Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens (original) (raw)
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Blood, 1994
Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chain restriction (kappa/lambda: 1.5/1) with moderate to strong intensity of membrane Ig staining. Cells from most cases (> 80%) were CD24+, FMC7+, and expressed strongly membrane CD22. The monoclonal antibodies CD10, CD23, and CD38 were positive in one-third of the cases; CD11c in 47%; and CD25 in 25% of cases. A minority of cases (< 20%) were positive with HC2, B-ly-7, and CD5. However, none of the 19 CD5+ cases had the phenotype characteristic of chronic lymphocytic leukemia (CD5+, CD23+, FMC7-, weak surface Ig and membrane CD22). None of the 17 CD25+ cases had the immunophenotype typical...
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 ...
American Journal of Clinical Pathology, 2003
We studied 42 cases of splenic small B-cell lymphoma (SBL) (21 women, 21 men; aged 32-82 years; median, 65 years) with a definitive diagnosis by the World Health Organization classification: chronic lymphocytic leukemia (CLL), 8; mantle cell lymphoma (MCL), 9; follicular lymphoma (FL), 12; marginal zone lymphoma, 13 (splenic [SMZL], 12; extranodal [EMZL], 1). Splenectomy was performed for diagnosis or therapy; splenic weights were 0.2 to 3.8 kg (median, 1.4 kg). In general, splenic SBLs showed white pulp (WP) expansion; morphologic features of the nodules recapitulated the corresponding lymph node histopathologic features. "Marginal zones" were observed commonly in SMZL and FL, may be present in MCL involving the spleen, and may be seen in hilar lymph nodes (HLNs) in SBLs other than SMZL. FL may simulate SMZL and can be distinguished by the presence of neoplastic follicles and HLN morphologic features. Extracellular hyaline deposits (EH) are common in FL and SMZL. MCL typically shows WP expansion by a monotonous small lymphocytic infiltrate, without diffuse red pulp (RP) infiltration or EH; leukemic MCL may show RP infiltration. Splenic morphologic features in CLL vary in WP or RP dominance; marginal zones usually are not observed in CLL.
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.
https://www.ijhsr.org/IJHSR\_Vol.7\_Issue.3\_March2017/IJHSR\_Abstract.053.html, 2017
Splenic lymphoma is an indolent form of mature B-cell neoplasm. It is very rare and overlaps with other lymphomas. The hall mark of clinical presentation is splenomegaly and it usually becomes symptomatic when spleen becomes massive and associated with cytopenia. We discuss here about a 48yr old female with malignant non Hodgkin's lymphoma-spleen. The case is published for its rarity.
Modern Pathology, 2005
Two unusual cases of large B-cell lymphoma with predominant splenic and bone marrow (BM) involvement and similar clinical and histopathologic features are described. Both patients presented with nonspecific constitutional symptoms, unexplained cytopenias, and splenomegaly. Splenectomy revealed diffuse red pulp involvement by large B-cell lymphoma. The perisplenic lymph nodes were also involved diffusely with effacement of normal nodal architecture, excluding a diagnosis of intravascular large B-cell lymphoma. BM biopsies revealed striking erythroid hyperplasia without overt morphologic evidence of involvement by lymphoma. Immunoperoxidase staining of the marrow biopsies with antibodies to CD20 and erythroidassociated antigens revealed involvement by large B-cell lymphoma morphologically resembling the early pronormoblasts. In both cases there was prominent, but not exclusive, intravascular/intrasinusoidal lymphomatous marrow infiltration. These cases represent an unusual variant of large B-cell lymphoma with distinctive patterns of splenic and BM involvement. Furthermore, they underscore the difficulties in identifying intrasinusoidal marrow infiltration by lymphoma in H&E-stained biopsy slides and demonstrate that this pattern of marrow infiltration may be seen in cases of large B-cell lymphoma distinct from the intravascular variant.
British Journal of Haematology, 2009
Five cases of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly are reported; three were splenectomized. BCL2/ IGH rearrangements were found in three cases; HLA-DRB1*07 in all. Bone marrow (BM) trephines showed a moderate lymphoid infiltrate with intrasinusoidal distribution resembling a splenic marginal-zone lymphoma. Splenic white pulp revealed an enlargement of the marginal-zone area; red pulp was infiltrated by the same lymphocytes engulfing the sinuses. Splenic and BM B-lymphocytes were CD79a + /CD20 + /IgM + /IgD + /bcl-2 + /CD27 + / DBA.44 ) /CD31 ) and polyclonal by immunophenotype/polymerase chain reaction. PPBL features an expansion of splenic marginal-zone Blymphocytes, which infiltrate BM sinusoids and circulate in the blood with no evidence of clonality, even in cases with progressive splenomegaly.