Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients (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...
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.
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.
Current Treatment Options in Oncology, 2007
Two subtypes of splenic marginal zone lymphoma (SMZL) are identified in the World Health Organization (WHO) classification: SMZL without villous lymphocytes and SMZL with villous lymphocytes in the peripheral blood (SLVL). SLVL is a rare leukemic and indolent B-cell chronic lymphoproliferative disorder (B-CLPD) that we have to differentiate from hairy cell leukemia (HCL), B prolymphocytic leukemia (B-PLL) and follicular lymphoma (FL). Morphological examination associated with immunophenotyping is, in most cases, likely to distinguish these CD5 negative entities. However, the diagnosis can be difficult to make on morphological criteria, especially in patients without absolute lymphocytosis. Based on histologic, cytogenetic and molecular studies, SLVL emerges as a distinct entity. SLVL has a relatively clinical benign course but a few patients could require treatment, because of a symptomatic splenomegaly and/or a severe cytopenia. In symptomatic patients HCV negative, the frontline treatment remains questionable. Splenectomy, regarded as the most effective treatment, could be required for diagnostic purposes: however, relapse occur in 30% of cases. Fludarabine (FDR), a purine analogue and deoxycoformycin (DCF) can induce a maintained response in a substantial proportion of patients with SLVL and could be used as a first line treatment. In HCV + SLVL patients, antiviral treatment using alpha interferon and ribavirin can induce regression of SLVL.
British Journal of Haematology, 2003
We studied 86 bone marrow biopsies (BMB) from 58 patients presenting with primary splenic marginal zone lymphoma (PSMZL). In 42 patients, a splenectomy was performed which enabled a histopathological diagnosis. In these patients, 44 biopsies were carried out before, and 25 after, splenectomy. In 16 recently observed patients, 17 BMB led to PSMZL diagnosis, and these patients were treated without splenectomy. Seven different patterns of infiltrates were recognized: intravascular, interstitial, nodular, massive, plasmacytic mimicking myeloma and transformation into large B-cell lymphoma (DLBCL). The association of an intravascular infiltrate and nodules with a germinal centre and/or a marginal zone favoured a diagnosis of MZL. Immunohistochemistry demonstrated the expression of B cell-associated antigens and, in 40% of the patients, a monotypic lymphoplasmacytic cell component. These patients often presented a serum M component and autoimmune disorders. In the past, such cases have been diagnosed as lymphoplasmacytic lymphoma. BM involvement was present in all patients. Successive biopsies showed progression and, after chemotherapy, a slight decrease in infiltrates. Transformation into DLBCL occurred in 11 of 34 patients. The patterns described are not specific for PSMZL and occur also in primary nodal MZL and, more rarely, in MALT-type lymphoma.
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.