Hematology, Transfusion and Cell Therapy最新文献

{"title":"CLINICAL PRESENTATION AND OUTCOMES OF PATIENTS WITH MYELODYSPLASTIC SYNDROME INTRODUCTION","authors":"Jehanzeb Ur Rehman","doi":"10.1016/j.htct.2025.103897","DOIUrl":"10.1016/j.htct.2025.103897","url":null,"abstract":"<div><h3>Objective</h3><div>Myelodysplastic Syndrome (MDS) is a clonal hematopoietic disorder that is characterized by dysplasia along with anaemia, thrombocytopenia or neutropenia and a risk of progression to Acute Myeloid Leukaemia (AML). In the United States, the yearly incidence of MDS is approximately 4 per 100 000 people, notably higher among older population rising tenfold by the age of eighty (80) years. Prognostic systems, such as the revised International Prognostic Scoring System (IPSS-R), offer rationally accurate estimates of survival at the population level. The goals of treatment in individuals having lower-risk MDS includes improving quality of life and minimizing Red Blood Cells (RBCs) and platelet transfusions. Therapeutic goals in patients having Higher-Risk MDS (HR-MDS), include decreasing the risk of transformation to AML and increasing survival. Haematopoietic Cell Transplantation (HCT) has the potential to cure MDS, but less than 10% of affected people undergo this treatment. Improvements in the understanding of MDS has resulted in newer management strategies for these patients. As a result, the treatment landscape for MDS patients is changing. All these advancements are expected to improve the survival rate of patients suffering from MDS. There is limited data on presentation and outcomes of MDS patients in Low Middle Income Countries (LMICs). The Aim of our study is to assess the clinical presentation and treatment outcomes in patients with MDS in a low middle income country.</div></div><div><h3>Methodology</h3><div>This is a single-centre retrospective cohort study with analytical design, which was approved by the hospital ethics committee (IRB-017/AFBMTC/Approval/2022). The study was conducted at the Armed Forces Bone Marrow Transplant Centre, a tertiary care facility located in Rawalpindi, Pakistan and included all consecutive patients having age > 15-years diagnosed with MDS as per revised WHO 2016 criteria from January 2019 till December 2023. The data of 128 patients was collected, followed-up and analyzed for disease and survival outcomes. Patients lost to follow up in the first 12 weeks of diagnosis or with insufficient extractable data were excluded from the study. The initial demographic and clinical information collected included age, gender, clinical presentation and laboratory parameters. Bone Marrow (BM) morphology and cytogenetics were used to establish the diagnosis of MDS. Subclassification was done using revised World Health Organization (WHO) 2016 classification of hematopoietic and lymphoid tumors. Patients were risk stratified using International Prognostic Scoring System (IPSS) and Revised-IPSS (R-IPSS) scoring as per available data. Initial treatment was stratified depending upon the aim of treatment (palliative, definitive), supportive treatments given were blood products and antibiotics. For palliative intent treatments included growth factors, immunosuppressants, lenalidomide, low dose cytarab","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103897"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A CASE OF THROMBOCYTOSIS AND HEMOTHORAX IN A PATIENT WITH ITP FOLLOWING ROMIPLOSTIM USE","authors":"Ali Turunç,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2025.103902","DOIUrl":"10.1016/j.htct.2025.103902","url":null,"abstract":"<div><h3>Introduction</h3><div>Immune Thrombocytopenia (ITP) is an autoimmune disease characterized by isolated thrombocytopenia and normal to large platelets in the peripheral blood smear. It is an autoimmune disorder that leads to peripheral platelet destruction and decreased platelet production. Romiplostim, a peptide-antibody fusion product, is a thrombopoietin receptor agonist indicated for use in patients with ITP. Romiplostim is indicated for patients with ITP who have had an inadequate response to first-line therapy. Side effects of the drug include increased bone marrow reticulin, reversal of severe thrombocytopenia, thrombocytosis and increased immunoblast proliferation. In this case report, we present a patient with ITP refractory to first-line therapies who was admitted to the intensive care unit for thrombocytosis and haemothorax after a single dose of Romiplostim.