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myeloproliferative syndrome treatment

X Qi, G Han, X Guo, V De Stefano, K Xu, Z Lu, H Xu, A Mancuso, W Zhang, X Han, D C Valla, D Fan
BACKGROUND: China may have the largest number of Budd-Chiari syndrome (BCS) cases in the world (at least 1914 original papers were published, and at least 20 191 BCS patients were reported). Considering the discrepancy in the clinical profiles and preferred treatment selection of primary BCS between the West and China, understanding its aetiology in these two different regions is very important. AIM: To review the data from large cohort studies and meta-analyses to illustrate the epidemiology of risk factors for BCS in the West and China...
October 13, 2016: Alimentary Pharmacology & Therapeutics
Norio Asou
Current treatment of acute myeloid leukemia (AML) still relies on intensive chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT). AML is a heterogeneous neoplasm characterized by distinct chromosomal and genetic abnormalities. Recent comprehensive gene analyses have highlighted distinct genetic subgroups that are associated with different responses to chemotherapy. Therefore, the molecular landscape of AML is fundamental to the development of novel therapeutic approaches and provides opportunities for individualization of therapy...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Silvina Laura Lompardía, Mariángeles Díaz, Daniela Laura Papademetrio, Matías Pibuel, Élida Álvarez, Silvia Elvira Hajos
Chronic myeloid leukemia (CML) is a myeloproliferative syndrome characterized by the presence of the Philadelphia chromosome which encodes a constitutively activated tyrosine kinase (BCR-ABL). The first line treatment for CML consists on BCR-ABL inhibitors such as Imatinib. Nevertheless, such treatment may lead to the selection of resistant cells. Therefore, it is of great value to find molecules that enhance the anti-proliferative effect of first-line drugs. Hyaluronan is the main glycosaminglican of the extracellular matrix which is involved in tumor progression and multidrug resistance...
October 8, 2016: Investigational New Drugs
Maria Helena Ornellas, Monique De França Silva, Cristiana Solza, Stella Beatriz Sampaio De Lucena Gonçalves, Liliane Silva De Almeida, Jackline De Paula Ayres-Silva, Taís Leite Seixas, Elenice Ferreira Bastos, Thomas Liehr, Gilda Alves
Myelodysplastic syndrome (MDS) cases comprise a heterogeneous group of hematological disorders that are characterized by impaired hematopoiesis, with cytopenias of different grades and risk of developing acute myeloid leukemia. MDS may rarely be associated with thrombocytosis. In such cases, myelodysplasia and myeloproliferative disorders may overlap, making correct diagnosis difficult. We herein describe a case of MDS with thrombocytosis, Janus kinase 2 gene mutation-positive and Perls' staining-negative, which was initially classified as essential thrombocythemia (ET)...
September 2016: Molecular and Clinical Oncology
Ya-Ling Zhao, Gui-Min Liu, Li-Jun Zhang, Wen-Tong Liang, Zhi-Yong Cheng
Jauns kinase (JAK)/transducer and activator of transcription(STAT) pathway is a classical approach to study the rapid changes of the gene expression in specific target cells by a variety of extracellular signals. The JAK and STAT transfer cytokine receptor signaling plays a unique role in multiple cellular and molecular biological changes.The abnormal signal of JAK/STAT pathway will lead to the hematopoietic abnormalities.Studies had shown that the abnormal activation of JAK2/STAT signaling pathway are in many kinds of malignant hematological diseases, such as in acute lymphoblastic/myeloid leukemia, chronic myeloid leukemia, lymphoma, myelodysplastic syndromes, myeloprofilerative neoplasm, especially in the patients of myeloproliferative neoplasm(MPN) with JAK gene mutation(JAK2V617F), this mutation has an important value for MPN diagnosis...
August 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
Carlo Mainetti, Caroline Guillod, Sandra Leoni-Parvex
Ingenol mebutate (IM) has recently been approved for the topical treatment of actinic keratoses. It appears to have a dual mechanism of action: rapid necrosis after gel application and a subsequent immune-mediated response, which targets any residual dysplastic epidermal cells. We report the successful treatment of a woman, who had been relapsing into Bowen's disease (BD) on her right forefinger for 8 years. During her clinical history, she had received an allogeneic, HLA-identical stem cell transplant for myeloproliferative syndrome with a JAK2V617F mutation and lobectomy of the pulmonary right lower lobe for adenocarcinoma...
