Hematology, Transfusion and Cell Therapy最新文献

{"title":"TREATMENT OF BLAST PHASE MYELOPROLIFERATIVE NEOPLASM WITH THE COMBINATION OF AZACITIDINE, VENETOCLAX AND RUXOLITINIB","authors":"Fidan Khalilova,&nbsp;Azer Kerimov,&nbsp;Gulnar Kerimova","doi":"10.1016/j.htct.2025.103926","DOIUrl":"10.1016/j.htct.2025.103926","url":null,"abstract":"<div><h3>Objective</h3><div>In the development of Myeloproliferative Neoplasm (MPN), transformation to the Blast Phase (BP) is often noted. Thus, the incidence of BP in Primary Myelofibrosis (PMF) is -9%‒13%, in Essential Thrombocythemia (ET) -1%‒4%, and in Polycythemia Vera (PV) -3%‒7%. As a result of the development of ET and PV, transformation to Myelofibrosis (MF) can also be noted. In this case, differentiation of PMF from post-ET-MF and post-PV-MF can be difficult. In the treatment of these diseases, an individual approach according to the history and comorbidity, increases the effectiveness of treatment.</div></div><div><h3>Methodology</h3><div>Patient U.T., born in 1955, was registered at the NCHBT in June 2019 with a diagnosis of PMF. At the time of initial admission, the patient complained of abdominal distension and severe weight loss over the past 6-months. During examinations, a splenomegaly (204 × 85 mm) was found. In hemogram: Hb ‒ 151 g/L, WBC ‒ 43 × 10⁹/L, PLT ‒ 779 × 10⁹/L were. Histological examination of the bone marrow revealed that the bone cavities were filled with fibrotic stroma, no fat cells were detected. Hematopoietic cells were diffusely scattered, the cellular composition consisted of granulocytic and megakaryocytic orders. A reduction in the erythroid order was noted. The number of megakaryocytes was increased, acute polymorphism was noted, atypical forms were abundant. Megakaryocytes formed dense and sparse clumps (up to 6-cells) and layers, their paratrabecular localization was noted. Areas of coarse-fiber collagen fibrosis were noted. During molecular genetic examination, the allelic load of the JAK2V617F mutation was 92.694%. The patient was treated with Hydrea (HY)from June 2019 to September 2019. Since the hemogram did not show positive dynamics, Interferon (IFN) 3 million units was administered intramuscularly 3 times a week from September 2019. After this administration, a relative decrease in spleen size was noted. Starting from March 2020, the patient's condition deteriorated again. In hemogram: Hb ‒ 161 g/L, WBC ‒ 34 × 10⁹/L, PLT ‒ 343 × 10⁹/L were. The spleen size was 200 × 84 mm on Ultrasound Scan (USS). The patient was prescribed HY 1000 mg p/day along with IFN. Positive dynamics were achieved as a result of treatment with HY+IFN. Hemogram: Hb ‒ 120 g/L, WBC ‒ 4 × 10⁹/L, PLT ‒ 476 × 10⁹/L; spleen in palpation was +4 cm. Treatment with HY+IFN was continued until April 2021. From April 2021, treatment was continued with HY alone. In May 2024, the patient's condition worsened. Morphological examination of the bone marrow showed 16% blasts, histological examination showed 20% blasts, blasts were of myeloid type. Transformation of the disease to the BP was recorded. The patient was prescribed 2 courses of low-dose Cytosar. Since no positive dynamics were noted and blasts in the bone marrow increased to 78.6%, treatment with Azacitidine (AZA) ","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103926"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534314","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":"IMMUNOPHENOTYPIC CHARACTERISTICS AND TREATMENT OUTCOME OF T-ACUTE LYMPHOBLASTIC LEUKEMIA IN ADULTS; AN IRAQI CENTER EXPERIENCE","authors":"Abdulsalam Al-Ani ,&nbsp;Alaadin Sahham Naji ,&nbsp;Luma Essa Hamodi ,&nbsp;Alaa Fadhil Alwan","doi":"10.1016/j.htct.2025.103882","DOIUrl":"10.1016/j.htct.2025.103882","url":null,"abstract":"<div><h3>Objective</h3><div>About 25% of Acute Lymphoblastic Leukemia (ALL) express T-cell antigens which being considered a predictive of high-risk group. This study was conducted to find out the immunophenotypic characteristics of T-cell Acute Lymphoblastic Leukemia (T-ALL) in young and adult Iraqi patients. Also, to determine the association of treatment outcome with the immunophenotype and the treatment regimen used.