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chronic neutrophilic leukemia

Prithviraj Bose, Lucia Masarova, Srdan Verstovsek
Although the Janus kinase (JAK) inhibitor ruxolitinib has long been the only drug licensed for treatment of the classic Philadelphia chromosome negative (Ph- ) myeloproliferative neoplasms, years of drug development efforts have begun to bear fruit with the recent approval of a novel monopegylated interferon alfa-2b, ropeginterferon alfa, for patients with polycythemia vera without symptomatic splenomegaly in Europe. Several newer JAK inhibitors (fedratinib, pacritinib, momelotinib) have shown activity in phase 3 trials in patients with myelofibrosis but have, for various reasons, not yet received regulatory approval; all these agents, however, remain in active clinical development...
2019: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Seitaro Terakura, Tetsuya Nishida, Masashi Sawa, Tomonori Kato, Kotaro Miyao, Yukiyasu Ozawa, Akio Kohno, Yasushi Onishi, Noriko Fukuhara, Masanobu Kasai, Nobuharu Fujii, Hisayuki Yokoyama, Hiroatsu Iida, Nobuhiro Kanemura, Atsushi Fujieda, Hiroatsu Ago, Yutaka Tsutsumi, Fumihiko Nakamura, Kazuhiro Yago, Yukiyoshi Moriuchi, Shuichi Ota, Haruhiko Ohashi, Atsumi Yanagisawa, Ritsuro Suzuki, Yachiyo Kuwatsuka, Yoshiko Atsuta, Koichi Miyamura, Makoto Murata
Almost comparable transplant outcomes were reported between HLA-matched unrelated donor transplantation (UDT) and cord blood transplantation (CBT). Herein, we conducted a prospective phase 2 study to assess efficacy and safety of single-unit myeloablative CBT in adult leukemia and myelodysplastic syndrome. Because day 180 survival of UDT was approximately 80%, we determined the alternative hypothesis of expected day 180 survival with successful engraftment rate as 80% and set the null hypothesis of threshold rate as 65%...
September 20, 2019: Biology of Blood and Marrow Transplantation
Hiroko Iizuka, Yasutaka Fukuda, Yosuke Mori, Noriaki Iwao, Michiaki Koike, Masaaki Noguchi, Norio Komatsu
A 72-year-old woman with chronic myeloid leukemia (CML) and cirrhosis complicated with blood blisters on her right upper arm and ascites was admitted. She presented with shock vital on admission. Initial gram staining of blood cultures showed gram-positive cocci in chains, suggesting streptococcal toxic shock syndrome (STSS). Amputation of the right upper arm was performed owing to necrotizing fasciitis. Despite continued antibiotic therapy and systemic management, the blood blisters rapidly spread to the skin of the whole body, and she died 41 h after admission...
2019: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Jia-Rong Ren, Xiao-Yu Zhu, Bao-Lin Tang, Xiang Wan, Juan Tong, Lei Zhang, Xu-Han Zhang, Kai-Di Song, Wen Yao, Guang-Yu Sun, Hui-Lan Liu, Zi-Min Sun
OBJECTIVE: To analyze the clinical outcomes of engraftment, graft-versus-host disease (GVHD) and survival in the patients with AML1-ETO positive acute myeloid leukemia (AML) treated with unrelated umbilical cord blood transplantation (UCBT). METHODS: Forty-Five patients with high-risk refractory AML1-ETO positive AML were treated with a single UCBT in a single center from July 2010 to April 2018. All the patients underwent a myeloablative preconditioning regimen,and cyclosporine A (CSA) combined with mycophenolate mofetil (MMF) was used to prevent GVHD...
August 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
Amira Marouf, Nicolas Chapuis, Anne Sophie Alary, Barbara Burroni, Olivier Kosmider, Marco Alifano, Didier Bouscary
Paraneoplastic leukemoid reaction is a challenging differential diagnosis when it presents at the time of diagnosis of cancer. Severe hyperleukocytosis with elevation of blood neutrophils and monocytes counts can evoke myeloid hematologic malignancies. We report the case of a patient who presented with blood and bone marrow features highly suggestive of chronic myelomonocytic leukemia. The diagnosis of primary lung sarcomatoid carcinoma was performed. Surgical removal of this tumor which will always remain the priority led to full normalization of blood cell count...
