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https://www.readbyqxmd.com/read/28927137/atypical-chronic-myeloid-leukemia-with-isochromosome-x-p10-a-case-report
#1
Masahide Yamamoto, Sayaka Suzuki, Jun-Ichi Mukae, Keisuke Tanaka, Ken Watanabe, Gaku Oshikawa, Tetsuya Fukuda, Naomi Murakami, Osamu Miura
Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/myeloproliferative neoplasm (MDS/MPN). Although recurrent chromosomal and genetic abnormalities are frequently observed in aCML, none are specific to this type of leukemia. The present study reported a case of aCML associated with i(X)(p10), a rare recurrent chromosomal abnormality of hematological malignancy. A 40-year-old female was referred to the Tokyo Medical and Dental University Hospital (Tokyo, Japan) due to slight leukocytosis and anemia...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28924539/genetic-polymorphisms-of-hemostatic-factors-and-thrombotic-risk-in-non-bcr-abl-myeloproliferative-neoplasms-a-pilot-study
#2
R Dambrauskienė, R Gerbutavičius, R Ugenskienė, R Jankauskaitė, A Savukaitytė, R Šimoliūnienė, M Rudžianskienė, R Gerbutavičienė, E Juozaitytė
The most important complications of Philadelphianegagive (non BCR-ABL) myeloproliferative neoplasms (MPNs) are vascular events. Our aim was to evaluate the effects of single nucleotide polymorphisms (SNPs), platelet glycoproteins (GPs) (Ia/IIa, Ibα, IIb/IIIa and VI), von Willebrand factor (vWF), coagulation factor VII (FVII), β-fibrinogen, and the risk of thrombosis in patients with non BCR-ABL MPNs at the Lithuanian University of Health Sciences. Kaunas, Lithuania. Genotyping was done for 108 patients. The TT genotype of the GP Ia/IIa c...
June 30, 2017: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28919910/chloroma-of-the-testis-in-a-patient-with-a-history-of-acute-myeloid-leukemia
#3
Mohammad Hossein Sanei, Matin Shariati
Chloroma, or granulocytic sarcoma, is a rare extramedullary solid hematologic cancer, found concomitant with acute myeloid leukemia. It is infrequently associated with other myeloproliferative disorders or chronic myelogenous leukemia. Chloroma of the testis after allogeneic bone marrow transplantation is particularly sparsely represented in the literature. It is suggested that an appropriate panel of marker studies be performed along with clinical correlation and circumspection to avoid misleading conclusions...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28914569/mutations-in-myeloproliferative-neoplasms-their-significance-and-clinical-use
#4
Fiorella Schischlik, Robert Kralovics
Clonal hematologic diseases of the blood such as polycythemia vera, essential thrombocythemia and primary myelofibrosis belong to the BCR-ABL negative Myeloproliferative Neoplasms (MPN). These diseases are characterized by clonal expansion of hematopoietic precursor cells followed by increased production of differentiated cells of the myeloid lineage. Initiation of clonal hematopoiesis, formation of a clinical phenotype as well as disease progression form part of MPN disease evolution. The disease is driven by acquired somatic mutations in critical pathways such as cytokine signaling, epigenetic regulation, RNA splicing, and transcription factor signaling...
September 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28903325/cxcl12-cxcr4-pathway-is-activated-by-oncogenic-jak2-in-a-pi3k-dependent-manner
#5
Hadjer Abdelouahab, Yanyan Zhang, Monika Wittner, Shinya Oishi, Nobutaka Fujii, Rodolphe Besancenot, Isabelle Plo, Vincent Ribrag, Eric Solary, William Vainchenker, Giovanni Barosi, Fawzia Louache
JAK2 activation is the driver mechanism in BCR-ABL-negative myeloproliferative neoplasms (MPN). These diseases are characterized by an abnormal retention of hematopoietic stem cells within the bone marrow microenvironment and their increased trafficking to extramedullary sites. The CXCL12/CXCR4 axis plays a central role in hematopoietic stem cell/ progenitor trafficking and retention in hematopoietic sites. The present study explores the crosstalk between JAK2 and CXCL12/CXCR4 signaling pathways in MPN. We show that JAK2, activated by either MPL-W515L expression or cytokine stimulation, cooperates with CXCL12/CXCR4 signaling to increase the chemotactic response of human cell lines and primary CD34(+) cells through an increased phosphatidylinositol-3-kinase (PI3K) signaling...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28902850/copy-number-variation-analysis-in-cytochromes-and-glutathione-s-transferases-may-predict-efficacy-of-tyrosine-kinase-inhibitors-in-chronic-myeloid-leukemia
#6
Alexander V Lavrov, Oksana A Ustaeva, Elmira P Adilgereeva, Svetlana A Smirnikhina, Ekaterina Y Chelysheva, Oleg A Shukhov, Yuriy V Shatokhin, Sergey V Mordanov, Anna G Turkina, Sergey I Kutsev
Chronic myeloid leukemia (CML) is a myeloproliferative disease characterized by the presence of BCR/ABL fusion gene in leukemic cells, which promotes uncontrolled cell proliferation. Up to 20% of CML patients show primary resistance or non-optimal response to tyrosine kinase inhibitor (TKI) therapy. We investigated the association between copy number variation (CNV) in glutathione S-transferases (GST) and cytochromes (CYP) and the response rate to TKI. We enrolled 47 patients with CML: 31 with an optimal response and 16 with failure at 6 months in accordance with European LeukemiaNet 2013 recommendations...
