Grace S. Park , Himachandana Atluri , Courtney D. DiNardo , Bryan Guillroy , Jean Horak , Effrosyni Apostolidou , Maryam Buni , Guillermo Montalban Bravo , Naveen Pemmaraju
{"title":"Management of hemolytic transfusion reactions in a patient with chronic myelomonocytic leukemia and rare antibodies: A case report","authors":"Grace S. Park , Himachandana Atluri , Courtney D. DiNardo , Bryan Guillroy , Jean Horak , Effrosyni Apostolidou , Maryam Buni , Guillermo Montalban Bravo , Naveen Pemmaraju","doi":"10.1016/j.lrr.2024.100485","DOIUrl":"10.1016/j.lrr.2024.100485","url":null,"abstract":"<div><div>Delayed hemolytic transfusion reaction (DHTR) poses a significant challenge in patients receiving blood transfusions. This case report highlights the complexities of managing DHTR in a newly diagnosed chronic myelomonocytic leukemia (CMML) patient with clinically significant JKa and little c antibodies during induction chemotherapy. A 46-year-old woman with CMML-2 who presented for induction chemotherapy was found to have hemolytic anemia. Due to presence of JKa and little c antibodies, she required intensive monitoring and supportive care measures. The coexistence of JKa and little c antibodies complicates transfusion management and chemotherapy tolerance in CMML patients.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100485"},"PeriodicalIF":0.7,"publicationDate":"2024-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142744315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acute lower limb ischemia revealing hypo granular acute promyelocytic leukemia","authors":"Sabrina Belmahi , Zainab Kajeiou , Loubna Yacoubi , Noussaiba Azzi , Mounia Slaoui , Abdelilah Berhili , Mohammed Bensalah , Rachid Seddik","doi":"10.1016/j.lrr.2024.100488","DOIUrl":"10.1016/j.lrr.2024.100488","url":null,"abstract":"<div><h3>Introduction</h3><div>Acute promyelocytic leukemia (AML-M3), classified as acute Myeloid leukemia with PML RARA according to the 5th edition of the World Health Organization classification of haematolymphoid tumors 2022 [1], is marked by abnormal promyelocyte proliferation and is known for high risks of bleeding and thromboembolic complications. We present a case where lower limb ischemia revealed this leukemia in a child.</div></div><div><h3>Case report</h3><div>An 11-year-old with minor ankle trauma developed severe lower limb ischemia, leading to the discovery of subtotal femoral artery thrombosis. Blood tests revealed hyperleukocytosis, thrombocytopenia, and anemia with 88 % blasts, confirming acute myeloid leukemia (AML-M3). Karyotyping showed a t(15;17) translocation, and the child was started on emergency chemotherapy.</div></div><div><h3>Discussion</h3><div>Acute promyelocytic leukemia (APL), classified as AML-M3 with PML-RARA, is characterized by abnormal promyelocytes and accounts for about 10 % of acute leukemias, mostly in middle-aged adults. It has two variants: common hypergranular and rare hypogranular forms. APL can present with bone marrow failure, anemia, bleeding, and occasionally thromboembolic events, as seen in this case. The ischemia mechanism is not fully understood but may involve vessel obstruction by blasts or hypercoagulability. Diagnosis relies on clinical, morphological, phenotypic, and cytogenetic evidence, with treatment involving all-trans retinoic acid (ATRA) and arsenic trioxide (ATO).</div></div><div><h3>Conclusion</h3><div>Hypogranular acute promyelocytic leukemia (AML3v) is a rare form and is even rarer when it is discovered following an ischaemic event, which is what makes our case so special.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100488"},"PeriodicalIF":0.7,"publicationDate":"2024-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142744314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Quang Hao Nguyen , Minh Phuong Vu , Ha Trang Kieu , Duc Binh Vu , Ha Thanh Nguyen , Quoc Khanh Bach
{"title":"Treatment of Vietnamese patients diagnosed with myelodysplastic neoplasms: Practical experience in a developing country","authors":"Quang Hao Nguyen , Minh Phuong Vu , Ha Trang Kieu , Duc Binh Vu , Ha Thanh Nguyen , Quoc Khanh Bach","doi":"10.1016/j.lrr.2024.100490","DOIUrl":"10.1016/j.lrr.2024.100490","url":null,"abstract":"<div><h3>Background</h3><div>Treatment of patients diagnosed with myelodysplastic neoplasms (MDS) is difficult and the outcome is still limited, especially in developing countries. We conducted this study in order to share some experience in treating patients diagnosed with MDS in developing countries.