of OS and CSS.ConclusionsSurgery can significantly improve the prognosis of patients with Ahmann Ⅰ/Ⅱ primary splenic lymphoma, but there was no survival difference in the Ahmann Ⅲ patients with or without surgery. For patients with SMZL, surgery was effective for improving OS and CSS.