</div></div><div><h3>Case</h3><div>A 34-year-old female patient, who had been followed in the hematology clinic for ITP for approximately 5-years, was admitted to the clinic because of deep thrombocytopenia and deep anaemia due to menometrorrhagia. On admission, the platelet count was 2000 (10³/µL) and the hemoglobin level was 5.4 g/dL, and she was taking eltrombopag 75 mg at the time of admission. He was tachycardic and hypotensive and was given erythrocyte suspension. Clinical and radiological examination revealed no bleeding foci other than menstrual and oral mucosal bleeding. The patient was treated with steroids, IVIG and rituximab during the follow-up period in the clinic. Despite the treatments given, bleeding control could not be achieved and a single dose of 2 mcg/kg Romiplostim was administered to the patient in the 2^nd week of hospitalization. The patient's platelet count began to rise rapidly on the 3^rd day after treatment and was measured at 1,650,000 (10³/µL) on the 5^th day. At the same time, the patient developed dyspnea and a chest scan was performed, which revealed a hemothorax. The patient was admitted to intensive care. Drainage was performed with a chest tube. Acquired von Willebrand Factor (vWF) deficiency due to thrombocytosis was considered as the cause of the hemorrhage. The vWF level in the blood was found to be low. The thrombocytosis was controlled by platelet apheresis. After clinical improvement, platelet levels were normalized, and the patient was discharged.</div></div><div><h3>Conclusion</h3><div>Romiplostim is a generally well tolerated agent. Current evidence suggests that it increases platelet counts, reduces bleeding, reduces the need for rescue therapy, reduces the amount of corticosteroids required, improves quality of life and, in isolated cases, is associated with remission of ITP. However, it should be used with caution as serious side effects include increased bone marrow reticulin, reversal of severe thrombocytopenia, thrombocytosis and increased immunoblastic proliferation</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103902"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"THERAPEUTIC APHERESIS FOR EPIDERMOLYSIS BULLOSA AND SECONDARY THROMBOCYTOSIS IN NORWEGIAN SCABIES: A CASE REPORT","authors":"Mine Ezgi Payaslı,&nbsp;Gökhan Demirci,&nbsp;Zeliha Yıldız Kandemir,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2025.103913","DOIUrl":"10.1016/j.htct.2025.103913","url":null,"abstract":"<div><h3>Introduction</h3><div>Secondary thrombocytosis is a well-recognized response to chronic inflammation, infections, and systemic disorders, but its association with dermatologic diseases such as Norwegian scabies and epidermolysis bullosa is rare. In severe cases of epidermolysis bullosa, therapeutic apheresis may be used as part of supportive care. This case highlights a young patient with extreme thrombocytosis managed with myelosuppressive therapy and therapeutic apheresis for epidermolysis bullosa.</div></div><div><h3>Case presentation</h3><div>A 20-year-old female with Norwegian scabies and epidermolysis bullosa was admitted due to fatigue and worsening skin lesions. Laboratory findings included severe thrombocytosis (PLT: 947,000 µL), microcytic anemia (Hb: 8.3 g/dL, MCV: 69.6 fL), elevated inflammatory markers (CRP: 118 mg/L, sedimentation rate: 63 mm/h), and positive direct Coombs test. Imaging revealed multiple mildly enlarged lymph nodes (axillary, inguinal, iliac) and hepatosplenomegaly, but bone marrow biopsy showed normocellular marrow with increased megakaryocytes. Molecular testing for JAK2, CALR, MPL, and BCR-ABL mutations was negative, ruling out Essential Thrombocythemia (ET) and Chronic Myeloid Leukemia (CML). Since the patient’s thrombocytosis was determined to be secondary to chronic inflammation, she was treated with Hydroxyurea (Hydrea) 2 × 500 mg/day and aspirin, leading to a gradual decrease in platelet counts, confirming a reactive process rather than a primary hematologic disorder. Concurrent corticosteroid therapy for epidermolysis bullosa resulted in significant improvement in dermatologic symptoms and inflammatory markers. Given the severity of epidermolysis bullosa, therapeutic apheresis was performed as part of supportive treatment, contributing to clinical stabilization and symptom relief.