47,127 results match your criteria Myeloproliferative Disease


Developments in diagnosis and treatment of essential thrombocythemia.

Expert Rev Hematol 2019 Feb 22. Epub 2019 Feb 22.

b Universita degli Studi dell'Insubria , Varese 21100 , Italy.

Introduction: Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterized by thrombocytosis, increased risk of thrombotic/hemorrhagic events and clonal evolution into blast phase or myelofibrosis. Areas Covered: The authors will discuss biology, diagnosis, prognosis, therapy and outcome of ET. An accurate molecular-morphologic assessment is necessary in order to properly establish diagnosis and prognosis of ET. Read More

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http://dx.doi.org/10.1080/17474086.2019.1585239DOI Listing
February 2019

More on Blastic Plasmacytoid Dendritic-Cell Neoplasms.

N Engl J Med 2019 Feb 14;380(7):696. Epub 2019 Feb 14.

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February 2019

More on Blastic Plasmacytoid Dendritic-Cell Neoplasms.

N Engl J Med 2019 Feb 14;380(7):695-6. Epub 2019 Feb 14.

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February 2019

Pre-analytical parameters associated with unsuccessful karyotyping in myeloid neoplasm: a study of 421 samples.

Braz J Med Biol Res 2019 Feb 14;52(2):e8194. Epub 2019 Feb 14.

Hospital Israelita Albert Einstein, São Paulo, SP, Brasil.

Cytogenetics is essential in myeloid neoplasms (MN) and pre-analytical variables are important for karyotyping. We assessed the relationship between pre-analytical variables (time from collection to sample processing, material type, sample cellularity, and diagnosis) and failures of karyotyping. Bone marrow (BM, n=352) and peripheral blood (PB, n=69) samples were analyzed from acute myeloid leukemia (n=113), myelodysplastic syndromes (n=73), myelodysplastic syndromes/myeloproliferative neoplasms (n=17), myeloproliferative neoplasms (n=137), and other with conclusive diagnosis (n=6), and reactive disorders/no conclusive diagnosis (n=75). Read More

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http://dx.doi.org/10.1590/1414-431X20188194DOI Listing
February 2019

Ruxolitinib treatment reduces monocytic superoxide radical formation without affecting hydrogen peroxide formation or systemic oxidative nucleoside damage in myelofibrosis.

Leuk Lymphoma 2019 Feb 20:1-9. Epub 2019 Feb 20.

b Institute for Inflammation Research (IIR), Center for Rheumatology and Spine Diseases, Rigshospitalet, University of Copenhagen , Copenhagen , Denmark.

The role of excess reactive oxygen species (ROS) with consequent DNA/RNA damage is now recognized as a hallmark of cancer. In JAK2V617F mutated myeloproliferative neoplasms, ROS have been suggested to be important factors in disease initiation and progression. Ruxolitinib is the most widely used drug for myelofibrosis, because it improves symptom-score. Read More

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http://dx.doi.org/10.1080/10428194.2019.1579323DOI Listing
February 2019

Spontaneous Subdural Hematoma in a Patient with Polycythemia Vera.

World Neurosurg 2019 Feb 16. Epub 2019 Feb 16.

Department of Neurosurgery, Albany Medical Center, Albany, NY, USA.

Polycythemia vera (PV) is a myeloproliferative disorder usually characterized by an increase tendency towards thromboembolic events. Spontaneous hemorrhage/bleeding in PV patients is seldom reported in Neurosurgical literature. We report the case of a 76 year-old male with PV who developed a spontaneous subdural hematoma requiring surgical evacuation. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.01.258DOI Listing
February 2019

Prevalence of MPL (W515K/L) Mutations in Patients with Negative-JAK2 (V617F) Myeloproliferative Neoplasm in North-East of Iran.

Iran J Pathol 2018 25;13(4):397-402. Epub 2018 Sep 25.

Cellular and Molcular Research Center, Birjand University of Medical Sciences, Birjand, Iran.