</div></div><div><h3>Methodology</h3><div>The study was conducted using the laboratory data of the Central Flowcytometry Department at Baghdad Medical City between 7 January 2019 and 3 May 2020. The immunophenotypic records revealed 35 young and adult patients (age of 14-year or older) with T-ALL. The patients were classified into early (immature) T-cell Precursor (ETP) and Mature T-cell Precursor (MTP). Correlation of the patients’ outcome according to age, gender, and immunophenotypic expressions with the type of therapy used were evaluated.</div></div><div><h3>Results</h3><div>Thirty-five patients were diagnosed with T-ALL with a mean age of 28.1±12.3, and 74.3% were males. The stratification of patients according to the stage of leukemic T-cells maturation showed more frequent mature phenotype (cortical and medullary) than the immature (68.6% vs. 31.4%). The MTP markers expression showed a statistically significant higher rate in patients aged &lt; 20-year (p = 0.03) or male (p = 0.01). Most patients received hyperCVAD 26 (74.3%) protocol, and UKALL was administered in the remaining (25.7%). Remission was achieved in 82.9%, while 11.4% failed to respond and 8.7% died during induction. Remission was maintained in 54.3% with 5-months of median follow-up, and relapse was found in 11.4%. The Overall Survival (OAS) at one year was 55%, with a mean survival of 13.8+1.7 months without an association with the type of therapy, subtype of T-cell, and myeloid antigenic expression. Despite a higher remission rate and lower death rate among UKALL group compared to HyperCVAD, the difference is non-significant (p = 0.81). The better response of MTP compared to ETP lineage was the only significant value with the outcome (p = 0.045).</div></div><div><h3>Conclusion</h3><div>T-ALL is more commonly encountered in males. Remission was maintained in more than half of the patients with a better response and survival of the patients with MTP than those with the ETP subtype. The outcome was not affected by age, gender, or the treatment protocol.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103882"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534466","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":"RISK OF DEVELOPING ONCOHEMATOLOGICAL DISEASES IN INDIVIDUALS INFECTED WITH HEPATITIS C AND B VIRUS","authors":"Gunel Alıyeva,&nbsp;Aytan Shirinova","doi":"10.1016/j.htct.2025.103916","DOIUrl":"10.1016/j.htct.2025.103916","url":null,"abstract":"<div><h3>Objective</h3><div>There are data in the literature on the possible etiologic role of Hepatitis C Virus (HCV) in the development of some hematological malignancies. However, these studies were heterogeneous, for example, the control groups varied from blood donors to patients with other hematological malignancies and healthy individuals. In addition, most studies included a relatively small number of patients, which did not allow for unambiguous conclusions. Based on the above, the aim of this study was to identify a possible association between hepatitis B, C and hematological malignancies.</div></div><div><h3>Methodology</h3><div>797 patients of the National Center of Hematology and Transfusiology with various forms of malignant blood diseases and positive Hepatitis B (HbAgS) and C (anti-НСV) markers at the time of the diagnosis were analyzed. As a control group were used positive hepatitis B and C blood donors from the Central Blood Bank. The association between malignant blood diseases and infectious hepatitis was estimated using the Relative Risk (RR) and Odds Ratio (OR) with a 95% Confidence Interval. Differences were considered significant at p &lt; 0.05. Statistical analysis was performed using SPSS software.</div></div><div><h3>Results</h3><div>The study revealed a positive association between a positive anti-HCV status and the risk of developing Non-Hodgkin Lymphoma (NHL) (RR/OR = 11.7/13.3, p = 0), Acute Lymphoblastic Leukemia (ALL) (RR/OR = 6.76/7.22, p = 0), Chronic Lymphoid Leukemia (CLL) (RR/OR = 5.9/6.24, p = 0), Multiple Myeloma (MM) (RR/OR = 3.94/4.08, p = 0.008752) and Acute Myeloid Leukemia (AML) (RR/OR = 4.7/4.91, p = 0.000001). In cases of Myeloproliferative Neoplasms (MPN), a statistically significant association could not be determined. Hepatitis B was statistically significantly associated with NHL (RR/OR = 5.27/5.56, p = 0.000465), CLL (RR/OR = 5.31/5.61, p = 0.000001), MM (RR/OR = 5.31/5.61, p = 0.000001) and MPN (RR/OR = 4.3/4.48, p = 0.000570).