</div></div><div><h3>Conclusion</h3><div>This case underscores the importance of differentiating secondary thrombocytosis from primary myeloproliferative disorders and highlights therapeutic apheresis as a supportive intervention in severe epidermolysis bullosa. It emphasizes the role of multidisciplinary management, where targeting the underlying dermatologic inflammation can help control hematologic abnormalities. In complex inflammatory disorders, therapeutic apheresis may serve as an adjunct therapy, improving patient outcomes.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103913"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"VALIDATION OF LONG-TERM HANDLING AND STORAGE CONDITIONS FOR HEMATOPOIETIC STEM CELL PRODUCTS FOR AUTOLOGOUS TRANSPLANTS","authors":"Ahmed Al Bahrani","doi":"10.1016/j.htct.2025.103889","DOIUrl":"10.1016/j.htct.2025.103889","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Objective&lt;/h3&gt;&lt;div&gt;Hematopoietic Stem Cells (HPSCs) are multipotent stem cells that can differentiate into lymphoid and myeloid progenitors, giving rise to White Blood Cells (WBCs), Red Blood Cells (RBCs), and platelets. HPSCs are a widely used treatment for many hematological non-malignant and malignant disorders. HPSCs can be used in the fresh or cryopreserved state for future use. Fresh HPSCs are typically stored at 2–6°C for up to 72 hours and are primarily used for allogeneic transplants or autologous transplants in myeloma and lymphoma patients. However, in some cases of autologous donations, HPSC transplantation is delayed more than three days after collection. In such situations, the cells are thawed after short-term preservation, resulting in a 35% cell viability loss. This study aimed to investigate the quality of HPSCs products after long-term storage exceeding 72 hours.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methodology&lt;/h3&gt;&lt;div&gt;Between July 11, 2021, and February12, 2022, the bone marrow and stem cell transplant center at King Fahad Specialist Hospital (KFSH-D) collected 12 autologous mobilized PBHSCs according to established procedures. All participants provided written informed consent to participate in this study. The study design protocol was approved by the Institutional Review Boards. This study was conducted under the principles of the Declaration of Helsinki. Following PBHSC collection, samples for quality testing were obtained from the PBHSC bags as a control. Under sterile conditions and using a class II A2 biosafety cabinet, 5–15 mL of the PBHSCs product bag was transferred to a sterile transfer bag using a bag spike or a sterile connecting device. All products were stored in a continuously monitored refrigerator set at a temperature between 2–6°C. Viability, CD34+ enumeration, and Total Nucleated Cells (TNC) count were subsequently determined at 0, 72, and 120 hours. Product sterility was also evaluated at 0 and 120 hours.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Twelve PBHSCs products were prepared in the transfer bags. All products contained a minimum of 287.9 cells/μL based on the CD34+ counts. Of the 12 products collected, 66.7% were from male autologous donors, and the remaining 33.3% were female donors. During hypothermal storage at 2–6°C, a gradual loss of total cell viability, CD34+ cell recovery, and TNC recovery were observed, but these losses were not significant. Total cell viability cells decreased by 2.18%±1.84% after 72 hours and by 7.40% ± 4.12% after 120 hours. The mean recovery of CD34+ reached 83.83% ± 5.35% after 120 hours. The mean TNC recovery was 89.93% ± 8.39% after 72 hours and 76.18% ± 14.09% after 120 hours. The stability characteristics of the PBHSC products stored for different intervals (72 hours and 120 hours). No significant differences were observed between the fresh PBHSCs and those stored for 120 hours of hypothermal storage. Blood culture was used to evaluate the sterility of the PBHSCs on the collec","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103889"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Investigation of haematological, inflammatory parameters and the incidence of alloimmunization in multi-transfused sickle cell diseased patients","authors":"Florence Ifechukwude Aboderin ,&nbsp;Taofeeq Oduola ,&nbsp;Glenda Mary Davison ,&nbsp;Oluwafemi Omoniyi Oguntibeju","doi":"10.1016/j.htct.2025.103937","DOIUrl":"10.1016/j.htct.2025.103937","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Introduction&lt;/h3&gt;&lt;div&gt;Sickle cell disease is a haemoglobinopathy caused by an aberrant mutation of the beta chain with the amino acid valine replacing glutamic acid at the 6th position. Patients with sickle cell disease suffer from complications including chronic inflammation and the development of allogeneic antibodies due to multiple blood transfusions. This study investigated the association between haematological, inflammatory markers and alloimmunization in multi-transfused patients with sickle cell disease.