2016: Dermatology: International Journal for Clinical and Investigative Dermatology
V Valkova, J Jircikova, M Trnkova, K Steinerova, P Keslova, M Lanska, Z Koristek, L Raida, M Krejci, K Kruntoradova, T Dolezal, K Benesova, P Cetkovsky, M Trneny
UNLABELLED: Although allogeneic haematopoietic stem cell transplantation (allo-HSCT) offers a unique curative potential, it may be connected with high treatment-related morbidity and mortality. Besides many organ complications, allo-HSCT may significantly affect quality of life (QOL). PATIENTS AND METHODS: Between January 2011 and December 2012, five hundred and ninety patients (pts) from 6 transplant centers in the Czech Republic filled in the questionnaire for the quantitative measurement of QOL using Functional Assessment of Cancer Therapy-General (FACT-G) version 4...
2016: Neoplasma
Shalini Koppisetty, Brain L Edelman, Atul Rajpurkar
Myeloid sarcoma (MS) is a rare extramedullary tumor composed of immature cells of myeloid lineage that destroy the original tissue architecture in which it is found. It is most commonly identified in patients with acute myelogenous leukemia, and less often in myelodysplastic syndromes (MDSs) and other myeloproliferative disorders. It is most commonly reported in the periosteum, bone, skin, and lymph nodes but has been reported in many other sites of the body. Herein, we describe a case of MS involving the periprostatic tissue and review of literature of MS of the prostate...
July 2016: Urology Annals
Mohammed Shafi Abdulsalam, Hari Chandana Ghanta, Prabu Pandurangan, Maya Menon, Sheba S K Jacob
Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterised by marked peripheral eosinophilia and end organ damage attributable to eosinophilia without secondary cause. Early recognition and treatment are essential to prevent morbidity and mortality. Cytoreduction with Steroids, Hydroxyurea and Imatinib are the main stay of treatment. Molecular studies like Fip1-like-1 fused with platelet derived growth factor receptor alpha (FIP1L1-PDGFRα) etc., are recommended in view of therapeutic implication...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
Ting Zhou, Stephanie N Perez, Ziming Cheng, Marsha C Kinney, Madeleine E Lemieux, Linda M Scott, Vivienne I Rebel
Perturbations in CREB binding protein (CREBBP) are associated with hematopoietic malignancies, including myelodysplastic syndrome (MDS). Mice hemizygous for Crebbp develop myelodysplasia with proliferative features, reminiscent of human MDS/myeloproliferative neoplasm-unclassifiable (MDS/MPN-U), and a proportion goes on to develop acute myeloid leukemia (AML). We have also shown that the Crebbp+/- non-hematopoietic bone marrow microenvironment induces excessive myeloproliferation of wild-type cells. We now report that transplantation of unfractionated Crebbp+/- bone marrow into wild-type recipients resulted in either early-onset AML or late-onset MDS and MDS/MPN-U...
2016: PloS One
Sebastián Ortiz, Maria T Orero, Karla Javier, Carolina Villegas, Irene Luna, Pedro Pérez, Mónica Roig, María López, Sofía Costa, Félix Carbonell, Rosa Collado, David Ivars, Mariano Linares
BACKGROUND: The incidence of alloimmunisation in myelodysplastic syndromes (MDS) during the era of supportive treatment ranges from 9 to 56%. However, it is unknown if the widespread use of hypomethylating agents has changed the risk of immunisation. The aim of this study is to evaluate the impact of azacitidine (AZA) therapy on red blood cell (RBC) alloimmunisation in transfused patients with MDS, myelodysplastic syndromes/myeloproliferative syndromes (MDS/MPS) and secondary acute myeloid leukaemia (AML)...
June 24, 2016: Blood Transfusion, Trasfusione del Sangue
Eleftheria Hatzimichael, Konstantinos Lagos, Amalia Vassou, Dora Gougopoulou, Alexandra Papoudou-Bai, Evangelos Briasoulis
Loss of a section of the long arm of chromosome 5, as a sole cytogenetic abnormality, characterizes a rare type of myelodysplastic syndrome [del(5q) MDS] and the co-existence of the JAK2 V617F mutation occurs in a small subset of these cases. Patients with isolated del(5q) MDS have a relatively favorable prognosis, with transformation to acute myeloid leukemia occurring in <10%, and their disease responds well to lenalidomide. However the optimal therapeutic approach for patients with del(5q) MDS in coexistence with the JAK2 V617F mutation, which is common to myeloproliferative neoplasms, remains to be elucidated...
July 2016: Molecular and Clinical Oncology
Masaaki Hidaka, Susumu Eguchi
Budd-Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Western countries. Multi-step management is required for such patients. Interventional revascularization and TIPS procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC...