</div></div><div><h3>Conclusion</h3><div>Thus, it can be concluded that hepatitis C and B viruses may play a role in the development of some hematological malignancies, such as leukemia and lymphoma. HBV can contribute to the development of hematological malignancies by changing cell proliferation and causing chronic inflammation, which can disrupt the normal functioning of the immune system. Also, chronic inflammation activates cytokines and growth factors that can increase the risk of cell mutation, which in turn leads to the development of malignant tumors.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103916"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534559","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":"CORRELATION OF СD10+ B-LYMPHOCYTES AND PLASMA CELLS WITH OUTCOME IN BREAST CANCER","authors":"Svetlana Chulkova","doi":"10.1016/j.htct.2025.103920","DOIUrl":"10.1016/j.htct.2025.103920","url":null,"abstract":"<div><h3>Objective</h3><div>Bone Marrow (BM) is poorly understood from the point of view of the prognostic role of hematopoietic cells and subpopulations of lymphocytes in patients with Breast Cancer (BC). In recent years, more attention has been paid to the study of the innate immune system, which includes B-lymphocytes. They produce IgM antibodies, which play an important role in the induction of apoptosis.</div></div><div><h3>Methodology</h3><div>Study was carried out in 107 BC patients’ stage I‒II. Adjuvant chemotherapy ‒ 65.4% of patients, radiation therapy ‒ 49.8%, hormone therapy ‒ 84% of patients. Her2/neu\"-\" 80%, Her2/neu\"+\"-18%, TNBC-12%. The duration of the follow-up period after surgery was 8-years. Multiparameter flow cytometry was used, FACSCANTO II. Studies of BM lymphocyte subpopulations were carried out in the gate of CD45++ cells: CD19, CD20, CD5, CD38, CD10, CD45, HLA-DR, CD27. Radical resection -38.3% of patients, mastectomy ‒ 59.7%.</div></div><div><h3>Results</h3><div>B1-cells was higher in B-Her2\"+\", stage IIA, with 2 affected lymph nodes. B1-cells correlated with plasma cells. The total percentage of B-cells in BM was significantly associated with the prognosis of BC. B1 cells were associated with progression-free and disease-free survival. Disease progression was observed at low levels of B1 cells. In cases more than 10% B-lymphocytes in the BM of BC patients’ Overall Survival (OS) rates were more favorable (p = 0.01). Especially for BC with a high Ki-67. Disease progression was observed in 1/3 of BC patients with low levels of B1 cells. CD38 expression on B-cells was a prognostically favorable factor: the role is realized during 5–10-years of follow-up after surgery. Level CD38+ B-cells more than 10% correlated with high OS (p = 0.02). The presence of CD10+CD19+ B-lineage precursors was associated with a more favorable prognosis (OS, the threshold level 12%, р = 0.04). The prognostic role of the CD10 antigen was realized when patients were observed for more than 5-years.</div></div><div><h3>Conclusion</h3><div>Total relative number of (more than 10%) of BM CD19+ cells were significantly related to OS in BC. B-cell precursors and CD38+ B-cells were associated with favorable prognosis. Prognostic role of B-lineage precursors and CD38- positive cells was in the periods of 5–10 years after surgery.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103920"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534563","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":"COLD AGGLUTININ DISEASE IN A PATIENT WITH WALDENSTRÖM’S MACROGLOBULINEMIA: A DIAGNOSTIC AND THERAPEUTIC CHALLENGE","authors":"Ceren Kısa ,&nbsp;Feride Aslanca ,&nbsp;Melisa Ural ,&nbsp;Birol Güvenç","doi":"10.1016/j.htct.2025.103911","DOIUrl":"10.1016/j.htct.2025.103911","url":null,"abstract":"<div><h3>Introduction</h3><div>Cold Agglutinin Disease (CAD) is a form of Autoimmune Hemolytic Anemia (AIHA) caused by IgM antibodies binding to erythrocytes at low temperatures, leading to complement-mediated hemolysis. CAD can be primary (idiopathic) or secondary, often associated with lymphoproliferative disorders, infections, or autoimmune diseases. Waldenström’s Macroglobulinemia (WM), a rare B-cell malignancy characterized by IgM overproduction, is an uncommon but important cause of secondary CAD. This case highlights the diagnostic and therapeutic challenges of CAD in a patient with relapsed WM.