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;This was a cross-sectional study, that enrolled 100 participants; 50 young adults (18–48 years) with homozygous sickle cell disease (Sickle cell Group) from the Obafemi University Health Centre in Nigeria, and 50 age and sex matched individuals who did not have the disease (Control group) but who had also received blood transfusions. Complete blood counts and differentials were processed on an auto-analyser (SFRI H18 Light, France). Red cell antigen identification used the saline and anti-human globin method while the abnormal haemoglobinopathy was evaluated using electrophoresis. ABO and Rhesus blood groups were analysed using a direct method on tile, and the determination of inflammatory markers including C-reactive protein, tumour necrosis factor-alpha, interleukin-6, and interleukin-1β was by the enzyme-linked immunosorbent assay technique. The data were statistically analysed using SPSS version 24.0 and GraphPad Prism. Additionally, the student &lt;em&gt;t&lt;/em&gt;-test and Chi-square test were employed as appropriate. Data were presented as mean ± standard deviation, with a &lt;em&gt;p&lt;/em&gt;-value &lt;0.05 considered statistically significant&lt;strong&gt;.&lt;/strong&gt;&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Result&lt;/h3&gt;&lt;div&gt;As expected, the Sickle Cell group had an increased rate of alloimmunisation and significantly reduced haemoglobin and red cell parameters except for the mean cell volume. Although both groups had platelet counts within the reference range the Sickle Cell group had significantly higher counts than the Control group. The Sickle Cell group displayed evidence of inflammation with significantly increased levels (&lt;em&gt;p&lt;/em&gt;-value = 0.001) of C-reactive protein and tumour necrosis factor-alpha. This was supported by higher white cell counts and neutrophilia. The majority of the antibodies detected in sickle cell disease were anti-Kell, Jka and Fya while the controls showed a higher prevalence of anti-M and Kell antibodies. Despite the elevated inflammatory markers, no significant correlation was observed between these and the rate of alloimmunization.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;In this study, the Sickle Cell group had an elevated rate of alloimmunization with higher levels of anti-kell, Jka and Fya as well as inflammatory markers. However, despite these findings, no significant correlation between inflammatory markers and alloimmunization could be detected. This suggests that elevated alloimmunization rat","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 3","pages":"Article 103937"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144587862","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ACUTE MYELOID LEUKEMIA PRESENTING AS ISOLATED MYELOID SARCOMA: A CASE REPORT","authors":"Elif Canpolat Hırfanoğlu ,&nbsp;Ali Turunç ,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2025.103910","DOIUrl":"10.1016/j.htct.2025.103910","url":null,"abstract":"<div><div>Myeloid Sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare extramedullary tumor consisting of immature myeloid cells. It can occur as an isolated entity, concurrently with Acute Myeloid Leukemia (AML), or as a relapse manifestation. In cases where myeloid sarcoma presents without prior hematologic malignancy and with normal peripheral blood counts, diagnosis can be significantly delayed, leading to disease progression. Recognizing MS as a potential early sign of AML is crucial to initiating timely treatment. A 48-year-old female with a history of hypertension and a prior L1 vertebral compression fracture in 2016 presented with new-onset lumbar pain in 2024. Lumbar MRI revealed a paraspinal soft tissue lesion at the T12‒L1 level, prompting further investigation. The