International journal of hematology-oncology and stem cell research最新文献

Background: Duffy antibodies play a significant role in hemolytic transfusion reactions and hemolytic disease of the fetus and newborn, Duffy(FY) blood group genotyping an essential part of transfusion medicine. The purpose of this study was to assess the importance of Duffy (FY) DNA typing in conducting transfusion compatibility testing and improving Red Blood Cell matching during transfusion. Materials and Methods: In this study, 135 blood samples from SCD patients from the Southwest of Iran were included. All samples were tested with Anti-Fya and Anti-Fyb using the hemagglutination technique, and 64 samples with the fy(a+b-) and fy(a-b-) phenotypes were genotyped using DNA sequencing methods. Results: The prevalence of alloimmunization in this population was 13.04%. fy(a-b+) was the most common phenotype (37/135, 27.4%), followed by fy(a+b-) (35/135, 26%), fy(a+b+) (34/135, 25.2%); and fy(a-b-) (29/135, 21.4%). Among the 64 fy(a+b-) and fy(a-b-) samples, 40 (62.5%) patients had FY*BES allele. 21 out of 40 samples were FY*BES/FY*BES, 17 were FY*A/FY*BES, and 2 were FY*B/FY*BES. Conclusion: The prevalence of GATA-1 mutation (FY*BES allele), in fy(a-b-) and fy(a+b-) patients was reported 62.5%. Therefore, it is possible to use the genotypic information as a database to facilitate the process of searching and supplying better-matched blood transfusion.

Background: Minimal Residual Disease (MRD) assessment is crucial for directing treatment decisions in Acute Lymphoblastic Leukemia (ALL). In low- and middle-income countries, limited resources can present challenges to implementing MRD-guided therapy intensification for ALL. The study attempted to assess the relationship between MRD and other prognostic factors in ALL, focusing on treatment outcomes and disease progression. Materials and Methods: A retrospective observational study was conducted at Ramaiah Medical College and Hospital in Bengaluru, examining patient data from January 2021 to December 2021. MRD status was determined post-induction using flow cytometry. Patients were classified into various groups based on factors such as type of ALL (B-cell or T-cell), NCI risk status (standard or high), cytogenetic risk (favorable, poor, or intermediate), CNS status, prednisone response, and MRD levels at the end of induction. Results: Out of 72 patients, 25% were MRD-positive, with a male: female ratio of 2.13:1. B-ALL was diagnosed in 49 patients and T-ALL in 23, with 75% categorized as high-risk by NCI criteria. Cytogenetic analysis revealed a diverse profile (23.61% PR, 48.61% IR, 27.78% FR), and 58.33% exhibited a good prednisone response (GPR). At the end of the induction phase, 25% tested positive for MRD, with B-ALL showing a lower MRD rate at 15.2%. Age and NCI risk status significantly influenced MRD outcomes, with 75% of participants classified as high-risk. Conclusion: This study demonstrates a significant association between MRD positivity and factors such as age, NCI risk status, and B-ALL diagnosis, underscoring the complex interaction of these variables in predicting treatment outcomes for ALL patients.

Background: Angiogenesis is essential for the survival of neoplasms. Our aim was to describe the clinical profile of primary central nervous system lymphoma (PCNSL) patients at our institution and explore the immunohistochemical expression of OCT4 and nestin in the tumor microenvironment especially in relation to angiogenesis. Materials and Methods: All cases of PCNSL from 2016 to 2022 were retrospectively studied, and clinical and radiological characteristics of the patients were obtained. Descriptive statistics were used. Results: 26 cases were studied; 24 cases (92.3%) were B-cell lymphomas: 23 diffuse large B-cell, and one Burkitt lymphoma. 7.7 % were of T lineage. 13 women and 13 men, had age ranges between 33-71 years (mean 58.16 years). Three patients (12 %) had immunosuppression. Nestin staining revealed hypertrophic astrocytes forming patches about blood vessels with positive cytoplasmic staining in endothelium and pericytes (5-10% of the intra-tumor arterioles). These findings were seen in both B and T lymphomas. OCT4 nuclear expression was only observed in five large B-cell lymphomas and seemed to have relationship with mitoses/HPF (high power field). Conclusion: The novel finding of endothelial, pericytes and hypertrophic astrocytes staining with nestin, points to the involvement of stem cells promoting angiogenesis as a result of a dialogue between neoplastic cells and vascular stem cells. OCT4 expression seems to have a relationship with cell proliferation whose clinical significance should be investigated in prospective studies.