</div></div><div><h3>Case presentation</h3><div>A 66-year-old female was diagnosed with Waldenström’s Macroglobulinemia (WM) in 2012 based on a bone marrow biopsy. She initially received R-CHOP chemotherapy, achieving remission in 2015, followed by Autologous Stem Cell Transplantation (ASCT) in October 2015. After relapse in 2016, she was treated with bortezomib-rituximab followed by bortezomib monotherapy between 2016 and 2018. In October 2023, she started ibrutinib therapy for disease control. During routine blood tests in October 2023, hematologic discrepancies were noted: Hemoglobin (Hb): 7 g/L, Hematocrit (Hct): 13%, which corrected to Hb: 10.5 g/L and Hct: 31.2% after warming the sample to 37°C, raising suspicion for Cold Agglutinin Disease (CAD). Direct Coombs test was positive (1/16 IgM titer), confirming the diagnosis. Given the underlying lymphoproliferative disorder, the patient was started on rituximab therapy for CAD management while continuing ibrutinib for WM.</div></div><div><h3>Conclusion</h3><div>This case underscores the importance of considering CAD in patients with hematologic malignancies presenting with unexplained anemia and hemoglobin/hematocrit discrepancies. It highlights the necessity of warming blood samples in suspected cases, preventing misinterpretation of CBC results. Additionally, it demonstrates the crucial role of rituximab in managing CAD secondary to WM by targeting IgM-producing B-cells. Early recognition and treatment of secondary CAD in lymphoproliferative disorders can prevent complications and improve patient outcomes.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103911"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534619","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":"T-CELL LYMPHOMA DIAGNOSIS AND TREATMENT IN KOSOVO, A CROSS SECTIONAL STUDY","authors":"Adriatik Berisha","doi":"10.1016/j.htct.2025.103930","DOIUrl":"10.1016/j.htct.2025.103930","url":null,"abstract":"<div><h3>Background</h3><div>T-cell Lymphoma is a relatively common hematological malignancy in Kosovo compared to the other lymphoid malignancies. Among the other subtypes, Anaplastic large T-cell lymphoma is the most common. The diagnosis of this disease has increased in the last few years and the treatment with chemotherapy and other supportive care has still many challenges. In this study we aimed to better define the presenting features of these diseases in Kosovo.</div></div><div><h3>Methods</h3><div>Cross sectional retrospective epidemiological study. The data was collected during the period of June 2018 to June 2023.The data were collected from the chemotherapy treatment protocol books in the Hematology clinic of the UCC Kosovo. The studied population was constituted by patients aged 18-years old and older, both genders, diagnosed the treated with T-cell Lymphoma in the Hematology clinic of Kosovo. The diagnosis was made based on histopathological and immunohistochemical analysis of lymph nodes or bone marrow biopsies.</div></div><div><h3>Results</h3><div>During the period considered time-period, 44 patients were diagnosed and treated with T-cell lymphoma, the most common was Anaplastic large T-cell lymphoma (n = 9, 19.5%) followed by Enteropathy associated T-cell lymphomawith (n = 7, 14.6%), and NK/T-cell lymphoma with (n = 5, 9.7%). Other cases included a T Lymphoma/Leukemia accompanied by cirrhosis hepatis and the only case of gamma/delta T-cell lymphoma. Among the 44 TCL, 29 were treated with CHOP regimen as first line chemotherapy.</div></div><div><h3>Conclusions</h3><div>TCL are relatively common in Kosovo, with 44 cases diagnosed over 5-years. The majority of patients were treated with the CHOP chemotherapy protocol as first line therapy. The results of the treatments were successful in achieving remissions in a small number of patients. The patients that did not achieve remission received a second treatment protocol with mixed results and were sent to transplant center. Prolonged survival was exceptional, confirming the need for new targeted approaches.