Case Presentation: A 45-year-old male with a history of smoking and hypertension presented to his primary care provider for progressive left-sided pleuritic chest pain without night sweats or fever and reported 2 months of intentional weight loss with diet and exercise. He was sent for an outpatient CT which revealed splenomegaly of 16x19cm with possible area of internal hemorrhage and active extravasation of the splenic artery, which was then embolized. The concern for the splenic hematoma was initially believed to be due to trauma as the patient had multiple episodes of falls or due to primary splenic lymphoma. PT/PTT, platelet count, and von Willebrand factor (vWF) antigen and Ristocetin cofactor activity were within normal limits. There was also no history of a personal or family history of a bleeding disorder. Peripheral blood flow cytometry did not reveal any abnormal or diagnostic phenotype. After embolization, the patient complained of persistent, severe left upper quadrant pain associated with left shoulder pain and endorsed bilateral axillary and groin lymphadenopathy during outpatient follow up without fever, chills, nausea or vomiting. The patient was readmitted 12 days post-embolization for persistent abdominal pain and presumption of urgent splenectomy, which was complicated by pancytopenia with thrombocytopenia to 54K/uL, leukopenia to 1.56, LDH to 7920 and CRP elevated at 31.6. The patient was given pneumococcal, meningococcal and H. influenzae vaccinations, and an open splenectomy was performed. CT revealed generalized LAD and multiple liver lesions with metastatic disease. The pathology report was consistent with Diffuse Large B-cell Lymphoma (DLBCL), BCL2+, BLC6+, MUM1+, weakly + CD5, kappa predominance CD3 and CD5 stain T-cells and ki-67 index of 70-80%. cMYC negative, Cyclin D1 negative, weakly positive. Bone marrow biopsy with positive B-cell gene arrangement. The patient was then started on Rituximab and prednisone 100mg po daily for 5 days, vaccinations and dose-adjusted CHOP on for hepatic function.

Discussion: Diffuse Large B-Cell Lymphomas account for most non-Hodgkin’s lymphomas. However, Primary Splenic DLBCL are exceedingly rare and typically present with abdominal pain and splenomegaly, with emergency splenectomy as the most effective therapeutic and diagnostic tool. The initial presentation of our patient was thought to be secondary to splenic hematoma secondary to falls. However, due to inconsistent diagnostic workup and persistent abdominal pain, a splenectomy was necessary. Primary Splenic DLBCL encompass about 1% of non-Hodgkin’s Lymphomas and the literature describing these processes are limited. Primary Splenic DLBCL can be further divided into three stages: 1) confinement to the spleen, 2) presence of hilar lymphadenopathy and 3) extra-splenic nodal or hepatic involvement. Abdominal pain has been described to be the most common presenting symptom with elevated LDH and CRP amongst the most frequently elevated laboratory markers with mean ki-67 index at 72% with mean age of diagnosis at 56.6, which is younger than mean age of that of all types of DBCL (70 years old).

Conclusions: While Diffuse B-Cell Lymphomas comprise the majority of non-Hodgkin Lymphomas, Primary Splenic DLBCL are a rare manifestation of this malignancy. In patients presenting with abdominal pain and splenomegaly, it is important to consider this diagnosis in the differential.

IMAGE 1: CT of the abdomen and pelvis showing significant enlargement of the spleen measuring 19 cm in oblique AP diameter and 16 cm in craniocaudal length.

IMAGE 2: CT of the chest, abdomen, pelvis showing extensive lymphadenopathy in bilateral axillary region. The largest lymph node in the right axillary group measures approximately 4.3 x 3.4 cm. The largest lymph node in the left axilla measures approximately 4.3 x 2.7 cm.