RESEARCH ARTICLE | |
1. | Disseminated Intravascular Coagulation in Acute Promyelocytic Leukemia Patients: A Retrospective Analysis of Outcomes and Healthcare Burden in US Hospitals Rushin Patel, Darshil Patel, Mrunal Patel, Jessica Ohemeng-Dapaah, Afoma Onyechi, Zalak Patel, Chieh Yang, Safia Shaikh doi: 10.4274/tjh.galenos.2024.2023.0479 Pages 1 - 8 Objective: Acute promyelocytic leukemia (APL) is associated with an elevated risk of developing disseminated intravascular coagulation (DIC). The purpose of this study was to assess the outcomes of hospitalizations related to DIC in APL and their impact on healthcare. Materials and Methods: This study entailed a cross-sectional and retrospective analysis of the US National Inpatient Sample database. We identified adults with APL and categorized them into groups of patients with and without DIC. Our focus areas included in-hospital mortality, length of stay, charges, and complications associated with DIC. Unadjusted odds ratios/coefficients were computed in univariate analysis, followed by adjusted odds ratios (aOR)/coefficients from multivariate analysis that accounted for confounding factors. Results: Our analysis revealed that APL patients with DIC had a substantially higher aOR for mortality (aOR: 6.68, 95% confidence interval [CI]: 4.76-9.37, p<0.001) and a prolonged length of stay (coefficient: 10.28 days, 95% CI: 8.48-12.09, p<0.001) accompanied by notably elevated total hospital charges (coefficient: $215,512 [95% CI: 177,368-253,656], p<0.001), thereby emphasizing the reality of extended medical care and economic burden. The presence of DIC was associated with increased odds of sepsis, vasopressor support, pneumonia, acute respiratory failure, intubation/mechanical ventilation, and acute kidney injury, reflecting heightened vulnerability to these complications. Patients with DIC demonstrated significantly higher odds ratios for major bleeding, intracranial hemorrhage, gastrointestinal bleeding, red blood cell transfusion, platelet transfusion, fresh frozen plasma transfusion, and cryoprecipitate transfusion, highlighting the pronounced hematological risks posed by DIC. Conclusion: This study has revealed the significant associations between DIC in APL and various outcomes, underscoring the clinical and economic implications of these conditions. The hematological risks further increase patients’ vulnerability to bleeding events and the need for transfusions. |
2. | Real-Life Data on the Efficacy and Safety of Letermovir for Primary Prophylaxis of Cytomegalovirus in Allogeneic Hematopoietic Stem Cell Recipients: A Single-Center Analysis Martyna Wlodarczyk, Agata Wieczorkiewicz Kabut, Krzysztof Bialas, Anna Koclega, Izabela Noster, Patrycja Zielinska, Grzegorz Helbig doi: 10.4274/tjh.galenos.2024.2024.0026 Pages 9 - 15 Objective: Cytomegalovirus (CMV) reactivation is a life-threatening complication after allogeneic hematopoietic stem cell transplantation (HSCT). Introduction of letermovir (LMV) seems to improve posttransplant outcomes, but delayed-onset CMV reactivation still remains a challenge. In this study, we report on our first experience with LMV prophylaxis in 93 CMV-seropositive adult patients receiving HSCT in our center. Materials and Methods: We retrospectively analyzed the data of 93 adult CMV-seropositive recipients receiving LMV as CMV prophylaxis after HSCT for hematological malignancies between 2019 and 2023. The starting LMV dose was 480 mg daily, reduced to 240 mg daily for those receiving cyclosporin A co-administration. CMV DNA in the blood was measured by real-time polymerase chain reaction weekly for the first 2 months after transplantation, then every other week until the end of immunosuppressive treatment. LMV was continued to day +100 or to CMV reactivation. Results: The median recipient age at the time of transplant was 51 (range: 20-71) years. All patients received grafts from peripheral blood, mostly for acute myeloid leukemia (60%). The median time from transplantation to LMV initiation was 3 (range: 0-24) days. While 55% of patients were transplanted from matched related donors, 32% had unrelated donors and 13% underwent haploidentical HSCT. Four patients (4%) had CMV “blips” while on LMV, but the drug was continued and repeated assays were negative. Only 2 patients (2%) experienced