Syndecan‐1 (CD138) is a transmembrane heparin sulfate proteoglycan expressed on distinct stages of differentiation of B‐lymphoid cells. Its prognostic value in B‐cell chronic lymphocytic leukemia (B‐CLL) has not been evaluated so far. The serum concentration of sCD138 and some angiogenesis‐involved cytokines: vascular endothelial growth factor (VEGF), basis fibroblast growth factor (bFGF), and endostatin were studied in 52 previously untreated patients with B‐CLL. We found that bFGF and sCD138 levels were significantly higher in B‐CLL patients than in controls. In patients with sCD138 level or endostatin level below the median value the lymphocyte count was higher than in patients with serum level of those cytokines above the median value. In patients with progressive disease bFGF level was significantly higher and sCD138 level significantly lower than in patients with stable one. Moreover, high sCD138 level was associated with longer lymphocyte doubling‐free survival, and, on the limit of statistical significance, a high endostatin level was associated with shorter progression‐free survival. We conclude that serum sCD138 level is increased in early stage B‐CLL patients and may have a positive prognostic value as to the dynamics of the disease.

Dysregulation of ribosome biogenesis and alteration of ribosome composition, including alteration in ribosomal RNA (rRNA) 2'-O-ribose methylation, can play a role in malignant transformation and cancer progression. Several studies recently reported that components of the rRNA methylation complex are associated with leukemogenesis. However, no study ever investigated the alteration of ribosome biogenesis factors in the most common pediatric malignancy - B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The objective of this study was to examine the expression of factors building the rRNA methylation complex, either the protein components (1 methyl-transferase (FBL), NOP56, NOP58, NHP2L1) or some RNA components (box C/D snoRNAs: SNORD35B, SNORD65, SNORD46, SNORD50A, SNORD38B), as well as CMYC, and nucleolin (NCL) - a protein involved in rRNA synthesis. Clinical effects in children with BCP-ALL were also investigated. The factors involved in ribosome biogenesis were studied in 28 children with BCP-ALL with the use of real-time polymerase chain reaction (RT-PCR) using the BioMark HD System (Fluidigm, San Francisco, USA) in cDNA prepared from the bone marrow samples collected at diagnosis. Strong correlations were observed between NOP56, NOP58 and NCL, and multiple weaker correlations were observed in the box C/D snoRNA category, and between box C/D snoRNA and transcripts coding proteins of the rRNA methylation complex. The expression of analyzed transcripts did not correlate with the initial white blood cells count (WBC) or with bone marrow blast percentage. Ribosome biogenesis upregulation with overexpression of FBL and NOP56, and CMYC was found in patients who subsequently relapsed and the upregulation signature was not associated with known risk predictors. This is the first report on the clinical aspect of ribosome biogenesis in pediatric BCP-ALL, and it shows that overexpression of CMYC and C/D box nucleoproteins FBL and NOP56 is an antecedent event in patients who subsequently relapse. The dysregulation pattern is different from the previous reports in acute myeloid leukemia (AML), suggesting that dysregulation of ribosome biogenesis is specific for BCP-ALL.

Previously, we showed that cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) is overexpressed in chronic lymphocytic leukaemia (CLL) and its expression is correlated with the expression of the major regulators of G1 phase progression: cyclins D2 and D3, and cyclin-dependent kinase inhibitory protein 1 (p27^KIP1). In the present study, we blocked CTLA-4 on the surface of both CLL cells and normal B lymphocytes to investigate the impact of CTLA-4 on the expression of the mentioned G1 phase regulators. We found that in CLL patients and in healthy individuals, the median proportions of cyclin D2-positive cells as well as cyclin D3⁺ cells significantly decreased following CTLA-4 blockade. Moreover, CTLA-4 blockade led to an increase in the median frequencies of p27^KIP1-positive cells, although this increase was marked only in CLL patients. Our study showed that CTLA-4 affects the expression of the key regulators of G1 phase progression in CLL cells as well as in normal B lymphocytes and may contribute to a better understanding of the role of CTLA-4 in the regulation of G1 phase progression.

Interactions between APRIL (TNFSF13) and its receptor TACI (TNFRSF13B) are implicated in providing survival benefits for chronic lymphocytic leukaemia (CLL) cells. Here we explored the relationship between TNFSF13 and TNFRSF13B SNPs and expression of APRIL and TACI molecules and performed extended case-control study to evaluate earlier observations. Expression of APRIL and TACI was detected by FACS for 72 and 145 patients, respectively, and soluble APRIL was measured by ELISA in plasma of 122 patients. Genotypes were determined in 439 CLL patients and 477 control subjects with TaqMan Assays or restriction fragment length polymorphism (RFLP). The rs4968210GG genotype of TNFSF13 was associated with a lower percentage of CD19⁺APRIL⁺ cells in CLL patients when compared to (AA + GA) genotypes (p-value = 0.027). Homozygosity at rs11078355 TNFRSF13B was associated with higher CD19⁺ TACI⁺ cell percentage in CLL patients (p-value = 0.036). The analysis of extended groups of patients and healthy controls confirmed the association of TNFSF13 rs3803800AA genotype with a higher CLL risk (OR = 2.13; CI95% = 1.21; 3.75; p-value = 0.007), while the possession of TNFRSF13B rs4985726G allele (CG + GG) genotype was associated with lower risk of CLL (OR = 0.69; CI95% = 0.51; 0.95; p-value = 0.02). Genetic variants of TNFSF13 and TNFRSF13B may have an impact on APRIL and TACI expression and may be considered as possible CLL risk factors.

Introduction: Primary renal lymphoma is extremely rare, accounting for less than 1% of all lymphomas. Objectives: The aim of this study was to describe a group of patients with primary renal lymphoma diagnosed in hematology and oncology centers aligned with the Polish Lymphoma Research Group (PLRG). Patients and methods: This was a retrospective analysis of adult patients with primary renal lymphoma diagnosed at PLRG centers. Results: Thirty-two patients were diagnosed in seven centers over 24 years (2000–2023). The most common type of lymphoma was diffuse large B-cell lymphoma (DLBCL). The median progression-free survival (PFS) after first-line treatment was 2.1 (95% CI: 1.07–4.18) years, while the median overall survival (OS) was 6.3 (95% CI: 1.82–6.34) years. The median age at diagnosis was 63.3 years old, and 59.4% of the patients were females. In multivariate Cox regression for PFS, only creatinine > 1.5 mg/dL (HR 10.2, 95% CI: 2.08–50.09, p = 0.004) and hemoglobin (Hgb) < 10 g/dL (HR 8.39, 95% CI: 1.88–37.49) were associated with inferior PFS. Patients who achieved complete remission (CR) after first-line of treatment had significantly longer PFS (median 4.18, 95% CI: 2.02–4.18 vs. median 0.73, 95% CI: 0.50–0.79, p = 0.004) and OS (median not reached vs. median 1.49, 95% CI: 0.43–6.33, p = 0.001). Patients treated with nephrectomy had longer OS (median not reached vs. median 5.07, 95% CI: 1.32–5.08, p = 0.05). However, in multivariate Cox regression for OS, only hypoalbuminemia was an independent factor for inferior survival (HR 5.44, 95% CI: 1.12–26.38, p = 0.04). Conclusions: Primary renal lymphoma is an extremely rare type of lymphoma with a poor prognosis. The prognosis may improve with future advances in treatment, including nephrectomy.