patient’s hematologic parameters were within normal limits, with a white blood cell count of 8290 µL, hemoglobin of 13 g/dL, and platelet count of 400,000 µL. The lesion was surgically excised, and histopathological examination confirmed myeloid sarcoma. Following this diagnosis, hematology consultation was requested, and bone marrow aspiration and biopsy were performed. Although the blast percentage was only 7%‒8%, flow cytometry findings were consistent with AML. PET-CT revealed hypermetabolic activity in the paravertebral region with a maximum SUV of 10.94 and abnormal uptake in both humeri and femurs, suggesting possible bone marrow involvement. The patient was diagnosed with AML and started on 7+3 induction chemotherapy with cytarabine and daunorubicin, along with radiotherapy for local disease control. This case highlights the diagnostic challenge of isolated myeloid sarcoma in the absence of peripheral blood abnormalities and emphasizes the importance of early hematologic evaluation. PET-CT played a crucial role in detecting subclinical bone marrow involvement, guiding treatment decisions. Recognizing myeloid sarcoma as a potential precursor to AML is essential for timely diagnosis and intervention, as early systemic chemotherapy can prevent disease progression and improve patient outcomes.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103910"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TREATMENT FOR HODGKIN’S LYMPHOMAS IN COUNTRIES WITH LIMITED RESOURCES","authors":"Khalid Halahleh","doi":"10.1016/j.htct.2025.103873","DOIUrl":"10.1016/j.htct.2025.103873","url":null,"abstract":"<div><div>The incidence and long-term clinical outcome of Hodgkin Lymphoma (HL) vary according to different patient, disease-related factors and geographic location. There have been dramatic changes in the staging and treatment of Hodgkin's Lymphoma (HL) over the last two decades. 75%‒80% of patients with classical HL can achieve long-term remission with contemporary risk-adapted frontline therapy in high income countries. However, 25%‒30% of patients with advanced-stage disease experience relapse or have primary refractory disease. For patients with Relapsed/Refractory HL (rrHL), salvage therapy followed by Stem Cell Transplantation (SCT) is the current standard of care. Despite the significant improvement in the diagnosis, staging, the use of risk-adapted approach, introduction of novel agents (CPI, Bv) in frontline setting, the use of post-transplant consolidation maintenance therapy, 50% of patients still experience disease progression, with poor prognosis and shortened survival. Most of the real-world data regarding treatment pathways and clinical outcomes in relapsed refractory HL published from high income countries in Euro and North America. There is a limited data on clinical characteristics and clinical outcomes of HL in low-resourced countries. Very few studies published so far with limited number of patients, single-center experiences, poor data quality, or lack of comprehensive information on patients, treatment, or clinical outcomes. In my presentation, we will highlight the disease entity from diagnosis, staging to treatment options worldwide; the availability and the use of novel agents in frontline and in relapsed refractory setting, availability of stem cell transplantation procedures and compare the clinical outcomes of HL patients in both high- and low-resourced countries.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103873"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"TRANSFORMATION OF FOLLICULAR LYMPHOMA INTO DIFFUSE LARGE B-CELL LYMPHOMA AFTER A DECADE OF REMISSION: A CASE REPORT","authors":"Elif Canpolat Hırfanoğlu ,&nbsp;Müjgan Çözeli ,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2025.103914","DOIUrl":"10.1016/j.htct.2025.103914","url":null,"abstract":"<div><h3>Introduction</h3><div>Follicular Lymphoma (FL) is the second most common subtype of Non-Hodgkin Lymphoma (NHL) and is generally indolent. However, a significant proportion of patients experience histologic transformation to Diffuse Large B-Cell Lymphoma (DLBCL), which leads to a more aggressive clinical course and worsened prognosis. Transformation typically occurs within the first few years of diagnosis, but this case presents a rare instance of transformation after a decade of complete remission, emphasizing the importance of long-term monitoring.