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Atypical chronic myeloid leukemia

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https://www.readbyqxmd.com/read/27899359/how-i-treat-atypical-chronic-myeloid-leukemia
#1
Jason Gotlib
Atypical chronic myeloid leukemia, BCR-ABL1-negative (aCML) is a rare myelodysplastic syndrome/myeloproliferative neoplasm for which no current standard of care exists. The challenges of atypical CML relate to its heterogeneous clinical and genetic features, high rate of transformation to acute myeloid leukemia, and historically poor survival. Therefore, allogeneic hematopoietic stem cell transplantation should always be an initial consideration for eligible patients with a suitable donor. Non-transplant approaches for treating aCML have otherwise largely relied on adopting treatment strategies used for MDS and MPN...
November 29, 2016: Blood
https://www.readbyqxmd.com/read/27807503/clinical-management-of-myelodysplastic-syndrome-myeloproliferative-neoplasm-overlap-syndromes
#2
Joseph A Clara, David A Sallman, Eric Padron
The myelodysplastic/myeloproliferative neoplasms (MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classification, the category includes atypical chronic myeloid leukemia (aCML), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), MDS/MPN-unclassifiable (MDS/MPN-U), and the provisional entity refractory anemia with ring sideroblasts and thrombocytosis (RARS-T). Although diagnosis currently remains based on clinicopathologic features, the incorporation of next-generation platforms has allowed for the recent molecular characterization of these diseases which has revealed unique and complex mutational profiles that support their distinct biology and is anticipated to soon play an integral role in diagnosis, prognostication, and treatment...
September 2016: Cancer Biology & Medicine
https://www.readbyqxmd.com/read/27789332/granulocyte-colony-stimulating-factor-receptor-signaling-in-severe-congenital-neutropenia-chronic-neutrophilic-leukemia-and-related-malignancies
#3
REVIEW
Pankaj Dwivedi, Kenneth D Greis
Granulocyte colony-stimulating factor is a hematopoietic cytokine that stimulates neutrophil production and hematopoietic stem cell mobilization by initiating the dimerization of homodimeric granulocyte colony-stimulating factor receptor. Different mutations of CSF3R have been linked to a unique spectrum of myeloid disorders and related malignancies. Myeloid disorders caused by the CSF3R mutations include severe congenital neutropenia, chronic neutrophilic leukemia, and atypical chronic myeloid leukemia. In this review, we provide an analysis of granulocyte colony-stimulating factor receptor, various mutations, and their roles in the severe congenital neutropenia, chronic neutrophilic leukemia, and malignant transformation, as well as the clinical implications and some perspective on approaches that could expand our knowledge with respect to the normal signaling mechanisms and those associated with mutations in the receptor...
October 24, 2016: Experimental Hematology
https://www.readbyqxmd.com/read/27721761/an-atypical-initial-presentation-of-chronic-myeloid-leukemia-with-central-nervous-system-and-lymph-node-blast-crises
#4
Khadega A Abuelgasim, Saeed Alshieban, Nada A Almubayi, Ayman Alhejazi, Abdulrahman R Jazieh
We describe the case of a young man with therapy-naive chronic myeloid leukemia who did not initially have any peripheral blood or bone marrow excess blasts but presented with extramedullary myeloid blast crises involving the central nervous system and multiple lymph nodes. Conventional cytogenetic tests were positive for t(9;22)(q34:q11) as well as for trisomy 8, 14 and 21 and del(16q). The patient's peripheral blood and bone marrow were positive for the BCR-ABL oncogene when analyzed by fluorescence in situ hybridization and polymerase chain reaction...
May 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27664113/an-exercise-in-extrapolation-clinical-management-of-atypical-cml-mds-mpn-unclassifiable-and-mds-mpn-rs-t
#5
Chetasi Talati, Eric Padron
According to the recently published 2016 World Health Organization (WHO) classification of myeloid malignancies, myelodysplastic/myeloproliferative neoplasms (MDS/MPN) include atypical chronic myeloid leukemia (aCML), MDS/MPN-unclassifiable (MDS/MPN-U), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), and MDS/MPN ring sideroblasts with thrombocytosis (MDS/MPN-RS-T). MDS/MPN-RS-T was previously a provisional category known as refractory anemia with ring sideroblasts with thrombocytosis (RARS-T) which has now attained a distinct designation in the 2016 WHO classification...