Background And Objective: Janus kinase 2 (JAK2) and Myeloproliferative Leukemia (MPL) mutations are confirmatory indicators for Myeloproliferative Neoplasm (MPN). The current study was performed to determine the frequency of MPL mutation in MPN patients without JAK2 mutation, in order to assign MPL mutation frequency in North-East of Iran.

Methods: Total of 105 negative JAK2 cases including 5 Myeloproliferative Disorders (MPD), 15 Polycytemia Vera (PV) and 15 Essential Thrombocytosis (ET) who referred to Qaem Medical Center were assigned to this study. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358559PMC
September 2018

F-FLT PET/MRI for bone marrow failure syndrome-initial experience.

EJNMMI Res 2019 Feb 15;9(1):16. Epub 2019 Feb 15.

Biomedical Imaging Research Center, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji-cho, Fukui, 910-1193, Japan.

Background: Bone marrow failure syndrome (BMFS) is a heterogeneous group of disorders associated with single- or multiple-lineage cytopenia and failure of normal hematopoiesis. We assessed the feasibility of integrated PET/MRI with 3'-deoxy-3'-F-fluorothymidine (F-FLT) to assess the pathophysiology of whole-body bone marrow for the diagnosis and monitoring of BMFS. Twenty-five consecutive patients with BMFS underwent a pre-treatment F-FLT PET/MRI scan. Read More

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http://dx.doi.org/10.1186/s13550-019-0490-0DOI Listing
February 2019
1 Read

More on Blastic Plasmacytoid Dendritic-Cell Neoplasms.

Authors:

N Engl J Med 2019 Feb;380(7):695-697

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http://dx.doi.org/10.1056/NEJMc1814963DOI Listing
February 2019

Current perspectives for the treatment of chronic myeloid leukemia

Turk J Med Sci 2019 Feb 11;49(1):1-10. Epub 2019 Feb 11.

With an annual incidence of 1-2 in a million, Ph*(+) chronic myeloid leukemia (CML) is a clonal hematopoietic stem cell disease that makes myeloid neoplastic cells breed out of control. This BCR-ABL(+) myeloproliferative disease makes up about 15%-20% of all leukemia cases in adults. CML is seen more in males than females, with a rate of three to two. Read More

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http://dx.doi.org/10.3906/sag-1810-81DOI Listing
February 2019
1 Read

[Clinical Analysis of 208 Patiets with BCR/ABL Negative Myeloproliferative Neoplasms].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):159-164

Department of Hematology, the Second Affiliated Hospital of Hebei Medical University, Shijiazhuang 050000, Hebei Province, China.E-mail:

Objective: To analyze the incidence, hemogram, genetics, clinical manifestations, therapeutic efficacy and outcome of patients with myeloproliferative neoplasms(MPN) so as to provide much more therapeutic basis for clinically studying the pathogenesis, diagnosis, and treatment as well as evaluating the prognosis of MPN patients.

Methods: The clinical data and related laboratory test results in 208 cases of BCR/ABL fusion gene regative MPN were collected and analyzed retrospectively.

Results: The MPN could occur at any age, but the highest incidence was observed in patients aged 40-79. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.025DOI Listing
February 2019
2 Reads

Long-term outcome after allogeneic hematopoietic cell transplantation for myelofibrosis.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

University Hospital Eppendorf, Hamburg, Germany.

Allogeneic hematopoietic stem cell transplant remains the only curative treatment for myelofibrosis. Most post-transplantation events occur during the first 2 years and hence we aimed to analyze the outcome of 2-year disease-free survivors. 1055 patients with myelofibrosis transplanted between 1995 and 2014 and registered in the registry of the European Society for Blood and Marrow Transplantation were included. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.205211DOI Listing
February 2019
3 Reads

Sustained Response to Imatinib in a Pediatric Patient with Concurrent Myeloproliferative Disease and Lymphoblastic Lymphoma Associated with a CCDC88C-PDGFRB Fusion Gene.