June 1, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
Sophia Maschalidi, Fernando E Sepulveda, Alexandrine Garrigue, Alain Fischer, Geneviève de Saint Basile
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, characterized by severe hyperinflammation and immunopathological manifestations in several tissues. These features result from organ infiltration by overactivated CD8 T-cells and macrophages, which produce high levels of pro-inflammatory cytokines, such as IFN-γ, TNF-α, IL-6, and IL-18. Recently, several Janus kinase 1/2 (JAK1/2) inhibitors, such as ruxolitinib, have been developed as immunosuppressive agents. They have proven beneficial effects in the treatment of myeloproliferative disorders and inflammatory conditions...
July 7, 2016: Blood
Haru Yamamoto, David A Khan
A 45-year-old man who presented with dyspnea and chest tightness was found to have obstructive lung disease and eosinophilia of 10,300 eosinophils/μL. The differential diagnosis encompassed causes of primary eosinophilia and secondary eosinophilia associated with pulmonary disease, including asthma, environmental allergic reaction, eosinophilic granulomatosis with polyangiitis, allergic bronchopulmonary aspergillosis, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, parasitic infections, tuberculosis, fungal infection, sarcoidosis, mastocytosis, drug reaction with eosinophilia and systemic symptoms, lymphoproliferative hypereosinophilic syndrome, and myeloproliferative hypereosinophilic syndrome...
May 2016: Allergy and Asthma Proceedings:
Florence Roufosse, Pierre Heimann, Frédéric Lambert, Pierre Sidon, Dominique Bron, Vincent Cottin, Jean-François Cordier
Chronic eosinophilic leukaemia associated with the FIP1L1-PDGFRA fusion gene (F/P+ CEL) is a rare cause of marked persistent hypereosinophilia, arising almost exclusively in male patients. Clinical presentations are heterogeneous with a higher incidence of eosinophil-mediated cardiomyopathy than in other hypereosinophilic syndrome variants. Features of chronic myeloproliferative disease are often present, including splenomegaly and elevated serum vitamin B12 levels. The diagnosis is made by fluorescence in situ hybridization (FISH) showing the deletion of the CHIC2 locus and/or RT-PCR showing the FIP1L1-PDGFRA fusion transcript...
2016: Respiration; International Review of Thoracic Diseases
Aziz Nazha, Thomas Prebet, Steven Gore, Amer M Zeidan
Chronic myelomonocytic leukemia (CMML) is a unique disease entity with overlap components of both myelodysplastic syndrome and myeloproliferative neoplasms. CMML is a clonal hematopoietic stem cell neoplasm characterized by monocytosis, cytopenias, and extramedullary manifestations such as splenomegaly. The disease is rare and has undergone revisions in its classification. We review the recent classification strategies as well as diagnostic criteria, focusing on the new insights into the genetic alterations and unique pathophysiology of the disease...
September 2016: Blood Reviews
Andrew L Sochacki, Melissa A Fischer, Michael R Savona
The discovery of JAK2 (V617F) a decade ago led to optimism for a rapidly developing treatment revolution in Ph(-) myeloproliferative neoplasms. Unlike BCR-ABL, however, JAK2 was found to have a more heterogeneous role in carcinogenesis. Therefore, for years, development of new therapies was slow, despite standard treatment options that did not address the overwhelming symptom burden in patients with primary myelofibrosis (MF), post-essential thrombocythemia MF, post-polycythemia vera MF, and myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) syndromes...
2016: OncoTargets and Therapy
Gregory L Hundemer, Ivy A Rosales, Yi-Bin Chen, Robert B Colvin, Nina E Tolkoff-Rubin
Myeloproliferative disorders are a rare cause of focal segmental glomerulosclerosis (FSGS), although the mechanism is unclear. Hydroxyurea is commonly used in these disorders for its cytoreductive properties; however, the effect of this treatment on proteinuria or kidney function remains unclear in cases of myeloproliferative disorder-associated FSGS. We describe the clinical course of a patient with polycythemia vera and nephrotic-range proteinuria, demonstrated to have FSGS on biopsy. The patient had a distant history of granulomatosis with polyangiitis (Wegener's), for which he routinely had his kidney function and proteinuria measured, allowing for early detection of nephrotic syndrome soon after being diagnosed with polycythemia vera...
September 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Lanshan Huang, Guillermo Garcia-Manero, Elias Jabbour, Maitrayee Goswami, Mark J Routbort, L Jeffrey Medeiros, Jeffrey L Jorgensen, Sa A Wang
AIMS: Hypomethylating agents (HMAs) exhibit clinical efficacy in patients with myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN). This study was performed to assess residual disease status by flow cytometry immunophenotyping (FCI) methods in patients with MDS or MDS/MPN treated with HMAs, and correlate the findings with clinical response. METHODS: CD34+ myeloid precursors were assessed in 85 patients with MDS and MDS/MPN treated with HMAs using FCI methods...
April 15, 2016: Journal of Clinical Pathology
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