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103930"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534310","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":"PROGNOSTIC VALUE OF CD56 EXPRESSION IN CHILDREN WITH ACUTE MYELOID LEUKEMIA","authors":"Reyhan Aliyeva ,&nbsp;Azer Kerimov ,&nbsp;Naila Iasmayil ,&nbsp;Gunel Aliyeva","doi":"10.1016/j.htct.2025.103885","DOIUrl":"10.1016/j.htct.2025.103885","url":null,"abstract":"<div><h3>Introduction</h3><div>Expression of lymphoid markers (CD2, CD3, CD5, CD7) in Acute Myeloid Leukemia (AML) is an important prognostic factor that affects the clinical outcome of these patients. CD56 antigen is a NK cell marker that is expressed in several lymphohematopoietic neoplasms, including AML. The presence of CD56 antigen on blast cells can affect the duration of Complete Remission (CR), and is also associated with short overall survival and resistance to therapy. We studied a cohort of children diagnosed with AML treated from 2022‒2024 and assessed the association of CD56 expression with therapy outcomes.</div></div><div><h3>Methodology</h3><div>To determine the frequency of CD56 by flow cytometry in children with AML and to study the prognostic significance of this marker. Materials and Methods: The study included 31 patients aged 0‒16 years diagnosed from January 2022 to December 2024. The study was conducted on a BD FACS CANTO flow cytometer using an 8-color panel of monoclonal antibodies. Marker expression on blast cells of more than 20% was considered positive.</div></div><div><h3>Results</h3><div>СреThe total observation period was 31 months. The patients were divided into 3 age groups: 0‒5-years ‒ 5 (16%), 5‒10-years ‒ 12 (38.7%), 10‒16 years ‒ 14 (45%) patients, male ‒ 17 (54.8%), female ‒ 14 (45%). In the general observation group, 19 (62%) patients were in complete clinical and hematological remission, 10 (34%) patients had bone marrow relapse, 4% had resistance to therapy. In 7 (23%) cases, positive expression of CD56 was observed, of which 3 (9.6%) cases of AML with signs of maturation, 1 (3%) case of promyelocytic, 3 (9.6%) cases of myelomonoblastic leukemia. Among CD56 positive AML patients, mutations such as t(8;21)(q22;q22), ct(15;17), t(11q23), inv(16) were detected. Survival analysis was performed using the Kaplan-Meier method. The achievement of complete remission in response to induction chemotherapy between CD56-positive and CD56-negative groups was almost identical (85% and 81%). Relapse-free survival between CD56 positive and negative variants was significantly different (67% vs. 48%). Among children with AML with CD56-positive, higher relapse and mortality rates were observed than in the CD56-negative group (p &lt; 0.05).</div></div><div><h3>Conclusion</h3><div>We consider CD56 expression as an independent prognostic factor. It is recommended to keep in mind that the presence of this marker is associated with some cytogenetic abnormalities. CD56 is a potential factor for poor prognosis in groups of children with AML and should be taken into account when stratifying risk groups.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103885"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534467","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":"PROGNOSTIC IMPLICATIONS OF HIGH-RISK GENETIC MUTATIONS IN MULTIPLE MYELOMA PATIENTS UNDERGOING SECOND AUTOLOGOUS STEM CELL TRANSPLANT","authors":"Taner Tan,&nbsp;Ahmet Umur Topcu,&nbsp;Ümit Barbaros Üre,&nbsp;Emre Osmanbasoglu,&nbsp;Sinem Civriz Bozdağ,&nbsp;Olga Meltem Akay","doi":"10.1016/j.htct.2025.103927","DOIUrl":"10.1016/j.htct.2025.103927","url":null,"abstract":"<div><h3>Objective</h3><div>High-risk genetic mutations significantly influence prognosis in multiple myeloma. Although autologous Hematopoietic Stem Cell Transplantation (auto-HSCT) is a cornerstone of multiple myeloma treatment, the prognostic impact of genetic abnormalities in patients undergoing a second auto-HSCT warrants further investigation. This study aims to evaluate the prognostic significance of high-risk genetic mutations in multiple myeloma patients undergoing a second auto-HSCT.</div></div><div><h3>Methodology</h3><div>This retrospective analysis evaluated 26 multiple myeloma patients who underwent a second auto-HSCT between May 5, 2017, and December 10, 2024. Detailed analysis was conducted on 19 patients with available pre-transplant Fluorescence In Situ Hybridization (FISH) data. Among these, 9 patients underwent tandem transplantation, and 10 underwent a non-tandem second auto-HSCT. Prognostic analyses focused on genetic abnormalities detected by FISH.