September 24, 2016: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/27494969/-not-available
#6
Matthieu Mosca, Gaëlle Vertenoeil, Katte Rao Toppaldoddi, Isabelle Plo, William Vainchenker
BIOLOGICAL ASPECTS OF JAK/STAT SIGNALING IN BCR-ABL-NEGATIVE MYELOPROLIFERATIVE NEOPLASMS: Myeloproliferative disorders more recently named Myeloproliferative neoplasms (MPN) display several clinical entities: chronic myeloid leukemia (CML), the classical MPN including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and atypical and unclassifiable NMP. The term MPN is mostly used for classical BCR-ABL-negative (myeloproliferative disorder) (ET, PV, PMF). These are clonal diseases resulting from the transformation of an hematopoietic stem cell and leading to an abnormal production of myeloid cells...
June 2016: Bulletin du Cancer
https://www.readbyqxmd.com/read/27459623/-transformation-of-secondary-myelodysplastic-syndrome-to-atypical-chronic-myeloid-leukemia-in-a-female-patient-with-acute-myeloid-leukemia
#7
S V Gritsaev, I I Kostroma, I M Zapreeva, A V Shmidt, S A Tiranova, V A Balashova, I S Martynkevich, Zh V Chubukina, N Yu Semenova, A V Chechetkin
Secondary myeloid neoplasia may be a complication of intensive cytostatic therapy. The most common types of secondary neoplasias are acute myeloid leukemia and myelodysplastic syndrome. The development of secondary atypical chronic myeloid leukemia (aCML) is an extremely rare phenomenon. The paper describes transformation of secondary myelodysplastic syndrome to aCML 6 months after its diagnosis. The development of aCML was accompanied by additional chromosomal aberration as monosomy of chromosome 17. No mutations in the JAK2, MPL, and CalR genes were detected...
2016: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/27408365/chronic-myeloid-leukemia-with-extramedullary-blast-crisis-two-unusual-sites-with-review-of-literature
#8
Kamal Kant Sahu, Pankaj Malhotra, Preithy Uthamalingam, Gaurav Prakash, Amanjit Bal, Neelam Varma, Subhash Chandar Varma
Extramedullary blast crisis (EBC) in chronic myeloid leukemia (CML) is a rare phenomenon and represents infiltration of leukemic blasts in areas other than bone marrow. Lymph node is the most common site of involvement by EBC. We herein present a case of CML who suffered from two discrete episodes of EBC at atypical locations (scalp and paravertebral) within an interval duration of nine months. A-38-year-old female was diagnosed as a case of CML with extramedullary blast crisis in scalp at presentation. She received treatment with imatinib 600 mg once daily through Novartis Oncology Access Program (NOA)...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27118564/a-novel-e8a2-bcr-abl1-intronic-fusion-through-insertion-of-a-chromosome-22-bcr-gene-fragment-into-chromosome-9-in-an-atypical-philadelphia-ph-chromosome-chronic-myeloid-leukemia-patient
#9
Lifeng Chen, Yaohui Wu, Yong You, Min Xiao, Ye Yao, Weiming Li
No abstract text is available yet for this article.
April 27, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27004153/chronic-myeloid-leukemia-a-case-of-extreme-thrombocytosis-causing-syncope-and-myocardial-infarction
#10
Rawaa Ebrahem, Brittany Ahmed, Salam Kadhem, Quoc Truong
Chronic myeloid leukemia (CML), a hematologic malignancy characterized by unregulated growth of myelogenous leukocytes, typically presents with symptoms of fatigue, anorexia, and splenomegaly. Laboratory studies often reveal a significant leukocytosis with neutrophilia. A moderate thrombocytosis may be present, but is not usually problematic. The following case discusses a patient who presented with syncope, a convulsive episode, and non ST-segment myocardial infarction secondary to symptomatic thrombocytosis of 2...
2016: Curēus
https://www.readbyqxmd.com/read/26870618/durable-disease-control-with-mek-inhibition-in-a-patient-with-nras-mutated-atypical-chronic-myeloid-leukemia
#11
Vishesh Khanna, Scott T Pierce, Kim-Hien T Dao, Cristina E Tognon, David E Hunt, Brian Junio, Jeffrey W Tyner, Brian J Druker
Atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) are rare hematologic neoplasms characterized by leukocytosis and a hypercellular bone marrow. Although recurrent mutations in the colony-stimulating factor 3 receptor (CSF3R) are frequently observed in patients with (CNL), the mutational landscape in (aCML) is less well-defined. In this report, we describe an 81-year-old male who was diagnosed with aCML. He presented with leukocytosis and anemia but no significant clinical symptoms...
