Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases (original) (raw)

Primary splenic diffuse large B-cell lymphoma: a case report

European Journal of Case Reports in Internal Medicine, 2023

Primary splenic lymphoma (PSL) is a rare disease and an improbable cause of splenomegaly or splenic nodules. On the contrary, splenic secondary involvement as part of an advanced lymphoproliferative disorder is more common. The authors present the case of a 49-year-old woman with a primary splenic diffuse large B-cell lymphoma (PS-DLBCL), in which the absence of other organs' involvement determined an ultrasound-guided biopsy of the spleen to achieve a definitive diagnosis. With this case the authors intend to emphasise the extensive differential diagnosis of splenomegaly, splenic nodules or infiltrates, the usefulness of splenic biopsy in establishing the diagnosis and recall a rare disease, with non-specific presenting symptoms, in which the diagnostic workup is challenging.

Primary Splenic Diffuse Large B Cell Lymphoma. Case Report and Literature Review

SN Comprehensive Clinical Medicine, 2020

We report a case of a localized, massive, diffuse large B cell splenic lymphoma diagnosed by splenectomy. A 61-year-old man complaining of abdominal pain was admitted to our hospital. On abdominal tomography examination, there was seen to be a significant increase in the spleen dimensions and a lesion of heterogenous density was observed to be covering almost all of the spleen parenchyma. Intraoperatively, a massive hematoma involving approximately 95% of the spleen and ischemic areas were observed, so it was decided to perform splenectomy. With an improvement in general condition and no active complaints, the patient was discharged, but then failed to attend for follow-up examinations. At 7 months after the splenectomy, the patient again presented at the Emergency Department with complaints of abdominal pain, nausea, and vomiting. On abdominal tomography, a solid mass was observed, approximately 12 × 9 cm in size with irregular contours in the margins of the splenectomy location. The diagnosis was reported as non-germinal center diffuse large B cell lymphoma showing involvement with the colon and small intestine segments. It was learned that the patient had not attended follow-up examinations and when the splenectomy material of 17.5 × 13.5 × 6 cm was examined, it was reported as non-germinal center diffuse large B cell lymphoma. This case is of importance in respect of the development of recurrence in less than a year as splenectomy only without systemic chemotherapy was insufficient in the treatment of diffuse large B cell lymphoma with isolated spleen involvement.

Bone Marrow Histopathology in the Diagnostic Evaluation of Splenic Marginal-zone and Splenic Diffuse Red Pulp Small B-cell Lymphoma

American Journal of Surgical Pathology, 2012

Primary splenic small B-cell lymphomas mostly comprise the distinct entity of splenic marginal-zone lymphoma (SMZL) and the provisional category of splenic lymphoma/leukemia unclassifiable, mainly represented by the hairy cell leukemia variant and splenic diffuse red pulp small B-cell lymphoma (SDRL). Until recently, histopathologic examination of splenectomy specimens was considered mandatory for the diagnosis of SMZL. However, nowadays, mainly because of advances in chemoimmunotherapy, splenectomy is performed much less frequently. We evaluated the diagnostic efficacy of bone marrow biopsy (BMB) histopathology in the diagnostic approach toward SMZL and SDRL and tested whether it may serve as a substitute for spleen histopathology in the differential diagnosis between these 2 entities. To this end, we conducted a paired assessment of BMB and spleen diagnostic samples from 46 cases with a diagnosis of SMZL (n = 32) or SDRL (n = 14) based on spleen histopathology. We demonstrate that detailed immunohistopathologic BMB evaluation offers adequate evidence for the confirmation of these entities and their differential diagnosis from other small B-cell lymphoma histotypes. Notably, the immunophenotypical profile of SMZL and SDRL was identical in both BMB and spleen specimens for 21 evaluated markers. Paired assessment of BMB and spleen specimens did not identify discriminating patterns of BMB infiltration, cytology, and/ or immunohistology between SMZL and SDRL. Accordingly, bone marrow histopathology contributes significantly in confirming the diagnosis of SMZL and SDRL. However, presently it is not possible to distinguish SMZL from SDRL on the basis of BMB evaluation alone; hence, histopathologic examination of the spleen remains the "gold standard" approach.