Acta Haematol 2019 Feb 6;141(2):119-127. Epub 2019 Feb 6.

Department of Pediatric Hematology-Oncology, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Ramat-Gan, Israel.

Background: The WHO defined myeloid and lymphoid neoplasms (MLN) with eosinophilia associated with PDGFRB, PDGFRA, FGFR1 rearrangements as a new entity in 2016. PDGFRB-rearranged MLN sensitive to imatinib were described in adult patients. We report the first pediatric patient with PDGFRB-rearranged myeloproliferative disorder associated with T-lymphoblastic lymphoma bearing the t(5; 14)(q33;q32) translocation who was successfully treated with imatinib only. Read More

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http://dx.doi.org/10.1159/000495687DOI Listing
February 2019
2 Reads

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN with RS-T) complicated by hyperleukocytosis and gene analysis in relation to leukocytosis.

J Clin Exp Hematop 2019 Feb 7. Epub 2019 Feb 7.

Myelodysplastic/myeloproliferative neoplasm (MDS/MPN) with ring sideroblasts and thrombocytosis (MDS/MPN with RS-T), which exhibits both an increased number of marrow ring sideroblasts and thrombocytosis, is a rare disorder classified as one of the newly established forms of MDS/MPN in the WHO 2016 classification. A 77-year-old female with marked thrombocytosis of 1,024×10/L was tentatively diagnosed with essential thrombocythemia in 2011, and the thrombocytosis was controlled using hydroxycarbamide and low-dose busulfan. In 2016, the leukocyte count increased to a peak value of 68. Read More

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http://dx.doi.org/10.3960/jslrt.18037DOI Listing
February 2019
1 Read

Vitamin D receptor-mediated skewed differentiation of macrophages initiates myelofibrosis and subsequent osteosclerosis.

Blood 2019 Feb 4. Epub 2019 Feb 4.

Hematology, Department of Medicine, Kobe University Graduate School of Medicine, Kobe, Japan;

Myelofibrosis in myeloproliferative neoplasms (MPNs) with mutations such as JAK2V617F is an unfavorable sign for uncontrollable disease progression in the clinic and is complicated with osteosclerosis whose pathogenesis is largely unknown. Because several studies have revealed that macrophages are indispensable supporter for bone forming osteoblasts, we speculated that macrophages might play a significant role in the proliferation of collagen-producing myofibroblasts in marrow fibrotic tissues. Here, we show that myelofibrosis critically depends on macrophages whose differentiation is skewed by vitamin D receptor (VDR) signaling. Read More

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http://dx.doi.org/10.1182/blood-2018-09-876615DOI Listing
February 2019
3 Reads

GATA2 hypomorphism induces chronic myelomonocytic leukemia in mice.

Cancer Sci 2019 Feb 2. Epub 2019 Feb 2.

Department of Molecular Hematology, Tohoku University Graduate School of Medicine, Sendai, Japan.

The transcription factor GATA2 regulates normal hematopoiesis, particularly in stem cell maintenance and myeloid differentiation. Various heteroallelic GATA2 gene mutations are associated with a variety of hematological neoplasms, including myelodysplastic syndromes and leukemias. Here, we report that impaired GATA2 expression induces myelodysplastic and myeloproliferative neoplasm development in elderly animals, and this neoplasm resembles chronic myelomonocytic leukemia in humans. Read More

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http://doi.wiley.com/10.1111/cas.13959
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http://dx.doi.org/10.1111/cas.13959DOI Listing
February 2019
13 Reads

Differential effect of inhibitory strategies of the V617 mutant of JAK2 on cytokine receptor signaling.

J Allergy Clin Immunol 2019 Jan 29. Epub 2019 Jan 29.