August 10, 2019: Annals of Thoracic Surgery
Sang Mee Hwang, Sung-Min Kim, Youngwon Nam, Jinhyun Kim, Sungsik Kim, Yong-Oon Ahn, Yong Park, Sung-Soo Yoon, Sue Shin, Sunghoon Kwon, Dong Soon Lee
Chronic myelomonocytic leukemia (CMML) typically shows monocytosis in the peripheral blood (PB), which must be differentiated from reactive monocytosis. To determine the clonality of CMML, we performed molecular and cytogenetic analysis in Korean patients. To investigate whether monocytes in the PB harbored clonal mutational changes, we performed single-cell sequencing after selecting monocytes, neutrophils, and lymphocytes by morphology-aided laser microdissection. Targeted sequencing was performed in 35 patients with CMML with 41 bone marrow samples...
September 2019: Leukemia Research
Haijiao Zhang, Beth Wilmot, Daniel Bottomly, Kim-Hien T Dao, Emily Stevens, Christopher A Eide, Vishesh Khanna, Angela Rofelty, Samantha Savage, Anna Reister Schultz, Nicola Long, Libbey White, Amy Carlos, Rachel Henson, Chenwei Lin, Robert Searles, Robert H Collins, Daniel J DeAngelo, Michael W Deininger, Tamara Dunn, Than Hein, Marlise R Luskin, Bruno C Medeiros, Stephen T Oh, Daniel A Pollyea, David P Steensma, Richard M Stone, Brian J Druker, Shannon K McWeeney, Julia E Maxson, Jason R Gotlib, Jeffrey W Tyner
Chronic neutrophilic leukemia (CNL), atypical chronic myeloid leukemia (aCML), and myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U) are a group of rare and heterogeneous myeloid disorders. There is strong morphologic resemblance among these distinct diagnostic entities as well as a lack of specific molecular markers and limited understanding of disease pathogenesis, which has made diagnosis challenging in certain cases. The treatment has remained empirical, resulting in dismal outcomes...
September 12, 2019: Blood
J F Yao, G X Zhang, Y Y Shen, R L Zhang, Y He, J L Wei, E L Jiang, D L Yang, S Z Feng, M Z Han
Objective: To evaluate the outcomes of human leukocyte antigen (HLA) matched unrelated donor hematopoietic stem cell transplantation (MUD-HSCT) for adult acute myeloid leukemia (AML) in a single center. Methods: Consecutive adult AML who received MUD-HSCT in our center from January 2008 to April 2017 were studied retrospectively, comparing with patients undergoing matched sibling donor (MSD) -HSCT in the same period. The rates of overall survival (OS) , disease free survival (DFS) , relapse, non-relapse mortality (NRM) , engraftment, acute and chronic graft-versus-host disease (aGVHD and cGVHD) were analyzed...
June 14, 2019: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Michel Assane Ndour, Djiby Sow, Demba Diedhiou, Fabrice Senghor, Muriel Diembou, Moustapha Diouf, Awa Faye, Ibrahima Mané Diallo, Ahmed Limane Barrage, Léa Marie Kabou, Maimouna Ndour Mbaye, Anna Sarr
Pyoderma gangrenosum (PG) is a rare non-infectious neutrophilic dermatosis often unknowed. It usually presents with inflammatory skin ulcer, very painful, with rapid evolution. It is commonly found in a context of malignancy, inflammatory bowel disease, rheumatic and/or haematological disease. Its diagnosis is very often late after multiple therapeutic failures. We report a case of pyoderma gangrenosum whose diagnosis was not obvious. A patient was admitted to our department for a persistent dermatological lesion and adverse evolution despite debridements and the administration of antibiotics...