</div></div><div><h3>Methods</h3><div>This was a retrospective study that included 32 patients with newly MDS. 13 lower-risk patients, including 2 patients with MDS 5q- were treated with erythropoiesis stimulating agent (ESA). 19 patients with higher risk were treated with hypomethylating agent (HMA), which was decitabine.</div></div><div><h3>Results</h3><div>In the ESA treatment group, the rate of hematologic improvement-erythroid was 69.2 %, the rate of total hematologic improvement (with 3 lineages improvement) was 61.5 %. In the HMA treatment group, the overall response rate was 52.6 %. The follow-up times were 42 months. The overall survival (OS), leukemic transformation-free survival (LFS), and progression-free survival (PFS) of the ESA treatment group were 30.44, 28.91, and 28.29 months; respectively. The OS, LFS, and PFS of the HMA treatment group were 34.27, 31.45, and 26.83 months; respectively<strong>.</strong></div></div><div><h3>Conclusions</h3><div>Patients with lower risk MDS, including MDS 5q-, may benefit from treatment with erythropoiesis stimulating agent (ESA). Patients with higher risk MDS may have a favorable outcome with decitabine (HMA) treatment.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100490"},"PeriodicalIF":0.7,"publicationDate":"2024-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142744312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Septic arthritis as breakthrough invasive fusariosis after cord blood transplantation","authors":"Shinichi Katsuoka , Hidehiro Itonaga , Yasushi Sawayama , Masahiko Chiwata , Haruka Watanabe , Yuichi Yamada , Machiko Fujioka , Takeharu Kato , Shinya Sato , Koji Ando , Masato Tashiro , Takahiro Takazono , Yoshitaka Imaizumi , Koichi Izumikawa , Katsunori Yanagihara , Hiroshi Mukae , Yasushi Miyazaki","doi":"10.1016/j.lrr.2024.100483","DOIUrl":"10.1016/j.lrr.2024.100483","url":null,"abstract":"<div><div>A 63-year-old male received a third allogeneic hematopoietic stem cell transplantation with voriconazole prophylaxis for relapsed acute myeloid leukemia. He developed septic arthritis without any typical skin lesions due to fungal infection on day 42. Treatment with liposomal amphotericin B was initiated following surgical debridement; however, he died of progressive fungal infection. Ribosomal DNA sequencing identified <em>Fusarium solani</em> species complex (FSSC) harboring voriconazole resistance. This clinical course indicates that breakthrough invasive fusariosis (azole-resistant FSSC infection) needs to be considered as a pathogen when patients with hematological malignancies develop septic arthritis without typical skin lesions during voriconazole prophylaxis.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"22 ","pages":"Article 100483"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142358671","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jeffrey Baron , Manu R. Pandey , Elizabeth A. Griffiths , Eunice S. Wang
{"title":"Treatment of Philadelphia chromosome-positive acute lymphoblastic leukemia in Jehovah's witness patients","authors":"Jeffrey Baron , Manu R. Pandey , Elizabeth A. Griffiths , Eunice S. Wang","doi":"10.1016/j.lrr.2024.100474","DOIUrl":"10.1016/j.lrr.2024.100474","url":null,"abstract":"","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"22 ","pages":"Article 100474"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213048924000645/pdfft?md5=3040d4d2694de947d12371d548eb36f2&pid=1-s2.0-S2213048924000645-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141849553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mobachir El Kettani , Kashish Shah , Hareem Farooq , Ke Li , Talha Badar
{"title":"Chronic myeloid leukemia with involvement of membranous labyrinth","authors":"Mobachir El Kettani , Kashish Shah , Hareem Farooq , Ke Li , Talha Badar","doi":"10.1016/j.lrr.2024.100471","DOIUrl":"10.1016/j.lrr.2024.100471","url":null,"abstract":"<div><p>This case report explains an extraordinary presentation of chronic myeloid leukemia (CML) in a 39-year-old male with a T315I mutation, presenting with acute bilateral hearing loss and imbalance secondary to myeloid blast crisis. Neurological involvement was confirmed through MRI brain and cerebrospinal fluid analysis. Initial treatment with ponatinib and FLAG (fludarabine, cytarabine, G-CSF) regimen showed promise, but complications necessitated discontinuation. The patient's complex clinical trajectory, marked by complications and intolerance to tyrosine kinase inhibitors, highlights the intricate nature of CML blast crisis with T315I mutation management. Recognizing atypical presentations and early mutation analysis are pivotal for tailored treatment strategies.