CMV reactivation while on LMV, on days 48 and 34 after HSCT, respectively. Seven patients (7%) developed late-onset CMV reactivation after a median of 124 days after HSCT (range: 118- 152 days) and they were successfully treated with ganciclovir. CMV disease was not observed. Grade III-IV acute graft-versus-host disease occurred in 6 patients (6%) during LMV treatment. LMV treatment was free of side effects. Conclusion: The median recipient age at the time of transplant was 51 (range: 20-71) years. All patients received grafts from peripheral blood, mostly for acute myeloid leukemia (60%). The median time from transplantation to LMV initiation was 3 (range: 0-24) days. While 55% of patients were transplanted from matched related donors, 32% had unrelated donors and 13% underwent haploidentical HSCT. Four patients (4%) had CMV “blips” while on LMV, but the drug was continued and repeated assays were negative. Only 2 patients (2%) experienced CMV reactivation while on LMV, on days 48 and 34 after HSCT, respectively. Seven patients (7%) developed late-onset CMV reactivation after a median of 124 days after HSCT (range: 118- 152 days) and they were successfully treated with ganciclovir. CMV disease was not observed. Grade III-IV acute graft-versus-host disease occurred in 6 patients (6%) during LMV treatment. LMV treatment was free of side effects. |
3. | Oncolytic Myxoma virus Increases Autophagy in Multiple Myeloma Alpay Yeşilaltay, Dilek Muz, Berna Erdal doi: 10.4274/tjh.galenos.2024.2023.0403 Pages 16 - 25 Objective: Multiple myeloma, which affects plasma cells, is the second most common hematological malignancy. Despite the development of new drugs and treatment protocols, patient survival has not reached the desired level. In this study, we investigated the effects of Myxoma virus (MYXV), an oncolytic virus, on autophagy in myeloma cells. Materials and Methods: We analyzed protein expressions of ATG-5, p62, Beclin-1, LC3B, and the apoptosis marker Bcl-2 as autophagy markers in human U-266 and mouse MOPC-315 myeloma cell lines subjected to different doses of MYXV. In addition, autophagic images of myeloma cells were investigated using transmission electron microscopy (TEM). Results: In the first 24 h, which is the early stage of autophagy, ATG-5 and Beclin-1 expression levels were increased in the U-266 and MOPC- 315 cell lines in the groups that had received MYXV at a multiplicity of infection of 15. At 48 h, a significant increase was detected in the expression of LC3B, which is a late indicator. Autophagosomes were observed in myeloma cells by TEM. Conclusion: MYXV shows an antimyeloma effect by increasing autophagy in myeloma cells. |
4. | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients Zehra Narlı Özdemir, Yıldız İpek, Pusem Patir, Gözde Ermiş, Rafiye Çiftçiler, Deniz Özmen, Mehmet Baysal, Vildan Gürsoy, Esra Yıldızhan, Serkan Güven, Tarık Ercan, Tayfun Elibol, Sinan Mersin, Eylem Genç, Eren Arslan Davulcu, Volkan Karakuş, Nergiz Erkut, Gürsel Güneş, Reyhan Diz Küçükkaya, Ahmet Emre Eşkazan doi: 10.4274/tjh.galenos.2024.2023.0430 Pages 26 - 36 Objective: In this study, we investigated the effects of calreticulin (CALR) and JAK2V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET). Materials and Methods: Seventeen centers from Türkiye participated in the study and CALR- and JAK2V617F-mutated ET patients were evaluated retrospectively. Results: A total of 302 patients were included, of whom 203 (67.2%) and 99 (32.8%) were JAK2V617F- and CALR-positive, respectively. CALR-mutated patients were significantly younger (51 years vs. 57.5 years, p=0.03), with higher median platelet counts (987x109/L vs. 709x109/L, p<0.001) and lower median hemoglobin levels (13.1 g/dL vs. 14.1 g/dL, p<0.001) compared to JAK2V617F-mutated patients. Thromboembolic events (TEEs) occurred in 54 patients (17.9%), 77.8% of which were arterial. Compared to CALR mutation, JAK2V617F was associated with a higher risk of thrombosis (8.1% vs. 22.7%, p=0.002). Rates of transformation to myelofibrosis (MF) and leukemia were 4% and 0.7%, respectively, and these rates were comparable between JAK2V617F- and CALR-mutated cases. The estimated overall survival (OS) and MF-free survival of the entire cohort were 265.1 months and 235.7 months, respectively. OS and MF-free survival durations were similar between JAK2V617F- and CALR-mutated patients. Thrombosis-free survival (TFS) was superior in CALR-mutated patients compared to JAK2V617F-positive patients (5-year TFS: 90% vs. 71%, respectively; p=0.001). Age at diagnosis was an independent factor affecting the incidence of TEEs. Conclusion: In our ET cohort, CALR mutations resulted in higher platelet counts and lower hemoglobin levels than JAK2V617F and were associated with younger age at diagnosis. JAK2V617F was strongly associated with thrombosis and worse TFS. Hydroxyurea was the most preferred cytoreductive agent for patients with high thrombosis risk. |
