Mediterranean Journal of Hematology and Infectious Diseases最新文献

Background: Clonal mature B-cell lymphoproliferative disorders (B-LPDs) are a heterogeneous group of neoplasia characterized by the proliferation of mature B lymphocytes in the peripheral blood, bone marrow and/or lymphoid tissues. B-LPDs classification into different subtypes and their diagnosis is based on a multiparametric approach. However, accurate diagnosis may be challenging, especially in cases of ambiguous interpretation. Multiparameter flow cytometry (MFC) represents an extensively used technique to detect the presence of different cellular lines in immunology and hematology. MFC results provide an essential contribution to the B-LPDs diagnostic process, even more so considering that panels are constantly integrating novel markers to improve diagnostic accuracy.

Objectives: The aim was to evaluate the contributing role of MFC routinary studies by analyzing the expression and the mean fluorescence intensity (MFI) of CD200, ROR1, and CD43 in various B-LPDs to evaluate their usefulness in the differential diagnosis of these diseases.

Methods: We retrospectively evaluated 2615 consecutive cases of newly collected samples (mostly from patients with lymphocytosis) analyzed by MFC carried out in the B-LPD diagnostic process referred to the Division of Hematology of the Sapienza University of Rome. We compared the results of CD200, ROR1, and CD43 expression percentage and their MFI between different subtypes of B-LPDs.

Results: In chronic lymphocytic leukemia (CLL), CD200, ROR1, and CD43 were always expressed with bright intensity. CLL samples presented high CD200 expression and MFI [CD200%, mean: 100 (range, 24-100); positivity rate: 100%; MFI, median = 125 (range, 10-1200)] statistically higher than mantle cell lymphoma (MCL) (p<0.001), which is usually negative for CD200, and variant hairy cell leukemia (vHCL, according to 2022 ICC) (p<0.001), but comparable with classic HCL (cHCL) (p>0.9). ROR1 resulted expressed in all CLL [ROR1%, mean: 100 (range, 52-100), positivity rate: 100%; MFI, median=50 (range, 10-202)] and MCL cases with comparable MFI (p>0.9). CD43 expression and MFI were significantly higher in CLL [CD43%, mean 99 (range, 59-100); positivity rate: 100%; MFI, median = 130 (range, 41-980)] than in MCL, vHCL, cHCL, and all the others mature B-cell neoplasia (p<0.001). CD200 and CD43 expression and MFI were significantly higher in cHCL compared to vHCL. Among the other mature B-cell neoplasia, CD200 was variably expressed in follicular lymphoma (FL), marginal zone lymphoma (MZL), diffuse large B-cell lymphoma (DLBCL), and lymphoplasmacytic lymphoma (LPL). ROR1 and CD43 presented a very low expression percentage in this latter group, being mostly negative. Persistent polyclonal B-cell lymphocytosis (PPBL) resulted in uniformly positive for CD200 and negative for ROR1 and CD43.

Conclusions: Our data suggest that evaluating C

Background: Red Blood Cell Exchange (RBCX) is a common treatment for pediatric sickle cell disease (SCD). Since inflammation with elevated proinflammatory cytokines plays a crucial role in SCD, this study hypothesized that RBCX might lower these cytokines and aimed to assess the impact of this technique on these markers.

Methods: Prospective and observational study of pediatric SCD patients (HbSS genotype) enrolled in a chronic RBCX program at a Portuguese Hospital from October 2022 to August 2024. Blood samples were collected before and after RBCX to assess hematological and inflammatory markers. Data were analyzed using SPSSv25 ^® (Significance level p < 0.05); Informed consents were obtained.

Results: Thirty-one children (median age 10 years) were studied: 14 were treatment-naïve, and 17 were previously in a chronic RBCX program. The primary indication for starting the program was cerebrovascular disease prevention (81%). Analysis of 286 RBCXs showed no major adverse events or disease-related hospitalizations. Hemoglobin levels increased by 1.5g/dL post-RBCX; HbS, leukocytes, IL-1, and CRP decreased by 69%, 20%, 21%, and 13%, respectively. Other markers showed no significant changes. IL-1, ferritin, and procalcitonin showed high levels before RBCX; IL-6 showed high levels post-RBCX. Considering only naïve patients, they had higher pre-RBCX IL-1 levels than those with prior RBCX (difference of 22.6 pg/mL); IL-6 increased by 17.3% and IL-1 decreased by 23.9% post-RBCX (p < 0.001).

Conclusions: RBCX safely reduces HbS, leukocytes, and IL-1 levels, suggesting a modulatory effect on inflammation in SCD patients. Further research is needed to explore cytokine mechanisms in SCD.

Background: Iron deficiency anemia (IDA) is a major public health problem among children worldwide. Iron deficiency without anemia (IDWA) is at least twice as common as IDA. Some studies propose that oral iron fortification can modify the infant's gut microbiome, leading to intestinal inflammation.

Objectives: To determine whether oral iron therapy can lead to intestinal inflammation in children with IDA or IDWA.

Patients and methods: Fifty-six patients aged 6 months to 16 years (median age 7.6 years) with IDA or IDWA were randomly assigned to receive either iron (III)-hydroxide polymaltose complex (IPC) 5 mg/kg once daily (maximum dose 100 mg) or sucrosomial iron (SI)1.4 mg/kg once daily (maximum dose 29.4 mg). Safety and efficacy were studied after 30 and 90 days of treatment. In addition, fecal calprotectin as a marker of intestinal inflammation was measured simultaneously and compared to results obtained before therapy.

Results: A significant increase in serum ferritin was noted in both groups as the median ferritin level at baseline was 6.7 μg/L in the IPC group and 6.6 μg/L in the SI group, increasing to 15.9 μg/L and 12.1 μg/L respectively, after 90 days of treatment. However, there was no significant change in fecal calprotectin in either group. In addition, no differences in the trend over time were observed between the two groups regarding fecal calprotectin, serum ferritin, and hemoglobin.

Conclusions: IPC and SI were equally effective in treating IDA and IDWA. At the recommended doses, oral iron therapy does not seem to induce intestinal inflammation.

Multiple myeloma (MM), characterized by abnormal proliferation of clonal plasma cells, is an incurable hematological malignancy. Various immunotherapy strategies have emerged as an efficacious approach for the treatment of MM, including monoclonal antibodies, antibody-drug conjugates, bispecific antibodies, and chimeric antigen receptor T (CAR-T) cells. Anti-B-cell maturation antigen (BCMA) CAR-T cells have revolutionized the treatment of MM patients with relapsed/refractory disease and their clinical use was approved for the treatment of these patients. Despite this progress, the efficacy of CAR-T cells in MM is limited by the responsiveness of only a part of the treated patients, the relapse of other patients, the cost of the treatment and the diminished response in patients with prior exposure to anti-BCMA targeting agents. Ongoing clinical trials are evaluating the use of CAR-T cells at an earlier stage of MM disease and the use of CAR-T cells targeting other membrane antigens expressed on malignant plasma cells.