Ludwig Institute for Cancer Research, 1200 Brussels, Belgium; de Duve Institute, Université catholique de Louvain, 1200 Brussels, Belgium; WELBIO (Walloon Excellence in Life Sciences and Biotechnology), Brussels, Belgium. Electronic address:

Background: Janus Kinase 2 (JAK2) plays pivotal roles in signaling by several cytokine receptors. The mutant JAK2 V617F is the most common molecular event associated with myeloproliferative neoplasms. Selective targeting of the mutant would be ideal for treating these pathologies by sparing essential JAK2 functions. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193011
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http://dx.doi.org/10.1016/j.jaci.2018.12.1023DOI Listing
January 2019
2 Reads

[The impact of meisoindigo on apoptosis and proliferation of SET2 cell line by JAK-STAT pathway].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):29-34

Institute of Hematology and Blood Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin 300020, China.

To observe the effect of meisoindigo on apoptosis and proliferation of JAK2/V617F heterozygous mutation cell line-SET2 cell line to further explore the role of JAK-STAT pathway in this effect. Cell apoptosis after treated with different concentration of meisoindigo (0, 5, and 10 μmol/L) was evaluated by flow cytometry at different time points (24, 48, 72 h). Cell proliferation with CCK8 test was evaluated at different time points (24, 48, 72, 96 h) after administered with different concentration of meisoindigo (0, 5, 10, and 20 μmol/L). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.006DOI Listing
January 2019
2 Reads

Acute ischemic stroke in the setting of essential thrombocytemia (clinical cases).

Ter Arkh 2018 Aug;90(7):102-104

National Research Center of Hematology, Moscow, Russia.

This article describes several clinical cases of acute ischemic stroke among patients suffering from essential thrombocytemia. Ambiguity of etiological factors of stroke is demonstrated among patients with this pathology. Thrombocytosis and high allele load in the Jak2 gene play an important role (even with normal platelet count) in progression of cerebrovascular disease. Read More

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http://dx.doi.org/10.26442/terarkh2018907102-104DOI Listing
August 2018
1 Read

Cepeginterferon alfa-2b in the treatment of chronic myeloproliferative diseases.

Ter Arkh 2018 Aug;90(7):23-29

National Research Center for hematology, Moscow, Russia.

Aim: A comparative evaluation of the effectiveness of different therapeutic strategies in patients with polycythemia vera (PV) and essential thrombocythemia (ET).

Materials And Methods: Patients with PV or ET, diagnosed according to the criteria WHO 2016 were included in the study. The primary endpoint - 6 months of therapy (clinical-hematological and molecular responses). Read More

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http://dx.doi.org/10.26442/terarkh201890723-29DOI Listing
August 2018
9 Reads

Massive Hemoptysis Due to the Rupture of Thoracic Aortic Aneurysm Caused by Leukemic Cell Infiltration in a Patient With Chronic Myelomonocytic Leukemia.

J Clin Med Res 2019 Feb 5;11(2):145-150. Epub 2019 Jan 5.

Department of Clinical Pathology, Asahi General Hospital, Chiba, Japan.

Hemoptysis is occasionally experienced in patients with hematological malignancies who have respiratory tract infection and severe thrombocytopenia. Thrombocytopenia due to hematological disease is one cause of hemoptysis. Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy characterized by both a myeloproliferative neoplasm and a myelodysplastic syndrome. Read More

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http://dx.doi.org/10.14740/jocmr3712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340676PMC
February 2019
1 Read

Relationship between symptom burden and disability leave among patients with myeloproliferative neoplasms (MPNs): findings from the Living with MPN patient survey.

Ann Hematol 2019 Jan 29. Epub 2019 Jan 29.

UT Health San Antonio Cancer Center, San Antonio, TX, USA.

Patients with myeloproliferative neoplasms (MPNs) experience burdensome symptoms that negatively affect their quality of life. How MPN symptoms relate with medical disability leave (MDL) among patients with the disease has not been previously examined. Using data collected from the Living with MPNs patient survey, symptom burden and functional status were compared in patients who reported taking MDL due to their MPN versus patients who reported no changes in employment status. Read More

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http://dx.doi.org/10.1007/s00277-019-03610-4DOI Listing
January 2019
1 Read

Sequentially inducible mouse models reveal that Npm1 mutation causes malignant transformation of Dnmt3a-mutant clonal hematopoiesis.