Background: Allogeneic hematopoietic stem cell transplantation (HSCT) has long been a curative intervention for acute leukemia, though outcomes in older patients remain suboptimal due to higher non-relapse mortality (NRM) and relapse rates. Innovations in conditioning regimens and supportive care have made HSCT accessible to patients over 50, but age-related disparities in outcomes persist. Materials and Methods: This 10-year retrospective cohort study reviewed all patients who underwent first-time allogeneic HSCT for acute leukemia. Patients were stratified by age at HSCT (≥ 50 years and < 50 years), and outcomes were assessed for overall survival (OS), disease-free survival (DFS), NRM, and relapse incidence (RI). Results: Of the 1199 patients, 152 were 50 years or older. Five-year OS rates were markedly lower in patients ≥ 50 years compared to younger patients (48.70% vs. 59.35%; P= 0.024 for AML and 23.60% vs. 41.96%; P= 0.025 for ALL). Moreover, older patients demonstrated significantly higher NRM rates (35.95% vs. 23.53%; P= 0.045 for AML and 78.14% vs. 26.76%; P= 0.005 for ALL) and a notably increased incidence of grade III-IV acute graft-versus-host disease (aGVHD). Interestingly, no significant differences were observed between the two age groups regarding DFS rates and RI. Conclusion: Older acute leukemia patients undergoing allogeneic HSCT face significant challenges, including elevated NRM and GVHD rates. While relapse rates were comparable, survival outcomes favored the younger cohort. These findings emphasize the need for age-adapted transplantation strategies, using reduced-intensity conditioning (RIC) regimens and further research to refine risk stratification and improve management approaches for older patients.

Hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment option for several malignant and non-malignant hematologic disorders, including transfusion-dependent thalassemia (TDT). However, HSCT is associated with short-term and long-term complications. One of the recognized causes of morbidity and mortality in TDT patients is heart-related complications. Additionally, cardiac involvement is likely to be more common in patients proceeding to HSCT. Thus, the risks of cardiac complications should be carefully weighed against the benefits of the primary disease cure. This review attempted to discuss the cardiac considerations in TDT patients undergoing HSCT.

Individually customized grafts have become standard for reconstructing extensive chest wall defects resulting from surgical interventions for sternal malignant neoplasms. However, the outcomes of these graft implantations can be further improved by administering patient-derived cells, which have minimal oncological risks. In 2021, a 52-year-old woman with chondrosarcoma (pT2N0M0G2, stage IIB) was admitted to the Department of Thoracic Surgery. The patient presented with a large tumor in the body of the sternum, measuring 81 × 94 × 91 mm, according to the computed tomography (CT) scan. To address this, an individualized endoprosthesis was modeled and created using the original 'pincer-dock' construction based on CT-scan screens. The mononuclear cell fraction (MNCs) was obtained from the patient's peripheral blood one week before surgery using a Haemonetics cell separation device and cryopreserved until the day of the procedure. The resulting 30 mL MNC suspension contained 12 mln cells per 1 mL. We performed flow cytometry analysis using a FACS Aria III flow cytometer to confirm the presence of mesenchymal stromal cells in the MNCs. We also performed immunostaining for S-100, a common tumor marker for benign and malignant diseases, and D2-40, a marker for the lymphatic endothelium that reacts with Kaposi's sarcoma and a subset of angiosarcomas. None of the cells were positive for either marker. Approximately 3 ml of the MNC suspension was injected into each rib edge and 30 ml into the operating field immediately after resection. The titanium endoprosthesis was placed in the sternal defect, and the body of the endoprosthesis was securely covered with a laparoscopically mobilized omental flap. After a one-year follow-up, the patient showed no signs of recurrence or post-surgical complications. These outstanding functional and cosmetic results highlight the potential for the broader clinical utilization of minimally manipulated cells in personalized medicine in oncology. These results could pave the way for wider clinical application of peripheral blood-derived minimally manipulated cells in personalized medicine as an adjuvant for titanium endoprosthesis reconstruction of osteochondral defects in patients with sarcoma.