2015: Curēus
https://www.readbyqxmd.com/read/26870268/decitabine-for-the-treatment-of-atypical-chronic-myeloid-leukemia-a-report-of-two-cases
#12
Huifang Jiang, Zhonglin Wu, L I Ren, Diehong Tao, Hongyan Tong
Atypical chronic myeloid leukemia (aCML) is a hematopoietic stem/progenitor cell disorder, predominantly involving neutrophils. At present, a limited number of studies regarding the treatment of aCML have been published, and the therapies that are currently available exhibit unsatisfactory outcomes. In the present study, the cases of two aCML patients treated with decitabine (DCA) therapy who achieved remission are presented. A 48-year-old male, who presented with fatigue and a cough that had lasted two months, and a 69-year-old male who presented with dizziness, fatigue and shortness of breath with exercise, were diagnosed with aCML following bone marrow examination, flow cytometry and chromosome banding analysis...
January 2016: Oncology Letters
https://www.readbyqxmd.com/read/26847385/complete-cytogenetic-response-to-nilotinib-in-a-chronic-myeloid-leukemia-case-with-a-rare-e13a3-b2a3-bcr-abl-fusion-transcript-a-case-report
#13
Bei Liu, Wei Zhang, Haizhen Ma
In the present study, an atypical case of chronic myeloid leukemia (CML) in a 32-year-old male was reported. CML cases with e13a3 breakpoint cluster region (BCR)-ABL transcripts are extremely rare. Reverse transcription quantitative‑polymerase chain reaction (RT-qPCR) was initially negative due to the primer corresponding to ABL a2 sequences and diagnosis was based upon analysis of the bone marrow smear, fluorescence in situ hybridization and karyotype analysis. RT‑qPCR analysis with the ABL primer, which was located in ABL exon 3 to enable the detection of fusions with either ABL a2 or exon a3 demonstrated the presence of the BCR‑ABL fusion transcript e13a3...
March 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/26722598/chronic-myeloid-leukemia-with-variation-of-translocation-at-ph-ins-22-9-q11-q21q34-a-case-report
#14
Zhiqiong Wang, Wen Zen, Fankai Meng, Xing Xin, Li Luo, Hanying Sun, Jianfeng Zhou, Lifang Huang
Chronic myeloid leukemia (CML) is most frequently observed in middle-aged individuals. In most patients, normal marrow cells are replaced by cells with an abnormal G-group chromosome, the Philadelphia (Ph) chromosome. The Ph chromosome that is characterized by the translocation (9;22) (q34;q11) is noted in 90-95% of patients diagnosed with CML. Studies have also shown that CML can be associated with various other cytogenetic abnormalities, with 5-10% of these cases showing complex translocation involving another chromosome in addition to the Ph chromosome...
2015: International Journal of Clinical and Experimental Pathology
https://www.readbyqxmd.com/read/26637732/what-s-different-about-atypical-cml-and-chronic-neutrophilic-leukemia
#15
REVIEW
Kim-Hien T Dao, Jeffrey W Tyner
Atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) are rare myeloid neoplasms defined largely by morphologic criteria. The discovery of CSF3R mutations in aCML and CNL have prompted a more comprehensive genetic profiling of these disorders. These studies have revealed aCML to be a genetically more heterogeneous disease than CNL, however, several groups have reported that SETBP1 and ASXL1 mutations occur at a high frequency and carry prognostic value in both diseases. We also report a novel finding-our study reveals a high frequency of U2AF1 mutations at codon Q157 associated with CSF3R mutant myeloid neoplasms...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26486739/clinical-validation-of-a-multipurpose-assay-for-detection-and-genotyping-of-calr-mutations-in-myeloproliferative-neoplasms
#16
Meenakshi Mehrotra, Rajyalakshmi Luthra, Rajesh R Singh, Bedia A Barkoh, John Galbincea, Pramod Mehta, Rashmi S Goswami, Kausar J Jabbar, Sanam Loghavi, L Jeffrey Medeiros, Srdan Verstovsek, Keyur P Patel
OBJECTIVES: To develop a polymerase chain reaction (PCR)-based approach to detect CALR mutations in myeloproliferative neoplasms (MPNs) in a clinical laboratory. METHODS: DNA was extracted from bone marrow aspirate samples of 67 JAK2 wild-type MPNs (22 with matched peripheral blood), 54 cases of unclassifiable myelodysplastic syndrome/MPN, and 16 cases of atypical chronic myeloid leukemia. We used genomic DNA to detect somatic mutations in exon 9 of CALR and PCR with fluorescently labeled and M13-tagged primers and subjected the products to capillary electrophoresis (CE) followed by Sanger sequencing...