Leukemia 2019 Jan 28. Epub 2019 Jan 28.

The Jackson Laboratory, Bar Harbor, ME, USA.

Clonal hematopoiesis (CH) is a common aging-associated condition with increased risk of hematologic malignancy. Knowledge of the mechanisms driving evolution from CH to overt malignancy has been hampered by a lack of in vivo models that orthogonally activate mutant alleles. Here, we develop independently regulatable mutations in DNA methyltransferase 3A (Dnmt3a) and nucleophosmin 1 (Npm1), observed in human CH and AML, respectively. Read More

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http://www.nature.com/articles/s41375-018-0368-6
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http://dx.doi.org/10.1038/s41375-018-0368-6DOI Listing
January 2019
4 Reads

Severe Eosinophilia in Myelodysplastic Syndrome With a Defined and Rare Cytogenetic Abnormality.

Front Immunol 2018 9;9:3031. Epub 2019 Jan 9.

Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osaka-Sayama, Japan.

Myelodysplastic syndromes (MDS) are a heterogeneous group clonal disorders of hematopoietic stem cells (HSC) characterized by ineffective hematopoiesis that lead to variable grades of impaired blood cell production. Chromosomal aberrations are often detected in MDS patients and thus cytogenetic analysis is useful for the diagnosis of these disorders. Common recurring chromosomal defects, such as the -5/5q- and -7/7q- are relatively well characterized cytogenetic abnormalities in MDS, however, the biological significance of uncommon cytogenetic alterations is unknown. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334338PMC
January 2019
1 Read

Polycythaemia Vera among Sudanese Patients with Special Emphasis on JAK2 Mutations

Asian Pac J Cancer Prev 2019 Jan 25;20(1):41-44. Epub 2019 Jan 25.

Department of Haematology, Faculty of Medical Laboratory Sciences, Al-Neelain University, Khartoum, Sudan.

Background: In recent years, a somatic point mutation in the Janus Kinase 2 (JAK2) gene (1849 G→T, V617F) has been reported to occur in over 90% of patients with polycythemia vera (PV). Another JAK2 mutation in exon 12 had been described and shown capable of activating erythropoietin signaling pathways. Objective: In this study, we aimed to determine the frequency of Jak2 mutations (JAK2V617F and JAK2 exon 12) as well as their relationships with hematological parameters in Sudanese patients with myeloproliferative disorders (MPD). Read More

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http://dx.doi.org/10.31557/APJCP.2019.20.1.41DOI Listing
January 2019
1 Read

Juvenile myelomonocytic leukemia: who's the driver at the wheel?

Blood 2019 Jan 22. Epub 2019 Jan 22.

Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Germany.

Juvenile myelomonocytic leukemia (JMML) is a unique clonal hematopoietic disorder of early childhood. It is classified as an overlap myeloproliferative/myelodysplastic neoplasm by the World Health Organization (WHO) and shares some features with chronic myelomonocytic leukemia in adults. JMML pathobiology is characterized by constitutive activation of the Ras signal transduction pathway. Read More

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http://dx.doi.org/10.1182/blood-2018-11-844688DOI Listing
January 2019
5 Reads

MDS overlap disorders and diagnostic boundaries.

Blood 2019 Jan 22. Epub 2019 Jan 22.

Division of Hematology and Oncology, University of California, San Diego, Moores Cancer Center, La Jolla, CA, United States

Myelodysplastic syndromes (MDS) are clonal diseases defined by clinical, morphologic and genetic features often shared by related myeloid disorders. The diagnostic boundaries between these diseases can be arbitrary and not necessarily reflective of underlying disease biology or outcomes. In practice, measures that distinguish MDS from related disorders may be difficult to quantify and can vary as disease progression occurs. Read More

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http://dx.doi.org/10.1182/blood-2018-10-844670DOI Listing
January 2019
1 Read

Detection of fusion gene transcripts in the saliva of Nigerian patients with chronic myeloid leukemia.