</p></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"22 ","pages":"Article 100471"},"PeriodicalIF":0.7,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S221304892400061X/pdfft?md5=72e11a1ea336e7231d57c0f3856bed99&pid=1-s2.0-S221304892400061X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141849867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Real-world treatments and clinical outcomes in unfit AML patients receiving first-line treatment or best supportive care in Italy (CURRENT study)","authors":"Maria Paola Martelli , Nicola Di Renzo , Antonio Curti , Nicola Stefano Fracchiolla , Luca Maurillo , Morena Caira , Paola Finsinger , Giuliana Gualberti , Felicetto Ferrara , Attilio Olivieri","doi":"10.1016/j.lrr.2024.100453","DOIUrl":"https://doi.org/10.1016/j.lrr.2024.100453","url":null,"abstract":"<div><p>Real-world data on treatment patterns and outcomes of patients with acute myeloid leukemia unfit for intensive chemotherapy are lacking before the advent of precision medicine in this setting.</p><p>Herein, we present the Italian sub-analysis of the CURRENT study in AML patients unfit for first line intensive chemotherapy, evaluating patients’ outcomes between 2015 and 2018.</p><p>Among 74 evaluable patients, 62 received systemic treatments (most used therapy was hypomethylating agents), while 12 best supportive care.</p><p>Key results include both efficacy and safety data, as well as HCRU and treatment patterns. In first-line therapy cohort median OS was 13.4 months vs. 2.7 months for BSC.</p></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"21 ","pages":"Article 100453"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213048924000438/pdfft?md5=f5c1ed3740748c30eca95f71c3a15eda&pid=1-s2.0-S2213048924000438-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140063297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A META-ANALYSIS OF COMPLICATED CASES OF INFLAMMATORY BOWEL DISEASE AND BONE MARROW FAILURE SYNDROME IN JAPAN","authors":"T. Yagi , H. Kumagai , A. Shimada","doi":"10.1016/j.lrr.2024.100421","DOIUrl":"https://doi.org/10.1016/j.lrr.2024.100421","url":null,"abstract":"<div><h3>Introduction</h3><p>Inflammatory bowel disease (IBD) is characterized by chronic inflammation within the digestive tract. In myelodysplastic syndrome (MDS) and aplastic anemia (AA), inflammation in the bone marrow is thought to be one of the causes. There are case reports of MDS or AA combined with IBD. Among these patients, there were several cases who improved hematopoietic function and IBD symptoms after treatment for MDS or AA. However, there are no summarized reports of cases of IBD combined with MDS or AA in Japan. We retrospectively reviewed the reports of IBD combined with MDS or AA in Japan.</p></div><div><h3>Methods</h3><p>We collected reports on cases of IBD combined with MDS or AA in Japan using Igaku Chuo Zasshi [Ichushi] and PubMed, and reviewed those cases.</p></div><div><h3>Results</h3><p>We collected 45 cases of IBD combined with MDS or AA. There were 28 and 19 cases of IBD combined with MDS and AA, respectively. Two cases progressed from AA to MDS. Seven cases occurred in the same period. In 21 cases, MDS or AA occurred prior to IBD. In 5 cases, hematopoietic stem cell transplantation restored both IBD and hematopoietic function.</p></div><div><h3>Conclusions</h3><p>We performed the first literature review of combined cases of IBD and MDS or AA in Japan. Considering the timing of onset and treatment response, there may be common pathologies in IBD and MDS or AA. However, we could not collect treatment details. We plan to construct a nationwide database of complicated cases and elucidate the pathophysiology of these cases.</p></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"21 ","pages":"Article 100421"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213048924000116/pdfft?md5=eed8f5705846a42368e187499e377e4d&pid=1-s2.0-S2213048924000116-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Wakamatsu , H. Muramatsu , H. Sato , M. Ishikawa , D. Nakajima , R. Konno , Y. Kawashima , M. Hamada , Y. Okuno , O. Ohara , Y. Takahashi
