Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report
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%...
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SAGE Publishing
2025-03-01
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| Series: | Journal of Investigative Medicine High Impact Case Reports |
| Online Access: | https://doi.org/10.1177/23247096251325409 |
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| author | Bibek Saha MD Jenny J. Cao MBBCh Zachary S. Kelm MD, PhD Allison Reinhardt MD Alex Danielson MD Victoria Kalinoski-Dubose MD William Mundell MD |
| author_facet | Bibek Saha MD Jenny J. Cao MBBCh Zachary S. Kelm MD, PhD Allison Reinhardt MD Alex Danielson MD Victoria Kalinoski-Dubose MD William Mundell MD |
| author_sort | Bibek Saha MD |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-bd41a68992f74998bfd89c1ee4aaa403 |
| institution | DOAJ |
| issn | 2324-7096 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Journal of Investigative Medicine High Impact Case Reports |
| spelling | doaj-art-bd41a68992f74998bfd89c1ee4aaa4032025-08-20T03:01:46ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962025-03-011310.1177/23247096251325409Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case ReportBibek Saha MD0Jenny J. Cao MBBCh1Zachary S. Kelm MD, PhD2Allison Reinhardt MD3Alex Danielson MD4Victoria Kalinoski-Dubose MD5William Mundell MD6Department of Internal Medicine, Mayo Clinic, Rochester, MN, USADepartment of Internal Medicine, Mayo Clinic, Rochester, MN, USADepartment of Radiology, Mayo Clinic, Rochester, MN, USADepartment of Internal Medicine, Mayo Clinic, Rochester, MN, USADepartment of Internal Medicine, Mayo Clinic, Rochester, MN, USADepartment of Internal Medicine, Mayo Clinic, Rochester, MN, USADepartment of Hospital Internal Medicine, Mayo Clinic, Rochester, MN, USADiffuse 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.https://doi.org/10.1177/23247096251325409 |
| spellingShingle | Bibek Saha MD Jenny J. Cao MBBCh Zachary S. Kelm MD, PhD Allison Reinhardt MD Alex Danielson MD Victoria Kalinoski-Dubose MD William Mundell MD Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report Journal of Investigative Medicine High Impact Case Reports |
| title | Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report |
| title_full | Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report |
| title_fullStr | Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report |
| title_full_unstemmed | Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report |
| title_short | Concomitant Polycythemia Vera and Primary Splenic Diffuse Large B-Cell Lymphoma Complicated by Gastrosplenic Fistula: A Case Report |
| title_sort | concomitant polycythemia vera and primary splenic diffuse large b cell lymphoma complicated by gastrosplenic fistula a case report |
| url | https://doi.org/10.1177/23247096251325409 |
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