Niger J Clin Pract 2019 Jan;22(1):51-55

Department of Medical Laboratory Sciences, Faculty of Health Sciences and Technology, University of Nigeria, Enugu, Nigeria.

Background: The presence of BCR-ABL1 fusion gene resulting from a t(9; 22) reciprocal chromosome translocation is the molecular hallmark of chronic myeloid leukemia (CML). In the diagnosis and treatment of CML, peripheral blood or bone marrow samples are usually taken for analysis. However, both methods are invasive sample collection methods, thus a noninvasive saliva sample method for the detection of the fusion gene transcripts (BCR-ABL) was investigated in some Nigerians with CML. Read More

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http://dx.doi.org/10.4103/njcp.njcp_225_18DOI Listing
January 2019
1 Read

Epigenomic profiling of myelofibrosis reveals widespread DNA methylation changes in enhancer elements and ZFP36L1 as a potential tumor suppressor gene epigenetically regulated.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

Centro de Investigacion Medica Aplicada, Universidad de Navarra, Pamplona, Spain;

In this study we have interrogated the DNA methylome of myelofibrosis patients using high-density DNA methylation arrays. We detected 35,215 differentially methylated CpGs corresponding to 10,253 genes between myelofibrosis patients and healthy controls. These changes were present both in primary and secondary myelofibrosis, which showed no differences between them. Read More

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http://www.haematologica.org/lookup/doi/10.3324/haematol.201
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http://dx.doi.org/10.3324/haematol.2018.204917DOI Listing
January 2019
5 Reads

Antilymphocyte Globulin for matched sibling donor transplantation in patients with myelofibrosis.

Haematologica 2019 Jan 17. Epub 2019 Jan 17.

University Hospital Eppendorf, Hamburg, Germany.

Antihuman T-lymphocyte immunoglobulin is still much debated in the setting of transplant from an HLA matched related donor. Acute and chronic graft-versus-host disease are the main cause of morbidity and mortality after allogeneic hematopoietic stem cell in patients with myelofibrosis. The aim of this study was to evaluate the effect of antihuman T-lymphocyte immunoglobulin in a large cohort of patients with myelofibrosis. Read More

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http://dx.doi.org/10.3324/haematol.2018.201400DOI Listing
January 2019
4 Reads

Unfolding the Role of Calreticulin in Myeloproliferative Neoplasm Pathogenesis.

Clin Cancer Res 2019 Jan 17. Epub 2019 Jan 17.

Human Oncology and Pathogenesis Program, Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center.

In 2013, two seminal studies identified gain of function mutations in the Calreticulin (CALR) gene in a subset of JAK2/MPL-negative myeloproliferative neoplasm (MPN) patients. CALR is an endoplasmic reticulum (ER) chaperone protein that normally binds misfolded proteins in the ER and prevents their export to the Golgi and had never previously been reported mutated in cancer or to be associated with hematological disorders. Further investigation determined that mutated CALR is able to achieve oncogenic transformation primarily through constitutive activation of the MPL-JAK-STAT signaling axis. Read More

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http://clincancerres.aacrjournals.org/lookup/doi/10.1158/107
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http://dx.doi.org/10.1158/1078-0432.CCR-18-3777DOI Listing
January 2019
4 Reads

A Case of Myeloproliferative Neoplasm with BCR-FGFR1 Rearrangement: Favorable Outcome after Haploidentical Allogeneic Transplantation.

Case Rep Hematol 2018 6;2018:5724960. Epub 2018 Dec 6.

Cytogenetic Unit, Hospital Universitario de La Princesa, Madrid, Spain.