{"title":"DIAGNOSTIC UTILITY OF ALDEHYDE DEGRADATION DEFICIENCY SYNDROME USING PROTEOMIC ANALYSIS","authors":"M. Wakamatsu , H. Muramatsu , H. Sato , M. Ishikawa , D. Nakajima , R. Konno , Y. Kawashima , M. Hamada , Y. Okuno , O. Ohara , Y. Takahashi","doi":"10.1016/j.lrr.2024.100418","DOIUrl":"https://doi.org/10.1016/j.lrr.2024.100418","url":null,"abstract":"<div><h3>Introduction</h3><p>Inherited bone marrow failure syndrome (IBMFS) is characterized by a heterogeneous group of disorders with marked cytopenia in one hematopoietic cell lineage. Aldehyde Degradation Deficiency Syndrome (ADDS) is one of the newly discovered IBMFS, caused by a combined deficiency of ADH5 and ALDH2, which are important for the degradation of endogenously produced formaldehyde. Here, we utilized recent technological advances in data-independent proteomic analysis to establish a diagnostic testing for IBMFS, including ADDS.</p></div><div><h3>Methods</h3><p>We performed a multi-omics analysis of in-depth proteomic analysis, targeted capture DNA sequencing, and RNA sequencing among patients with IBMFS.</p></div><div><h3>Results</h3><p>In-depth non-targeted proteomic analysis was performed on 74 samples obtained from 60 patients with IBMFS and 14 healthy controls. We identified eight independent proteomic clusters (C1-C8), with ribosome pathway-related proteins specifically downregulated in C1 and C2, enriched for Diamond-Blackfan anemia and Schwachman-Diamond syndrome, respectively. In the 74 samples, four patients with ADDS showed significantly reduced ADH5 protein expression, whereas the remaining samples showed normal expression. To provide a large-scale rapid screening system in a practical clinical setting, targeted proteomic analysis was performed using a small panel, including ADH5 proteins, in a developmental cohort of 417 samples with hematological malignancies and healthy controls. ADH5 protein expression levels were significantly reduced in ADDS, and its sensitivity and specificity values were 100.0% and 97.5%, respectively.</p></div><div><h3>Conclusions</h3><p>We have performed the first integrated multi-omics analysis for IBMFS, and demonstrated that clinical applications of targeted proteomic assays would be useful in diagnosing for IBMFS, including ADDS.</p></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"21 ","pages":"Article 100418"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213048924000086/pdfft?md5=210b5b55130fba593fda02ab4629f2e9&pid=1-s2.0-S2213048924000086-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103419","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E. Diral, G. Bergonzi, S. Mastaglio, C. Tresoldi, P. Ronchi, M. Ponzoni, M. Cristante, D. Clerici, L. Vago, M. Bernardi, F. Ciceri
{"title":"MOLECULAR INTERNATIONAL PROGNOSTIC SCORING SYSTEM FOR MYELODYSPLASTIC SYNDROMES: A MONOCENTRIC EXPERIENCE","authors":"E. Diral, G. Bergonzi, S. Mastaglio, C. Tresoldi, P. Ronchi, M. Ponzoni, M. Cristante, D. Clerici, L. Vago, M. Bernardi, F. Ciceri","doi":"10.1016/j.lrr.2024.100431","DOIUrl":"https://doi.org/10.1016/j.lrr.2024.100431","url":null,"abstract":"<div><h3>Introduction</h3><p>Allogeneic HSCT (aHSCT) is the only curative treatment, reserved for IPSS-R higher risk (HR, > 3.5) MDS. Molecular data have been integrated within the recently validated IPSS-Mol score system, in order to better predict clinical outcome. However, IPSS-Mol is not still used to guide clinical decisions. We aim to investigate IPSS-Mol significance in a cohort of MDS patients transplanted at our center.</p></div><div><h3>Methods</h3><p>We retrospectively analyzed a cohort of 74 MDS patients undergoing aHSCT between 2010-2022 at our center according to IPSS-R risk score. All patients received treosulfan-based conditioning regimens and PBSC as stem cell source from matched related/unrelated or haploidentical donors. NGS testing for somatic myeloid mutations was performed retrospectively on cryopreserved marrow cells at diagnosis and MDS risk score was then re-calculated according to IPSS-Mol.</p></div><div><h3>Results</h3><p>27 patients (36%) were lower risk (LR) at diagnosis and underwent aHSCT for disease progression. All the other patients were HR (IPSS-R > 3.5) and received aHSCT as upfront or consolidation treatment. At least one oncogenic mutation was found in 86.5% of cases by NGS testing. With IPSS-Mol 10 LR patients (37%) were re-stratified as HR (of note, 6/12 patients with intermediate IPSS-R ≤ 3.5), while 7 HR patients (15%) were re-stratified as LR.</p></div><div><h3>Conclusions</h3><p>aHSCT remains the only curative strategy in HR MDS. NGS testing and application of IPSS-Mol allow to better prognosticate MDS, mostly in patients with LR MDS and specifically in intermediate risk (≤3.5) group. This could help in guiding treatment and specifically optimizing the use of aHSCT in MDS.</p></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"21 ","pages":"Article 100431"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213048924000219/pdfft?md5=1c5b2853f0f0334709793e7375ff3b13&pid=1-s2.0-S2213048924000219-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}