2017: PloS One
https://www.readbyqxmd.com/read/28885734/chronic-myelomonocytic-leukemia-masquerading-as-cutaneous-indeterminate-dendritic-cell-tumor-expanding-the-spectrum-of-skin-lesions-in-chronic-myelomonocytic-leukemia
#7
Sanam Loghavi, Jonathan L Curry, Guillermo Garcia-Manero, Keyur P Patel, Jie Xu, Joseph D Khoury, Carlos A Torres-Cabala, Priyadharsini Nagarajan, Phyu P Aung, Bernard R Gibson, Brandon P Goodwin, Brent C Kelly, Brinda Rao Korivi, L Jeffrey Medeiros, Victor G Prieto, Hagop M Kantarjian, Carlos E Bueso-Ramos, Michael T Tetzlaff
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features and characterized by persistent relative and absolute monocytosis in the peripheral blood.
September 8, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28885361/angioimmunoblastic-t-cell-lymphoma-and-hypereosinophilic-syndrome-with-fip1l1-pdgfra-fusion-gene-effectively-treated-with-imatinib-a-case-report
#8
Masayo Yamamoto, Katsuya Ikuta, Yasumichi Toki, Mayumi Hatayama, Motohiro Shindo, Yoshihiro Torimoto, Toshikatsu Okumura
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28882478/corrigendum-to-the-expression-of-death-inducer-obliterator-dido-variants-in-myeloproliferative-neoplasms-blood-cells-mol-dis-59-2016-25-30
#9
Maria Gabriela Berzoti-Coelho, Aline Fernanda Ferreira, Natalia de Souza Nunes, Mariana Tomazini Pinto, Maurício Cristiano Rocha Júnior, Belinda Pinto Simões, Carlos Martínez-A, Elizabeth Xisto Souto, Rodrigo Alexandre Panepucci, Dimas Tadeu Covas, Simone Kashima, Fabíola Attié Castro
No abstract text is available yet for this article.
September 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28881484/primary-cells-in-bcr-fgfr1-positive-8p11-myeloproliferative-syndrome-are-sensitive-to-dovitinib-ponatinib-and-dasatinib
#10
Niklas Landberg, Arta Dreimane, Marianne Rissler, Rolf Billström, Helena Ågerstam
OBJECTIVES: Translocations involving the Fibroblast growth factor receptor 1 (FGFR1) gene are associated with the 8p11 myeloproliferative syndrome (EMS), a rare neoplasm that following a usually short chronic phase progresses into acute myeloid or lymphoid leukemia. The treatment commonly involves chemotherapy and, if possible, allogeneic stem cell transplantation which is the only therapeutic option for long term survival. Given the aggressive course of EMS, we here evaluated tyrosine kinase inhibitors as treatment options to delay disease progression...
September 7, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#11
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28869184/the-treatment-landscape-of-myelofibrosis-before-and-after-ruxolitinib-approval
#12
Andrew T Kuykendall, Chetasi Talati, Najla Al Ali, Kendra Sweet, Eric Padron, David A Sallman, Jeffrey E Lancet, Alan F List, Kenneth S Zuckerman, Rami S Komrokji
INTRODUCTION/BACKGROUND: Myelofibrosis (MF) is a chronic myeloproliferative neoplasm that presents with a heterogeneous clinical phenotype and prognosis. Before the US Food and Drug Administration approval of ruxolitinib, treatment options were varied and had limited effect. The increased use of ruxolitinib has drastically altered the MF treatment landscape. In this study, we aimed to clarify the clinical situations in which ruxolitinib is being used and analyze its effect on this landscape...
August 5, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28866671/autoimmune-myelofibrosis-clinical-features-course-and-outcome
#13
Caroline I Piatek, Maria E Vergara-Lluri, Vinod Pullarkat, Imran N Siddiqi, Casey O'Connell, Russell K Brynes, Donald I Feinstein
BACKGROUND: Autoimmune myelofibrosis (AIMF) is an underrecognized cause of nonmalignant bone marrow fibrosis which occurs in the presence or absence of a defined systemic autoimmune disease. Patients with AIMF present with cytopenias and autoantibodies, and have a distinctive nonclonal myelofibrosis on bone marrow examination. AIMF is distinguished from primary myelofibrosis by the absence of splenomegaly, eosinophilia, or basophilia, and the absence of abnormal myeloid, erythroid, or megakaryocytic morphology...