Hematopoietic myeloproliferative neoplasms with FGFR1 rearrangement result in the 8p11 myeloproliferative syndrome that in the current Word Health Organization classification is designated as "myeloid and lymphoid neoplasm with FGFR1 abnormalities." We report the case of a 66-year-old man who had clinical features that resembled chronic myeloid leukaemia (CML), but bone marrow cytogenetic and fluorescent in situ hybridization (FISH) studies showed t(8;22)(p11;q11) and BCR-FGFR1 fusion gene. He was initially managed with hydroxyurea, and given the aggressive nature of this disease, four months later, the patient underwent an allogeneic hematopoietic stem-cell transplantation (HSCT) from an HLA-haploidentical relative. Read More

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http://dx.doi.org/10.1155/2018/5724960DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311782PMC
December 2018
2 Reads

Defective negative regulation of Toll-like receptor signaling leads to excessive TNF-α in myeloproliferative neoplasm.

Blood Adv 2019 Jan;3(2):122-131

Division of Hematology/Oncology, Department of Medicine, University of California, Irvine, Irvine, CA.

Patients with myeloproliferative neoplasms (MPN) have high levels of inflammatory cytokines, some of which drive many of the debilitating constitutional symptoms associated with the disease and may also promote expansion of the neoplastic clone. We report here that monocytes from patients with MPN have defective negative regulation of Toll-like receptor (TLR) signaling that leads to unrestrained production of the inflammatory cytokine tumor necrosis factor α (TNF-α) after TLR activation. Specifically, monocytes of patients with MPN are insensitive to the anti-inflammatory cytokine interleukin 10 (IL-10) that negatively regulates TLR-induced TNF-α production. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018026450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341195PMC
January 2019
4 Reads

Cytodiagnosis of extramedullary hematopoiesis in thyroid gland unravelling an asymptomatic hematological malignancy.

Diagn Cytopathol 2019 Jan 15. Epub 2019 Jan 15.

Department of Medicine, PGIMER, Dr. Ram Manohar Lohia Hospital, New Delhi, India.

Extramedullary hematopoeisis (EMH), also known as myeloid metaplasia can be seen in association with various hematological disorders. The common sites of EMH are liver, spleen and lymph nodes; but it can occur in almost any organ and in numerous locations. Involvement of the thyroid gland with EMH has rarely been reported. Read More

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http://dx.doi.org/10.1002/dc.24082DOI Listing
January 2019
8 Reads

Albumin binding and anticancer effect of magnesium oxide nanoparticles.

Int J Nanomedicine 2019 27;14:257-270. Epub 2018 Dec 27.

Department of Nanotechnology, Faculty of Advanced Science and Technology, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran,

Background: Recently, nanomaterials have moved into biological and medicinal implementations like cancer therapy. Therefore, before clinical trials, their binding to plasma proteins like human serum albumin (HSA) and their cytotoxic effects against normal and cancer cell lines should be addressed.

Methods: Herein, the interaction of magnesium oxide nanoparticles (MgO NPs) with HSA was studied by means of fluorescence spectroscopy, circular dichroism (CD) spectroscopy, and docking studies. Read More

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https://www.dovepress.com/albumin-binding-and-anticancer-eff
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http://dx.doi.org/10.2147/IJN.S186428DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312066PMC
February 2019
7 Reads

Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus.

BMJ Case Rep 2019 Jan 14;12(1). Epub 2019 Jan 14.

Internal Medicine and Infectious Disease Department, Centre Hospitalier Sud Ile de France, Melun, France.

Autoimmune myelofibrosis is a distinct clinicopathological entity that occurs with autoimmune disorders. We report the case of a 44-year-old woman admitted with pancytopenia and clinical features of systemic lupus erythematosus, Sjogren's syndrome and antiphospholipid antibodies syndrome. Bone marrow biopsy showed decreased global cells and an increase of reticulin fibres on argentic coloration, consistent with myelofibrosis. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22752
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http://dx.doi.org/10.1136/bcr-2018-227520DOI Listing
January 2019
7 Reads

[Differential diagnosis of hypereosinophilia].

