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REVIEW
Natasha Szuber, Attilio Orazi, Ayalew Tefferi
Chronic neutrophilic leukemia (CNL) is a rare BCR::ABL1-negative myeloproliferative neoplasm (MPN) defined by persistent mature neutrophilic leukocytosis and bone marrow granulocyte hyperplasia. Atypical chronic myeloid leukemia (aCML) (myelodysplastic "[MDS]/MPN with neutrophilia" per World Health Organization [WHO]) is a MDS/MPN overlap disorder featuring dysplastic neutrophilia and circulating myeloid precursors. Both manifest with frequent hepatosplenomegaly and less commonly, bleeding, with high rates of leukemic transformation and death...
April 21, 2024: American Journal of Hematology
#2
JOURNAL ARTICLE
Seon Young Kim, Ik-Chan Song, Jimyung Kim, Gye Cheol Kwon
We report a series of patients with CSF3R-mutant (CSF3Rmut ) atypical chronic myeloid leukemia (aCML), chronic neutrophilic leukemia (CNL) or other hematologic malignancies. We included 25 patients: 5 aCML and 4 CNL CSF3Rmut patients; 1 aCML, 2 CNL, and 2 myelodysplastic/myeloproliferative neoplasm, not otherwise specified patients without CSF3R mutation; and 11 CSF3Rmut patients with other diseases [8 acute myeloid leukemia (AML), 1 chronic myelomonocytic leukemia (CMML), 1 myelodysplastic syndrome (MDS), and 1 acute lymphoblastic leukemia (ALL)]...
April 18, 2024: Annals of Diagnostic Pathology
#3
Hongxia Chen, Ning Wang, Yin Li, Xiaohong Xie, Yi Yang
Atypical Chronic Myeloid Leukemia (aCML), a myeloproliferative neoplasm with poor prognosis, was reclassified as aCML by the ICC classification, and as MDS/MPN with neutrophilia by the WHO 2022 classification. Due to the heterogeneity of its clinical features and the lack of unique biomarkers, as well as limited treatment options, aCML currently lacks a standardized treatment protocol. In this case report, we reviewed a young man diagnosed with aCML who achieved complete clinical and hematologic remission subsequent to receiving a therapeutic regimen combining Venetoclax and Azacitidine...
2024: Frontiers in Oncology
#4
JOURNAL ARTICLE
Meenu Kesarwani, Zachary Kincaid, Mohammad Azhar, Mohammad Azam
Elevated MAPK and the JAK-STAT signaling play pivotal roles in the pathogenesis of chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML). While inhibitors targeting these pathways effectively suppress the diseases, they fall short in providing enduring remission, largely attributed to cytostatic nature of these drugs. Even combinations of these drugs are ineffective in achieving sustained remission. Enhanced MAPK signaling besides promoting proliferation and survival triggers a pro-apoptotic response...
March 26, 2024: Blood Advances
#5
Lu Liu, Xiaofeng Song, Wenhao Dong, Zhao Li, Dongmei Guo
BACKGROUND: Atypical chronic myeloid leukemia (aCML) is a BCR::ABL1 negative myelodysplastic/myeloproliferative neoplasm with poor overall survival. Some patients can be treated by allogeneic hematopoietic stem cell transplantation (allo-HSCT) from suitable donors. The effectiveness of decitabine or azacitidine (AZA) has recently been reported; however, their combined efficacy with selinexor has not yet been reported. CASE DESCRIPTION: In this study, we report the case of a patient with aCML who was successfully treated with selinexor combined with AZA...
2024: Frontiers in Oncology
#6
JOURNAL ARTICLE
Massimo Breccia
Atypical chronic myeloid leukemia (aCML) is included in the group of myelodysplastic/myeloproliferative neoplasms by the International Consensus Classification and has been renamed as MDS/MPN with neutrophilia by the fifth edition of World Health Organization classification. It is always characterized by morphologic identification of granulocytic dysplasia with >10% circulating immature myeloid cells, 2 distinguished features that differentiate this disease among the others. Somatic mutations may help to diagnose but are not specifically pathognomonic of the disease, with the most detected including ASXL1, SETBP1, NRAS, KRAS, SRSF2, and TET2 and with low-frequency CBL, CSF3R, JAK2, and ETNK1...
December 8, 2023: Hematology—the Education Program of the American Society of Hematology
#7
JOURNAL ARTICLE
Saskia K Klein, Gerwin A Huls, Otto Visser, Hanneke C Kluin-Nelemans, Avinash G Dinmohamed
MDS/ MPN with neutrophilia, until recently called atypical chronic myeloid leukemia (aCML), being part of the myelodysplastic/myeloproliferative neoplasms is a very rare disease with poor prognosis. Although emerging data reveal its cytogenetic and molecular profile, integrated survival and treatment data remain scarce. We analyzed a cohort of 347 adult patients diagnosed with MDS/ MPN with neutrophilia, registered in the Netherlands Cancer Registry between 2001 and 2019. Our demographic baseline data align with other cohorts...