PERSPECTIVE IN HEMATOLOGY | |
5. | Antiphospholipid Syndrome: To Classify or Not to Classify? Doruk Erkan doi: 10.4274/tjh.galenos.2024.2024.0003 Pages 37 - 40 Antiphospholipid syndrome (APS) is a systemic autoimmune disorder resulting in thrombosis, microvascular disease, morbidity in pregnancy, and/or non-thrombotic manifestations. The recently introduced 2023 American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) APS classification criteria, with significantly higher specificity compared to the revised Sapporo criteria, now reflect the current thinking about APS and provide a new foundation for future APS research. The purpose of this short commentary is to discuss the appropriate circumstances under which the 2023 ACR/EULAR classification criteria could be used and to demonstrate how the new criteria can be applied to simple case scenarios. |
IMAGES IN HEMATOLOGY | |
6. | The Many Faces of Multiple Myeloma Asya Tuğçe Bol, Güldane Cengiz Seval, Meral Beksaç, Işınsu Kuzu doi: 10.4274/tjh.galenos.2023.2023.0183 Pages 41 - 42 Abstract | |
7. | Advanced Cutaneous Peripheral T-cell Lymphoma-Not Otherwise Specified with Extensive Ulceronecrotic Dyschromic Plaques and Poor Outcome Vishnu Sharma, Vansh Bagrodia doi: 10.4274/tjh.galenos.2023.2023.0222 Pages 43 - 44 Abstract | |
8. | Unusual Clasmatosis Morphology Antonio La Gioia, Miriam Marsano, Fabiana Fiorini doi: 10.4274/tjh.galenos.2023.2023.0275 Pages 45 - 46 Abstract | |
LETTER TO EDITOR | |
9. | Epithelial Cells or Vascular Smooth Muscle Cells in a Peripheral Blood Smear? Wei Yang, Yongliang Wang, Wei Pan doi: 10.4274/tjh.galenos.2024.2023.0399 Pages 47 - 48 Abstract | |
10. | The Importance of Rare Genes on the Prognosis of B-cell Acute Lymphoblastic Leukemia Li Xiang, Yongliang Wang, Wei Pan doi: 10.4274/tjh.galenos.2024.2023.0400 Pages 49 - 50 Abstract | |
11. | Immunoglobulin Replacement Therapy for Hypogammaglobulinemia in Multiple Myeloma Should Not Be Ignored Qinggang Zhang, Yongliang Wang, Wei Pan doi: 10.4274/tjh.galenos.2024.2023.0401 Pages 51 - 52 Abstract | |
12. | Circulating Monocytes Phagocytosing Lymphocytes in the Small-Cell Variant of T-Cell Prolymphocytic Leukemia Shuai Zhang, Meng Ma, Yanping Liu, Ying Bu, Zhe Zhang, Yun Zhang doi: 10.4274/tjh.galenos.2023.2023.0422 Pages 53 - 54 Abstract | |
13. | Successful Management of Ibrutinib-Induced Thrombocytopenia in a Patient with Chronic Lymphocytic Leukemia: No Interruption, Only Reduction Simge Erdem, Meliha Nalçacı doi: 10.4274/tjh.galenos.2023.2023.0426 Pages 55 - 56 Abstract | |
14. | Ibrutinib-Associated Leukocytoclastic Vasculitis in a Patient with Chronic Lymphocytic Leukemia Ayşe Kaya, İbrahim Aras, Pervin Özkan Kurtgöz, Umut Çakıroğlu doi: 10.4274/tjh.galenos.2023.2023.0368 Pages 57 - 58 Abstract | |
15. | Nilotinib-Associated Multiple Silent Arterial Stenoses in a Patient with Chronic Myeloid Leukemia Mert Tokatlı, Rashad Ismayilov, Olgu Erkin Çınar, İbrahim C. Haznedaroğlu doi: 10.4274/tjh.galenos.2023.2023.0288 Pages 59 - 60 Abstract | |
16. | Ruxolitinib for the Treatment of Refractory Idiopathic Multicentric Castleman Disease: A Case Report Yu-han Gao, Ming-hui Duan, Jian Li, Lu Zhang doi: 10.4274/tjh.galenos.2024.2023.0477 Pages 61 - 63 Abstract | |
17. | Rifampicin-Induced Toxic Hepatitis in a Patient with Hemophilia After Chemical Synovectomy Mehmet Can Uğur, Semih Aydoğdu, Elçil Kaya Biçer, Can Balkan, Kaan Kavaklı doi: 10.4274/tjh.galenos.2024.2023.0420 Pages 64 - 65 Abstract | |
18. | Analysis of Hereditary FXII Deficiency Caused by Three Mutations Including a Novel Mutation Longying Ye, Meina Liu, Lihong Yang, Mingshan Wang, Yaosheng Xie doi: 10.4274/tjh.galenos.2024.2024.0016 Pages 66 - 68 Abstract | |