</div></div><div><h3>Case presentation</h3><div>A 78-year-old male was diagnosed with FL in 2014 following excisional biopsy of a left supraclavicular lymph node. The patient underwent six cycles of R-CHOP chemotherapy, achieving complete remission and remained asymptomatic for 10-years. In 2024, he presented with a rapidly enlarging anterior chest wall mass. A contrast-enhanced CT scan revealed a 46 × 76 cm pleural-based tumor invading the sternum and pectoral muscle. A tru-cut biopsy confirmed Diffuse Large B-Cell Lymphoma (DLBCL) with CD20 positivity. Notably, there were no systemic B symptoms (fever, weight loss, night sweats), but the rapid extranodal tumor growth raised suspicion for transformation. Given the patient’s age and disease aggressiveness, rituximab plus ibrutinib therapy was initiated instead of intensive chemotherapy. The patient’s response is being closely monitored.</div></div><div><h3>Conclusion</h3><div>This case underscores the importance of long-term surveillance in FL patients, as transformation to DLBCL can occur even after a decade of remission. The presence of a rapidly growing, painless mass should raise suspicion for transformation, particularly in the absence of B symptoms. Extranodal involvement is a critical prognostic factor and often necessitates targeted therapeutic approaches. The use of rituximab and ibrutinib in this elderly patient represents a modern, less intensive treatment option for transformed FL, reflecting evolving lymphoma management strategies.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103914"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534558","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"THE HISTORY OF CHRONIC MYELOID LEUKEMIA (CML): FROM ARSENIC TO TYROSINE KINASE INHIBITORS (TKI)","authors":"Rüdiger Hehlmann","doi":"10.1016/j.htct.2025.103867","DOIUrl":"10.1016/j.htct.2025.103867","url":null,"abstract":"<div><div>It took 20-years from the first description of CML in 1845 to the report by Lissauer in 1865, of an effective treatment with arsenic. After another 38-years the beneficial effect of splenic irradiation was described in 1903, and after 50 more years the palliative efficacy of the alkylating agent busulfan (Galton, 1953). Several other agents were found effective (dibromomannitol, hydroxyurea), and in 1979 first reports on the efficacy of bone marrow transplantation were published. After more than 100-years of trial and error, the observation in 1960 of the Philadelphia (Ph)-chromosome, a translocation between chromosomes 9 and 22, marked the first step to understanding the pathophysiology of CML and to a rational and causative treatment approach. The breakpoint on chromosome 9 occurred in the gene encoding the ABL-oncogene. Most of ABL was translocated to chromosome 22 next to a region called Breakpoint Cluster Region (BCR). The detection of a BCR:ABL fusion RNA in CML (R. Gale contributed), prompted transfection experiments to mice which developed CML-like phenotypes (Daley et al, Heisterkamp and Groffen, 1990). Since the ABL-oncoprotein is a tyrosine kinase deregulated by juxtaposition next to BCR, the search for an inhibitor of BCR-ABL was the logical next step. The choice of imatinib as the first BCR-ABL-TKI was fortuitous and led to a profound change of CML treatment. Other TKI followed, but none-prolonged survival of CML compared to imatinib. Still, progression to blast crisis occurred in 6%‒7% of imatinib-treated cases, but CML-specific survival increased to more than 90% and survival of CML-patients diagnosed and treated in the chronic phase of CML approached that of the general population.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103867"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Onset of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis triggered by sudden reactivation of asymptomatic latent Epstein-Barr virus infection","authors":"Masanari Mizuki ,&nbsp;Takuya Terakawa ,&nbsp;Yuka Umeki ,&nbsp;Hitomi Matsunaga ,&nbsp;Yoshiki Matsuoka ,&nbsp;Wataru Nakahara ,&nbsp;Shogo Matsui ,&nbsp;Mako Ikeda ,&nbsp;Kodai Ueshima ,&nbsp;Yuya Hayasaka ,&nbsp;Nayu Matsuoka ,&nbsp;Yuma Tada ,&nbsp;Takahiro Matsui ,&nbsp;Kazumasa Oka ,&nbsp;Shuji Ueda","doi":"10.1016/j.htct.2025.103939","DOIUrl":"10.1016/j.htct.2025.103939","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 3","pages":"Article 103939"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}