November 7, 2023: Blood Advances
#8
REVIEW
Margo B Gerke, Ilias Christodoulou, Theodoros Karantanos
Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are hematological disorders characterized by both proliferative and dysplastic features. According to the 2022 International Consensus Classification (ICC), MDS/MPN consists of clonal monocytosis of undetermined significance (CMUS), chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia (aCML), MDS/MPN with SF3B1 mutation (MDS/MPN-T-SF3B1), MDS/MPN with ring sideroblasts and thrombocytosis not otherwise specified (MDS/MPN-RS-T-NOS), and MDS/MPN-NOS...
July 27, 2023: Cancers
#9
REVIEW
Giovanni Iaquinta, Emilia Scalzulli, Silvia Angeloni, Ida Carmosino, Alessandro Costa, Claudia Ielo, Mauro Passucci, Chiara Masucci, Maurizio Martelli, Paola Grammatico, Massimo Breccia
Atypical chronic myeloid leukemia (aCML) is a rare MDS/MPN disease characterized by the absence of BCR::ABL1 rearrangement and well known typical mutations associated with myeloproliferative disorders. Mutational landscape associated with this disease was recently described with frequent involvement of SETBP1 and ETNK1 mutations. CCND2 mutations have not been frequently detected in MPN or MDS/MPN patients. We describe two cases of aCML with two CCND2 mutations in 280 and 281 codons which rapidly develop progressive characteristics, and we reviewed the literature about this unfavorable association, suggesting a role as a new possible marker of aggressive disease...
October 2023: Leukemia & Lymphoma
#10
REVIEW
Diletta Fontana, Elena M Elli, Fabio Pagni, Rocco Piazza
The myelodysplastic syndromes/myeloproliferative neoplasms (MDS/MPN) category comprises a varied group of myeloid neoplastic diseases characterized by clinical and pathologic overlapping features of both myelodysplastic and myeloproliferative neoplasms. For these reasons, these tumors are challenging in terms of diagnosis. The recent World Health Organization (WHO) 2022 classification and the International Consensus Classification (ICC) made changes in the classification of MDS/MPN compared to the previous 2016 WHO classification and improved the diagnostic criteria of these entities...
June 13, 2023: Cancers
#11
Lyndsey Sandow, Michael Heinrich
BACKGROUND: Atypical chronic myeloid leukemia (aCML) is a rare myelodysplastic/myeloproliferative neoplasm. There is no proven standard of care treatment and the only curative option available is hematopoietic stem cell transplant. In addition to traditional chemotherapy, targeted therapy has shown to be a promising. Avapritinib is a selective type 1 tyrosine kinase inhibitor with high potency for KIT D816V and was recently approved for treatment of systemic mastocytosis. Here we present a case of aCML with novel D816V mutation treated with avapritinib for 17 months leading to clonal extinction of the driver mutation...
2023: Leukemia Research Reports
#12
JOURNAL ARTICLE
Douglas Tremblay, Dahiel Sastow, John O Mascarenhas
No abstract text is available yet for this article.
June 8, 2023: Blood Advances
#13
EDITORIAL
Argiris Symeonidis, Ulrich Germing
No abstract text is available yet for this article.
2023: Frontiers in Oncology
#14
Somprakash Dhangar, Chandrakala Shanmukhaiah, Leena Sawant, Jagdeeshwar Ghatanatti, Aditi Shah, Leo Prince Mathan S, Babu Rao Vundinti
The sole t(8;22)(p11.2;q11.2)/BCR- FGFR1 chromosomal abnormality formerly known as aCML is an extremely rare disease entity with a history of rapid progression. Though patients resemble phenotypically chronic myeloid leukemia, the treatment of patients with sole BCR-FGFR1 rearrangement are still challenging for clinicians due to rapid progressive nature and unavailability of uniform treatment guidelines. In present case study, we describe a case of myeloid neoplasm with sole chromosomal abnormality of t(8;22)(p11...
