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

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.

Background: Anemia is a condition in which the number of red blood cells or the hemoglobin concentration within them is lower than normal. This study aims to show the intellectual structure of knowledge regarding anemia and gives a comprehensive and up-to-date image of research in this area. Materials and Methods: This is a descriptive-analytical study with a scientometric approach. The PubMed database was searched for research publications indexed under "anemia" including 8484 records between 2011 and 2020. Data were analyzed using Co-word analysis, clustering methods, and strategic diagrams with the help of SPSS and Ucinet 6 software.  Results: The keyword "Anemia Sickle Cell" and two pairs of frequently used keywords, namely "Anemia, Iron *Iron" were the most frequent in the research area. The results shaped the concepts of anemia in 9 clusters. The clusters "Hydroxyurea and sickle cell anemia", "Fetus transfusion", "Management of Thalassemia Major", "Hemolytic Uremic Syndrome", "Management and Control of Anemia", "Chronic Kidney Failure and Anemia", "Hematopoietic Stem Cell Transplantation" are topics that may be emerging or disappearing. The "Thalassemia and blood transfusion" are immature clusters. Conclusion: This study uses co-word networks that indicate important links between keywords of the research areas. Most research approaches are in the therapeutic aspects. Despite the importance of the effect of anemia on all levels of society, including economics, education, and other types of anemia, as well as its impact on learning and mental disorders, these subjects have not been given sufficient consideration.

Background: Considering the widespread COVID-19 pandemic and its impact, especially on children, particularly those with cancer, in terms of transmission risk, mortality, and the occurrence of the disease based on various studies in different countries, we decided to conduct this study to improve the care of children with cancer regarding COVID-19. Materials and Methods: A descriptive cross-sectional study with a confirmed diagnosis of COVID-19 consisted of obtaining 20 mL of blood samples from the participants in a random manner. Diagnostic examinations, including CT scans, chest X-rays, and a range of hematologic and blood tests, such as complete blood count, ESR, CRP, and D-Dimer, were performed on all patients. Results: This study contains 26 males and 12 females. The mean age of the patients was 3.81 ± 6.35 years. The majority of cancer patients with COVID-19 were diagnosed with Acute Lymphoblastic Leukemia (ALL) (47.7%). The most common symptoms of COVID-19 in the patients were fever (73.7%), cough (39.5%), and nausea/vomiting (21.1%). 40.4% of the patients had pathological findings suggestive of COVID-19 on their chest CT scans. 60.52% of the patients had an elevated Erythrocyte Sedimentation Rate (ESR), and 73.68% had an elevated C-reactive protein (CRP) level. Conclusion: Despite the outcomes of COVID-19 in most children with cancer in this study, children with cancer still experience risks from COVID-19, and it is unclear how delays and interruptions in cancer treatment and direct damage from the virus may impact long-term outcomes in these patients.

Background: Human platelet antigens (HPAs) play a clinically significant role in alloimmunization and the development of immune-mediated disorders such as immune thrombocytopenia (ITP), fetal and neonatal alloimmune thrombocytopenia (FNAIT), and post-transfusion purpura (PTP). Understanding the genetic profiles of HPAs is critical for preventing and treating these conditions. Given the limitations of serological methods in determining HPA genotypes, this study aims to investigate the association between the genotypes of HPA1, HPA2, HPA3, HPA4, and HPA15 antigens and autoimmune thrombocytopenia in Lorestan Province, utilizing the PCR-SSP method. Materials and Methods: This case-control study involved 80 individuals diagnosed with ITP and 120 healthy controls. DNA samples were extracted using a commercial DNA extraction kit, with concentrations quantified via a Nanodrop spectrophotometer. Genotyping was performed using the PCR-SSP method with specific primers for each HPA gene. The genotype data were verified using previously established sample sets. The frequencies of each HPA genotype were recorded, and a comparative analysis was conducted between the patient and control groups to evaluate the study hypothesis. Results: The results revealed that individuals carrying the HPA2b allele had a 5.31-fold increased risk of developing ITP, a statistically significant finding (P < 0.05, OR = 5.31). Similarly, the presence of the HPA15b allele was associated with a 6.54-fold increased risk (P < 0.05, OR = 6.54). Conclusion: These findings, in conjunction with previous studies, suggest the need for larger-scale investigations across different populations. Such research could aid in the early diagnosis and prediction of thrombocytopenia severity, inform treatment strategies, and facilitate the removal of pathogenic antibodies from circulation.

A 60-year-old female presented with abdominal pain, weight loss, and fatigue. Imaging revealed a pancreatic mass, bilateral pleural effusion, ascites, and lytic bony lesions. Investigations confirmed multiple myeloma with lambda light chain disease. Positron emission tomography-computed tomography (PET-CT) scan demonstrated extensive metabolically active soft tissue masses involving the pancreatic region, retroperitoneum, mediastinum, paravertebral regions, and multiple skeletal lesions with extraosseous soft tissue involvement, along with bilateral pleural effusions with metabolically active pleural and extrapleural deposits. The patient was initiated on a bortezomib, cyclophosphamide, and dexamethasone chemotherapy regimen with therapeutic thoracentesis for pleural effusion management. After two cycles, the patient showed remarkable clinical improvement. A repeat PET-CT scan revealed significant interval regression of soft tissue masses, metabolic activity resolution, and regression of pleural and extrapleural deposits. The extensive skeletal lytic lesions showed morphological stability but regression of associated metabolic activity and extraosseous soft tissue. This case highlights the potential of novel agent-based regimens in achieving exceptional responses in multiple myeloma patients with extensive extramedullary disease (EMD), including uncommon manifestations like pleural effusion. Early recognition and prompt initiation of appropriate therapy are crucial for improving outcomes in such cases.

Background: Up-regulation of the microRNA-625 and abnormal expression of the Sox2 gene have been studied and seen in several tumors. Few reports have also shown the aberrant expression of miR-625 and Sox2 expression in various cancers. Several studies have also confirmed that phosphatidylinositol 3' -kinase /protein kinase B pathways regulate hematological malignancies, including Acute Myeloid Leukemia (AML). Thus, this study aimed to investigate the effects of mir-625 up-regulation on proliferation, apoptosis, and cell cycle by targeting the Sox2 gene via the downstream Akt signaling pathway and cell cycle regulators, such as p21, p27, and cyclin E in the KG-1 cell line. Materials and Methods: Cells obtained from the KG-1 cell line were cultured and transfected with plasmid DNA (miR-625) and scrambled as the control using the Lonza electroporation system. Flow cytometry was used to evaluate cell cycle, proliferation, and apoptosis. Relative gene expression was validated by qRT-PCR. All data were analyzed using graph pad prism 7.01 and REST 2009. Results: KG-1 cells transfected with the mir625-GFP construct showed decreased proliferation, increased apoptosis, and induced cell cycle arrest. Low levels of Sox2, p21, cyclin E, and up-regulation of p27 were confirmed and validated by qRT-PCR ( P < 0.05 ). Conclusion: MiR-625 can be a promising approach to aid in the treatment of AML. However, further studies are required in this field.

Hairy cell leukemia (HCL) is a rare B-cell neoplasm that constitutes around 2 percent of all lymphoid leukemias and occurs more frequently in elderly males. The usual triad of HCL includes pancytopenia, splenomegaly, and hairy cells in the bone marrow. This is a case of an atypical presentation of biclonal HCL diagnosed on flow cytometry; the existence of biclonal HCL is extremely rare with very few case reports. Pure biclonal HCL should be regarded as an extraordinary finding among the so-called composite lymphomas.