2019: Pan African Medical Journal
Soo Han, John Ehrhardt, Savya Shukla, Adel Elkbuli, Yuri E Nikiforov, Seza A Gulec
BACKGROUND Theranostics is a combined diagnostic and treatment approach to individualized patient care. Kostmann syndrome, or severe congenital neutropenia, is an autosomal recessive disease that affects the production of neutrophils. Papillary thyroid carcinoma (PTC) is the most common type of thyroid malignancy associated with gene alterations, including in the mitogen-activated protein kinase (MAPK) signaling pathway gene. Translocation of the ETS variant 6/neurotrophic receptor tyrosine kinase 3 (ETV6/NTRK3) gene has been implicated in radiation-induced and pediatric forms of thyroid carcinoma but has rarely been described in sporadic PTC...
July 16, 2019: American Journal of Case Reports
Mouhab Ayas, Khawar Siddiqui, Abdullah Al-Jefri, Ali Al-Ahmari, Ibrahim Ghemlas, Hawazen Al-Saedi, Awatif Alanazi, Rafat Jafri, Mohamad F Ayas, Amal Al-Seraihi
Allogeneic hematopoietic cell transplantation (HCT) has been shown to restore normal hematopoiesis in patients with Fanconi anemia (FA), with excellent results in matched related donor HCT. Outcomes of alternative donor HCT are less favorable, however. In patients without FA, several reports have documented stable engraftment and/or a low risk of graft-versus-host disease (GVHD) using unmanipulated HLA-mismatched related donors and post-HCT cyclophosphamide (PT-CY) for GVHD prophylaxis. Data on the use of this approach in patients with FA are scarce, and thus we launched a study of HLA-mismatched related donor HCT in these patient...
July 12, 2019: Biology of Blood and Marrow Transplantation
Mengchang Xu, Yin Ye, Zhi'an Ye, Song'en Xu, Wei Liu, Jin Xu, Yiyue Zhang, Qifa Liu, Zhibin Huang, Wenqing Zhang
Chronic myeloid leukemia is induced by the BCR/ABL1 oncogene, which encodes a protein tyrosine kinase. We examined the effect of direct overexpression of the human p210 BCR/ABL1 oncoprotein in zebrafish. Humanized p210 BCR/ABL1 protein was detectable in Tg(hsp70:p210BCR/ABL1 ) transgenic zebrafish embryos and adult kidney marrow. Transgenic zebrafish developed chronic myeloid leukemia, which could be induced via cells into recipients. The expression of human BCR/ABL1 promoted myeloid lineages in Tg(hsp70:p210BCR/ABL1 ) transgenic embryos...
July 9, 2019: Haematologica
Bang-Shuo Zhang, Yong-Ping Chen, Jing-Long Lv, Yi Yang
OBJECTIVE: To compare the efficacy of nilotinib and imatinib in the treatment of chronic myeloid leukemia (CML). STUDY DESIGN: Analytical study. PLACE AND DURATION OF STUDY: Department of Hematology, Chongqing Three Gorges Central Hospital, China, from January 2016 to January 2018. METHODOLOGY: Eighty patients with CML were randomly divided into the nilotinib group (treated with nilotinib) and the imatinib group (treated with imatinib), 40 patients in each group...
July 2019: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
А А Аkleyev
A retrospective analysis of the peripheral blood cell composition (PBCC) has been performed among 43 chronically-exposed Techa riverside residents later diagnosed with chronic myeloid leukemia (CML) or acute leukemias (AL). Prior to CML development, a significant increase in peripheral blood neutrophil count has been noted in exposed individuals for many years. Neutrophil count grew most significantly after reduction of exposure dose rate to red bone marrow (RBM) to ≤0.1 Gy, which was typical both for CML and AL...
December 1, 2018: Radiation Protection Dosimetry
Lan Luo, Shu Fang, Shasha Zhao, Fei Li, Yu Zhou, Lixun Guan, Nan Yang, Zhenyang Gu, Tao Lin, Feiyan Wang, Chengying Zhu, Wenrong Huang, Daihong Liu, Chunji Gao
BACKGROUND Data about application of related haploidentical allogeneic hematopoietic stem cell transplantation (haplo-HSCT) on patients with high-risk or intermediate-risk acute myeloid leukemia (AML) in the first complete remission (CR1) are lacking. In this study, we report the outcomes of using unmanipulated haploidentical allogeneic peripheral blood stem cell transplantation (haplo-PBSCT) as post-remission therapy for patients with high-risk or intermediate-risk AML in CR1. MATERIAL AND METHODS From January 2008 to July 2016, 33 patients diagnosed as high-risk or intermediate-risk AML in CR1 undergoing haplo-PBSCT in our institution were enrolled for analysis...