Diffuse Large B cell Lymphoma with Primary Spleen Involvement: Report of Three Cases

Medicine Science | International Medical Journal, 2014

The spleen is the primary organ of lymphoma in only 1-2 % of all lymphoma patients. We presented three cases of primary splenic involvement of lymphoma that were treated. Presentation of cases: In the first case, four solid lesions were detected, and in the second case, a hypodense lesion of 3 cm was detected in the spleen. Two cases underwent splenectomy. No complication and recurrence were observed during 22-21 months followup, respectively. In the third case, a splenic mass invading pancreas was detected. Splenectomy and distal pancreatectomy were performed. No recurrence was observed during 8-year follow-up. In postoperative period, all cases were treated with cyclophosphamide, vincristine, doxorubicin, prednisone plus rituximab for 6 cycles. Primary splenic lymphoma refers to the involvement of the spleen only or with splenic hilar lymph node or local invasion without liver involvement. The pathologic diagnosis was diffuse large B-cell non-Hodgkin's lymphoma in the all cases. Splenic mass biopsy has some complication risks. Aspiration biopsy of splenic mass was performed in one of the three cases. Invasions should be evaluated in terms of malignity during splenectomy, and liver biopsy and biopsies of lymph nodes can be performed in case of lymphoma. Additionally diagnosis should be confirmed via postoperative bone marrow biopsy and positron emission tomography screening. Primary splenic involved lymphoma must be kept in mind for differential diagnosis in cases with splenic mass. Splenectomy is one of the most common modality for primary splenic lymphomas in terms of both diagnostic and curative treatment.

Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens

Journal of Clinical Pathology, 1987

The clinical, haematological, morphological and histological features of a series of 22 patients presenting with splenic lymphoma with circulating villous lymphocytes were assessed and compared with those of patients with other forms of chronic B cell leukaemia in an attempt to differentiate this condition from hairy cell leukaemia, prolymphocytic leukaemia, and chronic lymphocytic leukaemia, with which this condition has many features in common. The disease was twice as common in men than in women, with a mean (SD) age at diagnosis of 72 (9) years, and the most consistent presenting feature was massive enlargement of the spleen, which showed white and red pulp disease with a plasmacytic component. Small monoclonal bands were found in 60% of cases.

Primary splenic diffuse large B‐cell lymphoma presenting as a splenic abscess

eJHaem

Diffuse large B-cell lymphoma (DLBCL) arising in the spleen, also known as primary splenic DLBCL (PS-DLBCL), is a rare form of malignant lymphoma. It is defined as a lymphoma confined to the spleen or involving splenic hilar lymph nodes. Here we report a case of PS-DLBCL with CD30. The patient was a 62-year-old who presented with 2 weeks of left flank pain, chills, and abdominal distension. Computed tomography identified an 8-cm splenic mass with central necrosis interpreted as an abscess. A drain was placed, yielding purulent necrotic material; cytologically, only neutrophils were identified. However, purulent drainage continued for 28 days without resolution, prompting splenectomy. Pathological dissection revealed a multinodular mass with central necrosis. Microscopic examination revealed extensive karyorrhexis, abundant ghosts of large cells, and scattered large cells with pleomorphic, multilobated, and vesicular nuclei with moderately abundant cytoplasm. Immunohistochemical staining revealed large,

Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?

Blood, 2007

The presence of circulating villous lymphocytes (VLs) in lymphoma patients usually points to splenic marginal zone B-cell lymphoma (SMZL), even if the VLs can be found occasionally in other small B-cell lymphomas. However, those cells are variably described, and detailed cytologic characterization is often lacking. We identified lymphoma cases with numerous basophilic VLs among the large group of splenic lymphoma with VLs, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older men, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM + D, IgM + G, IgM or IgG, as well as CD76 and CD11c, frequently CD103, and rarely CD123. Spleen sections were peculiar, with atrophic white pulp and a monomorphic diffuse lymphoma infiltration in a congested red pulp. Bone marrow infiltration was interstitial and intrasinusoidal without extensive fibrosis. Cytogenetic analysis showed a ...

Diffuse large B-cell lymphoma presenting in the leukemic phase

Autopsy and Case Reports, 2016

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma comprising a heterogeneous group of disorders with variable histological and clinical behavior. Although other lymphomas may present in the leukemic phase more frequently, this appearance is unusually observed among DLBCL cases. Diagnosing lymphoma is not always easy, and the patient's clinical status quite often may hamper invasive procedures for diagnosis pushing the clinician to look for alternatives to reach the nearest possible accurate diagnosis. The authors report the case of a middle-aged man who presented the history of malaise, weight loss, and low-grade fever. The peripheral blood count showed leukocytosis with the presence of blasts and thrombocytopenia. The cytological morphology and immunophenotyping of the peripheral blood and bone marrow aspirate, as well as the bone marrow biopsy accompanied by a thorough immunohistochemical analysis, rendered the diagnosis of DLBCL in the leukemic phase. The patient was prescribed R-CHOP with a favorable outcome. Intra-abdominal lymph node biopsy was avoided because of the patient's critical medical condition. The authors highlight this rare form of presentation of DLBCL as well as the combination of peripheral blood, bone marrow aspirate, and bone marrow biopsy for reaching the diagnosis in cases were a lymph node sample is unavailable for the diagnostic work-up.