June 2023: Cancer Genetics
#15
JOURNAL ARTICLE
X Chen, Z Y Liu, R L Zhang, W H Zhai, Q L Ma, A M Pang, D L Yang, Y He, J L Wei, S Z Feng, M Z Han, E L Jiang
Objegtive: To investigate the efficacy and safety of preemptive/salvage therapy with venetoclax (VEN) in patients with recurrence after allogeneic hematopoietic stem cell transplantation (allo-HSCT) . Methods: Retrospective analysis the clinical data of 25 patients with minimal residual disease (MRD) positive or morphological recurrence after allo-HSCT treated with VEN in the hematological Hospital of Chinese Academy of Medical Sciences from 2021.2 to 2021.11, there were 15 MRD positive patients (preemptive treatment group) and 10 morphological recurrence patients (salvage treatment group) ...
July 14, 2022: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
#16
Dorela Lame, Michelangelo Pianelli, Erika Morsia, Alberto Carturan, Gaia Goteri, Stefania Mancini, Attilio Olivieri, Antonella Poloni
The presence of neutrophilic leukocytosis may underlie a wide variety of diseases. Some rare causes of neutrophilia might be chronic neutrophilic leukemia (CNL) and myelodysplastic/myeloproliferative neoplasm with neutrophilia (MDS with neutrophilia). Here we report a case of a 78-year-old woman who came to our ER due to severe leukocytosis and anemia on a routine check-up. The patient was asymptomatic and the last exams available showed a mild leukopenia and thrombocytopenia. The abdominal echography showed mild splenomegaly The patient underwent bone marrow (BM) examinations...
2023: Leukemia Research Reports
#17
JOURNAL ARTICLE
Mrinal M Patnaik, Ayalew Tefferi
DISEASE OVERVIEW: Atypical chronic myeloid leukemia (aCML) and myelodysplastic/myeloproliferative (MDS/MPN) neoplasms, not otherwise specified (NOS), are MDS/MPN overlap neoplasms characterized by leukocytosis, in the absence of monocytosis and eosinophilia, with <20% blasts in the blood and bone marrow. DIAGNOSIS: aCML, previously known as aCML, BCR::ABL1 negative, was renamed as aCML by the ICC classification, and as MDS/MPN with neutrophilia by the 5th edition of the WHO classification...
April 2023: American Journal of Hematology
#18
Giovanni Martino, Martina Quintini, Maria Paola Martelli, Stefano Ascani, Stefano Lazzi, Cristina Mecucci
A case of myelodysplastic/myeloproliferative neoplasm with neutrophilia (aCML) with SETBP1 and ASXL1 mutations.
July 2023: American Journal of Hematology
#19
MULTICENTER STUDY
Gonzalo Carreño-Tarragona, Alberto Álvarez-Larrán, Claire Harrison, José Carlos Martínez-Ávila, Juan Carlos Hernández-Boluda, Francisca Ferrer-Marín, Deepti H Radia, Elvira Mora, Sebastian Francis, Teresa González-Martínez, Kathryn Goddard, Manuel Pérez-Encinas, Srinivasan Narayanan, José María Raya, Vikram Singh, Xabier Gutiérrez, Peter Toth, Paula Amat-Martínez, Louisa Mcilwaine, Magda Alobaidi, Karan Mayani, Andrew McGregor, Ruth Stuckey, Bethan Psaila, Adrián Segura, Caroline Alvares, Kerri Davidson, Santiago Osorio, Robert Cutting, Caroline P Sweeney, Laura Rufián, Laura Moreno, Isabel Cuenca, Jeffery Smith, María Luz Morales, Rodrigo Gil-Manso, Ioannis Koutsavlis, Lihui Wang, Adam J Mead, María Rozman, Joaquín Martínez-López, Rosa Ayala, Nicholas C P Cross
Chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML) are rare myeloid disorders that are challenging with regard to diagnosis and clinical management. To study the similarities and differences between these disorders, we undertook a multicenter international study of one of the largest case series (CNL, n = 24; aCML, n = 37 cases, respectively), focusing on the clinical and mutational profiles (n = 53 with molecular data) of these diseases. We found no differences in clinical presentations or outcomes of both entities...
May 9, 2023: Blood Advances
#20
JOURNAL ARTICLE
Fernanda Figueroa-Faúndez, Catalina Vidal-Rojas, Vania Briones-Muñoz, Mauricio Chandía-Cabas
BCR-ABL1 negative atypical chronic myeloid leukemia (aCML) is a rare type of myeloproliferative / myelodysplastic syndrome characterized by leukocytosis and proliferation of dysplastic neutrophilic precursors in the absence of positivity for the BCR-ABL1 fusion gene. We report a 66-year-old woman and a 57-year-old man with aCML, who initially presented with general malaise and weight loss, associated with anemia, thrombocytopenia, and leukocytosis with left shift and dysplasia in the neutrophil series. Both evolved unfavorably after admission and died a few days later due to multiple organ failure...
March 2022: Revista Médica de Chile
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