June 21, 2019: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Chutima Kunacheewa, Pakaporn Thongthang, Patompong Ungprasert, Eakkapol Utchariyaprasit, Weerapat Owattanapanich
OBJECTIVES: The addition of lenalidomide (LEN) to azacitidine (AZA) may further improve the outcomes of acute myeloid leukemia (AML) patients as well as patients with high-risk myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia (CMML) patients although the evidence for this combination treatment is still relatively limited. This meta-analysis aimed to evaluate efficacy and adverse effects of AZA plus LEN for the treatment of patients with high-risk MDS, AML or CMML. METHODS: The current study systematically identified all cohort studies of patients with AML and/or MDS and/or CMML who received AZA in combination with LEN that reported the overall complete remission (CR) rate and/or overall response rate (ORR)...
December 2019: Hematology (Amsterdam, Netherlands)
Alan Y Hsu, Sheng Liu, Ramizah Syahirah, Kent A Brasseale, Jun Wan, Qing Deng
Neutrophil migration is essential for battling against infections but also drives chronic inflammation. Since primary neutrophils are terminally differentiated and not genetically tractable, leukemia cells such as HL-60 are differentiated into neutrophil-like cells to study mechanisms underlying neutrophil migration. However, constitutive overexpression or inhibition in this cell line does not allow the characterization of the genes that affect the differentiation process. Here we apply the tet-on system to induce the expression of a zebrafish microRNA, dre-miR-722, in differentiated HL-60...
June 5, 2019: Molecular Immunology
Damien Blez, Marion Blaize, Carole Soussain, Alexandre Boissonnas, Aïda Meghraoui-Kheddar, Natacha Menezes, Anaïs Portalier, Christophe Combadière, Véronique Leblond, David Ghez, Arnaud Fekkar
Bruton's tyrosine kinase inhibitor ibrutinib has become a leading therapy against chronic lymphoid leukemia. Recently, ibrutinib has been associated with the occurrence of invasive fungal infections, in particular invasive aspergillosis. The mechanisms underlying the increased susceptibility to fungal infections associated with ibrutinib exposure are currently unknown. Innate immunity, in particular polymorphonuclear neutrophils, represents the cornerstone of anti-Aspergillus immunity however the potential impact of ibrutinib on neutrophils has been little studied...
June 6, 2019: Haematologica
Balan Louis Gaspar, Prashant Sharma, Neelam Varma, Dmitry Sukhachev, Ishwar Bihana, Shano Naseem, Pankaj Malhotra, Subhash Varma
BACKGROUND: Modern automated hematology analyzers provide quantitative data on leukocyte size and structure that may be useful to distinguish reactive from neoplastic cellular proliferations. We compared leukocyte volume, conductivity and scatter (VCS) characteristics of chronic myeloid leukemia (CML), bcr-abl1-positive patients with those of non-neoplastic neutrophilia. MATERIALS AND METHODS: Complete blood counts and VCS data (LH750 hematology analyzers, Beckman Coulter) from 38 newly-diagnosed CML patients, 65 CML on imatinib mesylate therapy, 58 patients with elevated age-specific neutrophil counts due to varied causes, 100 pregnant women and 99 healthy controls were collated and compared...
April 2019: Biomedical Journal
J Zhu, B L Tang, K D Song, X H Zhang, X Y Zhu, W Yao, X Wan, H L Liu, Z M Sun
Objective: To compare the clinical efficacy of umbilical cord blood transplantation (UCBT) and hematopoietic stem cell transplantation from HLA-matched sibling donors (MSD-HSCT) in the treatment of myelodysplastic syndrome-EB (MDS-EB) or acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) . Methods: A cohort of 64 patients (including 38 cases of MDS-EB and 26 cases of AML-MRC) who received UCBT/MSD-HSCT from February 2011 to December 2017 were retrospectively analyzed. Results: ①Compared with MSD-HSCT group, UCBT group had a higher proportion of AML-MRC patients [52...
April 14, 2019: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
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