BackgroundPrimary splenic lymphoma (PSL) is a rare form of lymphoproliferative neoplasm, accounting for less than 2% of all lymphomas and approximately 1% of non-Hodgkin lymphomas. While the spleen is often involved in advanced lymphoproliferative disorders, its isolated primary involvement presents significant diagnostic challenges. PSL includes both indolent subtypes, such as splenic marginal zone lymphoma (SMZL), and aggressive forms such as diffuse large B-cell lymphoma (DLBCL).Case descriptionWe report two cases of PSL with distinct clinical and histological features. The first case involves a 65-year-old male with prior radioiodine treatment for thyroid carcinoma and occupational radiation exposure, who presented with progressive splenomegaly over four years. Splenectomy revealed SMZL characterised by infiltration of small, mature CD20-positive B-cells and a low proliferative index. The patient remains under observation without systemic treatment. The second case concerns a 78-year-old female with a history of breast cancer and hysterectomy, presenting with constitutional symptoms, splenomegaly and diffuse abdominal pain. Imaging demonstrated hypermetabolic splenic lesions and cervical lymphadenopathy. Splenectomy confirmed DLBCL with high Ki-67 index and CD20, BCL6 expression. Systemic chemotherapy with modified rituximab, cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine and prednisone (R-CHOP) was initiated.ConclusionThese cases underscore the diagnostic complexity of PSL, highlighting the importance of histopathological confirmation to distinguish between indolent and aggressive subtypes. In patients with isolated splenomegaly and non-specific systemic symptoms, timely evaluation – including imaging and splenectomy – is critical for accurate diagnosis and appropriate management.LEARNING POINTSUnexplained splenomegaly warrants consideration of primary splenic lymphoma in the differential diagnosis, even without lymphadenopathy or classic B symptoms, particularly when detected during abdominal imaging for hepatosplenic evaluation.Histopathologic and molecular confirmation is essential, as indolent subtypes (e.g. SMZL) and aggressive subtypes (e.g. DLBCL) may present identically but require fundamentally different treatment approaches.PET/CT-guided diagnostic strategy optimises outcomes by enabling accurate staging, identifying the most metabolically active site for biopsy, and informing the need for splenectomy versus direct systemic therapy.

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare subtype of B-cell lymphoma. NLPHL is usually indolent, involves lymph nodes and shows a favorable prognosis with high overall survival. In a minority of cases, patients may present and/or progress to advanced disease with involvement of the spleen, liver, and/or bone marrow. While splenic involvement by NLPHL is usually presumed evidence of advanced disease with poor prognosis, here we report to our knowledge, the first case of primary splenic NLPHL occurring in a child who showed no overt nodal disease and is currently free of disease 3 years post-splenectomy without additional treatment.

Aim: This study aimed to characterize canine splenic lymphoma through a comprehensive evaluation of gross, cytological, histopathological, and immunophenotypic features in both biopsy and necropsy specimens, and to distinguish primary splenic lymphomas from splenic involvement in multicentric disease. Study Design: An observational, descriptive investigation was undertaken on confirmed cases of canine splenic lymphoma submitted for routine diagnostic evaluation. Place and Duration of Study: The study was conducted in the Department of Veterinary Pathology, Madras Veterinary College, Chennai - 07, for one year (June 2011 to June 2012). Methodology: Eight canine cases were diagnosed as splenic lymphoma, comprising six multicentric diffuse large B-cell lymphomas (DLBCL) and two primary splenic B-cell lymphoblastic lymphomas (B-LBL). Specimens were obtained from surgically excised splenic biopsies and post-mortem examinations. Grossly, all spleens exhibited marked splenomegaly, with weights ranging from 120 to 560 g. Patient data revealed a mean age of 8.56 years, with no sex predilection and representation from multiple breeds. Cytological and histopathological classification was performed according to the updated Kiel and World Health Organization (WHO) criteria. Immunohistochemical analysis employed a Pan T-cell marker (CD3) and a B-cell marker (CD79a) to determine lineage. Results: All cases demonstrated a CD3-negative/CD79a-positive immunophenotype, consistent with high-grade B-cell lymphoma. Six cases represented splenic involvement in multicentric lymphoma, whereas two were classified as primary splenic lymphomas. The cytological study of lymphoma revealed homogenous clonal population of lymphoblasts with lymphoglandular bodies with variation in multiple nucleoli and scanty basophilic cytoplasm. Histology showed the white and red pulps were completely substituted by B-LBL neoplastic cells. Cells showed anisokaryosis, prominent nuclei, and chromatin margination. Neoplastic cell showing nuclear indentation with large, single, prominent nucleolus. Histomorphological features corresponded to their cytological classification, and gross lesions were characterized by diffuse enlargement and loss of normal splenic architecture. Conclusion: The integration of gross pathology, cytology, histopathology, and immunohistochemistry enables accurate subtyping of canine splenic lymphoma, with CD3 and CD79a serving as reliable markers for lineage determination. In this series, high-grade B-cell lymphoma was the predominant subtype, underscoring its clinical relevance and the value of immunophenotyping in routine diagnostic practice.

Introduction: Granulomatous inflammation has been reported in association with both Hodgkin and non- Hodgkin lymphomas. Its presence alongside lymphoma is uncommon and may complicate the diagnostic process. Case Report: We report the case of a 72-year-old male who presented to our hospital with weight loss, night sweats, and general deterioration. His medical history included urothelial carcinoma treated with Bacillus Calmette–Guérin (BCG) therapy in 2020. Clinical examination revealed splenomegaly extending to the flank. An excisional splenic biopsy confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) with associated granulomatous inflammation. Conclusion: This case illustrates an atypical presentation of DLBCL with granulomatous inflammation, emphasizing the need for careful histopathological assessment in such scenarios.

Diffuse large B-cell lymphoma (DLBCL) is the most prevalent and aggressive subtype of non-Hodgkin lymphoma comprising 30% to 40% of cases. While DLBCL frequently arises in the lymph nodes, up to 40% of cases originate in extranodal tissues. Primary splenic DLBCL is extremely rare comprising only ~1% of DLBCL cases and can be complicated by rare entities including gastrosplenic fistula (GSF). In contrast to DLBCL, polycythemia vera (PV) is a myeloproliferative malignancy. Polycythemia vera can transform into other hematologic malignancies including post-polycythemia myelofibrosis, but associations with DLBCL are uncommon. We present the first case of PV with concomitant primary splenic DLBCL complicated by GSF. While the majority of splenic lesions are benign, they have a broad differential diagnosis including malignant etiologies. Cystic or solid morphology, the number of lesions, and vascularity on imaging heavily guide further management. Due to concern for imminent massive upper gastrointestinal bleeding in the setting of GSF, our patient was diagnosed and managed with prompt splenectomy and gastrectomy. Percutaneous splenic biopsy is also a safe and effective diagnostic modality, but was deferred in our case given increased bleeding risk with PV. In conclusion, primary splenic lymphoma should be in the differential for a splenic mass regardless of whether the patient has a prior hematologic malignancy, and management should be prompt especially if complicated by a GSF.

Navigating Rarity: A Case Report on Primary Splenic Lymphoma

Two rare cases of primary splenic lymphoma with diffuse large B-cell and small B-cell type: an Indonesian report

Distinct Signaling Profiles in Primary Splenic Small B-Cell Lymphomas

Genetic Characterization of Marginal Zone Lymphomas in Tissue and cfDNA

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.

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 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.

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

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.

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.

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.

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.

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.

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.