Primary vertebral lymphoma is an exceedingly rare entity. We hereby report a case of a 67-year-old male who presented to our department with fever, weight loss, and progressively worsening lower back pain radiating to the right hip. Physical examination showed pain on percussion of the dorsal and lumbar spine and tenderness on palpation of the right upper thigh area. The radiographic findings revealed numerous vertebral lesions with a right psoas abscess extending to the right upper thigh. Intravenous antibiotic therapy was initiated and the patient underwent an incision and drainage of the psoas abscess with a favourable outcome. However, given the suspicious imaging findings of the osseous lesions suggestive of malignancy, a vertebral biopsy was performed and yielded histo-pathological findings consistent with bone mantle cell lymphoma. To the best of our knowledge, this is the first case of an infected primary bone mantle cell lymphoma with multiple vertebral involvement. The diagnosis is challenging and can be confused with other diseases.

Background: Thalassemia is one of the most common blood disorders in Iran. Alpha-thalassemia is caused by the deletion of the alpha-globin gene. The frequency of deletions in the alpha-globin gene is associated with microcytosis and hypochromia, making hematological parameters valuable predictive tools in the initial identification of alpha-thalassemia patients. This study aimed to compare hematologic parameters such as Mean Corpuscular Volume (MCV), Mean Corpuscular Hemoglobin (MCH), red blood cell (RBC) count, and hemoglobin (HGB) levels in silent and minor patients, whose genotypes were genetically characterized, with normal patients to establish cut-off points for these groups. Materials and Methods: The study involved a total of 860 patients with alpha-thalassemia, including 267 cases of silent, 261 cases of minor, and 332 cases of normal alpha-thalassemia. Results: Analysis of blood indices based on sex revealed that the male group had higher values than the female group. Assessment of alpha-thalassemia in minor patients showed that the Cis form (-/αα) had higher microcytosis than the Trans form (-α/-α) in this group. This difference was also observed between α^-3.7α/ α^-3.7α and (αα)^-MED/αα as two different genetic forms in minor patients, with (αα)^-MED/αα being in the Cis form. Data indicated that the cut-off value was insignificant in silent patients compared to the normal group. However, minor patients with MCH≤23.7 and MCV≤74.9 had an AUC greater than 0.9 (p-value< 0.01), distinguishing them from the normal group. Conclusion: Comparing hematological parameters in these groups illustrated that MCV and MCH are the best predictor parameters for distinguishing between groups.

Waldenström macroglobulinemia (WM) is a rare lymphoproliferative malignancy presenting with para-proteinemia. The symptoms are attributable to both lymphoproliferation and IgM flare. Gastrointestinal manifestations are not uncommon. It is an indolent disease with good response to chemoimmunotherapy but with possible persistence of asymptomatic paraproteinemia. Resurgence of gastrointestinal symptoms in a patient of WM maintaining reasonable response warrant a thorough search for alternate pathology. Herein we describe a rare case of sequential occurrence of WM with Eosinophilic Gastrointestinal Disease posing a diagnostic and therapeutic challenge.

Background: Allogeneic hematopoietic cell transplantation (allo-HCT) is a complex procedure with the potential to provide curative treatment for various hematological disorders. This study aims to evaluate the outcomes of allo-HCT in hematological diseases and identify significant complications in a single-center setting. Materials and Methods: We conducted a retrospective analysis of 180 patients with hematological diseases who underwent allo-HCT between January 2011 and December 2021. Key outcomes, including indications for transplantation, overall survival, engraftment time, relapse rates, graft-versus-host disease (GVHD), and transplant-related mortality (TRM) were assessed. Results: The most common indications for allo-HCT were benign hematological diseases, particularly aplastic anemia, and thalassemia major. Despite the majority of patients receiving fully matched transplants, acute GVHD was observed in 30% of the cohort. Graft failure occurred in 13 patients, with primary and secondary graft failure rates of 1.6% and 5.5%, respectively. Sepsis emerged as the primary cause of non-relapsed mortality at day 100 and beyond. The overall survival rate in this study was 62%, with 79% of patients disease-free on their last visit. Conclusion: This study provides valuable insights into the treatment strategies and patient care of allo-HCT for hematological disorders by offering a comprehensive overview of multiple relevant outcomes. The findings underscore the significance of addressing complications and risk factors associated with allogeneic transplantation, including GVHD and infections. Future research should focus on further optimizing transplantation techniques to minimize complications and enhance patient survival.