</div></div><div><h3>Results</h3><div>The analyzed cohort included 10 males (52.6%) and 9 females (47.4%), with a mean age of 56.79-years (SD = 10.39, range 34–69). Median follow-up post-second transplantation was 31-months (IQR 18–54). Median intervals between two transplantations were 16-months (IQR 4–72.5) overall and 62-months (IQR 31–93) excluding tandem cases. High-risk genetic mutations were detected in 11 of 19 analyzed patients (57.9%): deletion 17p and amplification 1q (each 26.3%), t(4;14) (15.8%), deletion 1p (10.5%), and t(14;16) (5.3%). Patients with high-risk mutations had a higher mortality rate (54.5% vs. 25%), although not statistically significant overall (p = 0.198). Amplification 1q was significantly associated with increased mortality (80% vs. 28.6%, p = 0.048). Kaplan-Meier analysis revealed significantly shorter overall survival for patients with ≥ 2 high-risk mutations (16.10 vs. 59.43 months, p = 0.017), amplification 1q (32.50 vs. 60.57 months, p = 0.048), and deletion 17p (18.50 vs. 59.12 months, p = 0.030). Platelet engraftment was significantly delayed in patients with at least one high-risk mutation (12.64 vs. 10.88 days, p = 0.033). Neutrophil engraftment and hospital stay durations were not significantly different. Pre-transplant hemoglobin, platelet, and neutrophil counts showed no correlation with survival, engraftment times, or hospital stay duration. Survival outcomes were similar between tandem and non-tandem transplantation groups; however, within the tandem subgroup, genetic mutations were associated with higher mortality (66.7% vs. 0%, p = 0.058).</div></div><div><h3>Conclusion</h3><div>High-risk genetic mutations, particularly amplification 1q and deletion 17p, significantly predict poorer survival outcomes following second autologous Hematopoietic Stem Cell Transplantation (auto-HSCT) in multiple myeloma patients. Patients harboring these mutations exhibit higher mortality rates and delayed platelet engraftment, underscoring the clinical ","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103927"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534485","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":"CHIC2 DELETION-ASSOCIATED HYPEREOSINOPHILIA AND SUBSEQUENT JAK2 V617F POSITIVE THROMBOCYTOSIS","authors":"Mürüvvet Seda AYDIN,&nbsp;Emel Isleyen,&nbsp;Funda Ceran,&nbsp;Simten Dagdas,&nbsp;Gulsum Ozet","doi":"10.1016/j.htct.2025.103899","DOIUrl":"10.1016/j.htct.2025.103899","url":null,"abstract":"<div><h3>Background and aim</h3><div>Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK) are myeloid or lymphoid neoplasms driven by rearrangements involving genes encoding specific tyrosine kinases. These BCR::ABL1-negative diseases have long been recognized for their sensitivity to tyrosine kinase inhibitors. Herein, we present an interesting case diagnosed as myeloid neoplasm with abnormality of PDGFRA.</div></div><div><h3>Case presentation</h3><div>A 63-year-old female patient with known glaucoma, hypothyroidism, and vitiligo was admitted to our clinic 8-years ago with fatigue. The patient had splenomegaly and eosinophil-predominant (10 × 10⁹/L) leukocytosis (70.8 × 10⁹/L). Secondary causes of eosinophilia (rheumatologic, infectious and immunologic) were excluded. Although not directly attributed to the patient's hypereosinophilia, echocardiographic left ventricular contraction abnormality was observed. Bone marrow aspiration and biopsy were hypercellular and showed 35%‒40% eosinophils. With a preliminary diagnosis of hypereosinophilic syndrome, the patient was treated with steroids and then with hydroxyurea. Karyotype analysis was normal and FISH for t(9;22) was negative. The FISH panel revealed a 76% CHIC2 (4q12) deletion, but was negative for abnormalities of PDGFRB, FGFR1, and FIP1L1::PDGFRA fusion. The patient was switched to imatinib treatment. While the patient was followed in hematological remission for a long time with imatinib, thrombocytosis (807 × 10⁹/L) was detected in the patient six months ago. The patient had suppressed erythropoietin level (1.94 mu/mL) and JAK2 V617F mutation. Low-dose hydroxyurea was combined with imatinib. Hematological remission was regained.