September 2, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28863787/venous-thromboembolism-and-hyperhomocysteinemia-as-first-manifestation-of-pernicious-anemia-a-case-series
#14
W Ammouri, Z Mezalek Tazi, H Harmouche, M Maamar, M Adnaoui
BACKGROUND: Hyperhomocysteinemia has been suspected of favoring thrombosis. Several case-control studies and even a meta-analysis have confirmed a link between venous thrombosis and hyperhomocysteinemia. Homocysteine is due to genetic and acquired factors (poor diet in folate and vitamin B12, older age, renal impairment, thyroid diseases, and malignancies) induced by the intake and the concentrations of vitamin B9 or B12 in the majority of cases. CASES PRESENTATION: We report the cases of four Moroccan patients who presented with acute vein thrombosis of different sites: a 34-year-old man, a 60-year-old man, a 58-year-old man, and a 47-year-old woman...
September 2, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28860000/pre-engraftment-bloodstream-infections-after-allogeneic-hematopoietic-cell-transplantation-the-impact-of-t-repleted-transplant-from-haploidentical-donors
#15
Malgorzata Mikulska, Anna Maria Raiola, Federica Galaverna, Elisa Balletto, Maria Lucia Borghesi, Riccardo Varaldo, Francesca Gualandi, Livia Giannoni, Giordana Pastori, Daniele Roberto Giacobbe, Alessio Signori, Valerio Del Bono, Claudio Viscoli, Andrea Bacigalupo, Emanuele Angelucci
Bloodstream infections (BSI) are frequent and important infectious complications after hematopoietic cell transplantation (HCT). The aim of this study was to analyze the incidence, risk factors and outcome of pre-engraftment BSI after allogeneic HCT. A retrospective analysis of data from 553 consecutive patients transplanted in years 2010-2016 was performed. Sixty percent of patients received T-repleted unmanipulated haploidentical bone marrow with high dose post-transplant cyclophosphamide. BSI rate was 30%; among isolated 213 pathogens, 54% were Gram-positive, 43% Gram-negative and 3% fungi...
August 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859112/mathematical-modelling-as-a-proof-of-concept-for-mpns-as-a-human-inflammation-model-for-cancer-development
#16
Morten Andersen, Zamra Sajid, Rasmus K Pedersen, Johanne Gudmand-Hoeyer, Christina Ellervik, Vibe Skov, Lasse Kjær, Niels Pallisgaard, Torben A Kruse, Mads Thomassen, Jesper Troelsen, Hans Carl Hasselbalch, Johnny T Ottesen
The chronic Philadelphia-negative myeloproliferative neoplasms (MPNs) are acquired stem cell neoplasms which ultimately may transform to acute myelogenous leukemia. Most recently, chronic inflammation has been described as an important factor for the development and progression of MPNs in the biological continuum from early cancer stage to the advanced myelofibrosis stage, the MPNs being described as "A Human Inflammation Model for Cancer Development". This novel concept has been built upon clinical, experimental, genomic, immunological and not least epidemiological studies...
2017: PloS One
https://www.readbyqxmd.com/read/28859041/case-report-clinical-variation-in-children-with-thrombopoietin-receptor-c-mpl-mutations-report-of-2-cases
#17
Clara Lo, Elysia Alvarez, Robert S Ohgami, Michael Jeng
Congenital amegakaryocytic thrombocytopenia (CAMT, MIM# 604498) is a rare congenital bone marrow failure syndrome which presents early in life with abnormal bleeding because of thrombocytopenia. Classically, megakaryocytes are decreased to absent in the bone marrow. The development of aplastic anemia early in childhood has led to the recommendation for early stem cell transplantation. Quantitative or loss-of-function mutations in the myeloproliferative leukemia gene (c-mpl), whose gene product functions as the thrombopoietin receptor, have been identified as causative for CAMT...
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28854087/myeloproliferative-neoplasms
#18
LETTER
Sucha Nand
No abstract text is available yet for this article.
August 31, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28854086/myeloproliferative-neoplasms
#19
LETTER
Jerry L Spivak
New England Journal of Medicine, Volume 377, Issue 9, Page 894-896, August 2017.
August 31, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28853613/lung-endothelial-microrna-1-regulates-tumor-growth-and-angiogenesis
#20
Asawari Korde, Lei Jin, Jian-Ge Zhang, Anuradha Ramaswamy, Buqu Hu, Saeed Kolahian, Brenda Juan Guardela, Jose Herazo-Maya, Jill M Siegfried, Laura Stabile, Margaret A Pisani, Roy S Herbst, Naftali Kaminski, Jack A Elias, Jonathan T Puchalski, Shervin S Takyar
RATIONALE: VEGF down-regulates miR-1 in the lung endothelium, and endothelial cells play a critical role in tumor progression and angiogenesis. OBJECTIVES: To examine the clinical significance of miR-1 in NSCLC and its specific role in tumor endothelium. Method and measurements: MiR-1 levels were measured by Taqman assay. Endothelial cells were isolated by magnetic sorting. We used VE-cadherin promoter to create a vascular-specific miR-1 lentiviral vector and an inducible transgenic mouse...
August 30, 2017: American Journal of Respiratory and Critical Care Medicine
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