November 2015: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/26475333/the-colony-stimulating-factor-3-receptor-t640n-mutation-is-oncogenic-sensitive-to-jak-inhibition-and-mimics-t618i
#17
Julia E Maxson, Samuel B Luty, Jason D MacManiman, Jason C Paik, Jason Gotlib, Peter Greenberg, Swaleh Bahamadi, Samantha L Savage, Melissa L Abel, Christopher A Eide, Marc M Loriaux, Emily A Stevens, Jeffrey W Tyner
PURPOSE: Colony-stimulating factor 3 receptor (CSF3R) mutations have been identified in the majority of chronic neutrophilic leukemia (CNL) and a smaller percentage of atypical chronic myeloid leukemia (aCML) cases. Although CSF3R point mutations (e.g., T618I) are emerging as key players in CNL/aCML, the significance of rarer CSF3R mutations is unknown. In this study, we assess the importance of the CSF3R T640N mutation as a marker of CNL/aCML and potential therapeutic target. EXPERIMENTAL DESIGN: Sanger sequencing of leukemia samples was performed to identify CSF3R mutations in CNL and aCML...
February 1, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/26378157/activity-of-single-agent-decitabine-in-atypical-chronic-myeloid-leukemia
#18
Heidi Hausmann, Vijaya R Bhatt, Ji Yuan, Lori J Maness, Apar K Ganti
Atypical chronic myeloid leukemia is a rare entity that presents diagnostic and therapeutic challenges. Traditionally utilized therapeutic agents such as hydroxyurea or interferon result in a median survival of approximately two years, thus warranting identification of better options. We report a 49-year-old Caucasian female, who presented with extreme leukocytosis (white blood cells of 148,300/µL) with left shift, severe anemia, and thrombocytopenia. Following a diagnosis of atypical chronic myeloid leukemia, she was started on intravenous decitabine...
September 15, 2015: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/26341525/an-international-mds-mpn-working-group-s-perspective-and-recommendations-on-molecular-pathogenesis-diagnosis-and-clinical-characterization-of-myelodysplastic-myeloproliferative-neoplasms
#19
MULTICENTER STUDY
Tariq I Mughal, Nicholas C P Cross, Eric Padron, Ramon V Tiu, Michael Savona, Luca Malcovati, Raoul Tibes, Rami S Komrokji, Jean-Jacques Kiladjian, Guillermo Garcia-Manero, Attilio Orazi, Ruben Mesa, Jaroslaw P Maciejewski, Pierre Fenaux, Raphael Itzykson, Ghulam Mufti, Eric Solary, Alan F List
In the 2008 WHO classification, chronic myeloid malignancies that share both myelodysplastic and myeloproliferative features define the myelodysplastic/myeloproliferative group, which includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ring sideroblasts and thrombocytosis, and myelodysplastic/myeloproliferative unclassified. With the notable exception of refractory anemia with ring sideroblasts and thrombocytosis, there is much overlap among the various subtypes at the molecular and clinical levels, and a better definition of these entities, an understanding of their biology and an identification of subtype-specific molecular or cellular markers are needed...
September 2015: Haematologica
https://www.readbyqxmd.com/read/26274939/atypical-chronic-myeloid-leukemia-in-two-pediatric-patients
#20
Jason L Freedman, Ami V Desai, L Charles Bailey, Richard Aplenc, Bettina Burnworth, Barbara K Zehentner, David T Teachey, Gerald Wertheim
Atypical chronic myeloid leukemia, BCR-ABL1-negative, (aCML) is a rare myeloid neoplasm. Recent adult data suggest the leukemic cells in a subset of patients are dependent on JAK/STAT signaling and harbor CSF3R-activating mutations. We hypothesized that, similar to adult patients, the presence of CSF3R-activating mutations would be clinically relevant in pediatric myeloid neoplasms as patients would be sensitive to the JAK inhibitor, ruxolitinib. We report two cases of morphologically similar pediatric aCML, BCR-ABL1-negative based on WHO 2008 criteria...
January 2016: Pediatric Blood & Cancer
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