</div></div><div><h3>Discussion</h3><div>The majority of MLN-TK cases associated with PDGFRA rearrangements have cytogenetically cryptic deletion of 4q12 resulting in FIP1L1::PDGFRA. Although FIP1L1::PDGFRA fusion could not be demonstrated in this patient, it was thought that the patient had a myeloid neoplasm with abnormality of PDGFRA class due to CHIC2 deletion and typical clinical findings. The fact that the patient responded to treatment for many years seems to be evidence of this. The detection of JAK2 mutation during follow-up raised the question of whether this clone was present in the patient from the beginning or was acquired later.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103899"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534300","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":"IMPACT OF LENALIDOMIDE MAINTENANCE DOSAGE ON SURVIVAL OUTCOMES IN MULTIPLE MYELOMA","authors":"Zeynep Kürüm,&nbsp;Ayfer Gedük","doi":"10.1016/j.htct.2025.103903","DOIUrl":"10.1016/j.htct.2025.103903","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Objective&lt;/h3&gt;&lt;div&gt;This study aimed to assess the impact of lenalidomide maintenance dosing on clinical outcomes and the development of lenalidomide refractoriness in Multiple Myeloma (MM) patients who received maintenance therapy following Autologous Stem Cell Transplantation (ASCT) at our institution.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methodology&lt;/h3&gt;&lt;div&gt;A retrospective analysis was conducted on 82 MM patients who underwent lenalidomide maintenance therapy on a 21/28-day cycle post-ASCT. Low-dose maintenance was defined as a dose below the maximum allowable level, determined by the patient’s Glomerular Filtration Rate (GFR) and hematologic parameters at initiation. The Maximum Tolerable Dose (MTD) was defined as the highest dose a patient could tolerate based on these criteria.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;In the overall cohort, median PFS was 56.0-months (95% CI: 40.15‒71.85) and median OS was not reached. However, 88.3% of patients were alive at 60-months and 68.2% at 120-months. A response of ≥ VGPR was observed in 91% of patients receiving low-dose maintenance therapy, compared to 73.3% in those receiving treatment at the maximum tolerated dose (p = 0.005). While the median PFS was 56.0-months (95% CI 47.06‒64.93) in those receiving low-dose maintenance; the median PFS was 33-months (95% CI 23.36‒42.63) in those receiving maintenance at the MTD (p = 0.166). Dose reductions during maintenance therapy due to adverse effects were reported in 17 patients (20.7%). Of these, 11 patients (16.9%) were initially on low-dose maintenance, while 6 patients (40%) were on the MTD (p = 0.42). The median duration of maintenance therapy was 21 months (6–34) for patients on low-dose maintenance and 11 months (4–24) for those on the MTD (p = 0.114). Second-line treatment was administered to 40 patients (48.7%) who experienced progression. The median PFS2 was 25.36-months (95% CI 9.24–41.48), and the median OS2 was 73.23-months (95% CI 42.50–103.95). Median PFS2 was 25.43-months (95% CI 0.20‒50.66) and the survival rate was 70% at 60-months in those receiving lenalidomide-based second line therapy, while median PFS2 was 25.36-months (95% CI 6.30‒44.42) and the survival rate was 53.8% at 60-months in those receiving lenalidomide free therapy (p = 0.978, p = 0.902 respectively). Following low-dose maintenance therapy, the median PFS2 was 26.167-months (95% CI 5.571–46.762) in the lenalidomide free therapy group and 25.433-months (95% CI 0.08–50.787) in the lenalidomide-based second line therapy group (p = 0.581). Although median OS2 could not be calculated, at 60-months, the survival rate was 74.5% in patients receiving lenalidomide-based treatment, while it was 62.3% in patients receiving treatment without lenalidomide (p = 0.637).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;This study introduces the concept of low dose versus MTD lenalidomide maintenance. MTD does not confer a survival benefit and is associated with increased toxicity. Our findings support low","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103903"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534482","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}