Z Rheumatol 2019 Jan 11. Epub 2019 Jan 11.

Vaskulitiszentrum-Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus, Universität Tübingen, Kirchheim u. Teck, Deutschland.

Eosinophilia is defined as an elevated absolute number of eosinophilic leukocytes in peripheral blood or tissue. Its absolute number also defines the grade of eosinophilia. The main causes are allergic (including drug side effects) and infectious triggers but malignant and autoimmune diseases can also result in eosinophilia. Read More

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http://dx.doi.org/10.1007/s00393-018-0587-2DOI Listing
January 2019
1 Read

Cdk6 coordinates mutant MPN via NFκB and apoptotic networks.

Blood 2019 Jan 11. Epub 2019 Jan 11.

Institute of Pharmacology and Toxicology, University of Veterinary Medicine, Vienna, Austria;

Over 80% of patients with myeloproliferative neoplasms (MPNs) harbour the acquired somatic mutation. JAK inhibition is not curative and fails to induce a persistent response in most patients, illustrating the need for the development of novel therapeutic approaches. We describe a critical role for CDK6 in MPN evolution. Read More

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http://dx.doi.org/10.1182/blood-2018-08-872648DOI Listing
January 2019
3 Reads

Total body irradiation dose escalation decreases risk of progression and graft rejection after hematopoietic cell transplantation for myelodysplastic syndromes or myeloproliferative neoplasms.

Haematologica 2019 Jan 10. Epub 2019 Jan 10.

Fred Hutchinson Cancer Research Center and the University of Washington;

A nonmyeloablative regimen of fludarabine and 200 cGy total body irradiation combined with post-grafting immunosuppression with mycophenolate mofetil and a calcineurin inhibitor facilitates allogeneic hematopoietic cell transplantation from HLA-matched related or unrelated donors in older patients and/or those with comorbidities. However, outcomes of prior studies have been disappointing in patients with myelodysplastic syndromes or myeloproliferative neoplasms due to high incidences of progression or graft failure (together termed hematopoietic cell transplantation-failure). We hypothesized that escalating the total body irradiation dose may improve the outcomes and subsequently performed a phase II total body irradiation dose-escalation trial. Read More

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http://dx.doi.org/10.3324/haematol.2018.199398DOI Listing
January 2019
2 Reads

Disfiguring Nodular Cephalic Xanthoma Disseminatum: An Exceptional Variant of a Forgotten Entity.

Acta Derm Venereol 2019 01 9. Epub 2019 Jan 9.

Unit of Anatomic Pathology, Department of Molecular Medicine, IRCCS San Matteo Foundation, University of Pavia, IT-27100 Pavia, Italy.

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http://dx.doi.org/10.2340/00015555-3111DOI Listing
January 2019
2 Reads

Plerixafor for the Treatment of WHIM Syndrome.

N Engl J Med 2019 01;380(2):163-170

From the Laboratory of Molecular Immunology, National Institute of Allergy and Infectious Diseases (D.H.M., D.V., E.C., Q.L., P.M.M.), the Laboratories of Cellular Oncology (D.V.P., C.B.B.) and Pathology (S.P.), National Cancer Institute, the Department of Laboratory Medicine, Clinical Center (K.R.C.), the National Institute of Dental and Craniofacial Research (P.J.G.), and the National Institute on Deafness and Other Communication Disorders (D.A.B.), National Institutes of Health, and Kozloff and Trout MDs (H.H.T.), Bethesda, MD; the Infectious Diseases Unit and Primary Immunodeficiencies Unit, Hospital Dona Estefânia, Pediatric University Hospital (J.F.N.), and Centro de Imunodeficiências Primárias, Academic Medical Center of Lisbon (S.L.S.), Lisbon, Portugal; and the University of Chicago Medical Center, Chicago (E.A.B., E.M.L.).

WHIM syndrome (warts, hypogammaglobulinemia, infections, and myelokathexis), a primary immunodeficiency disorder involving panleukopenia, is caused by autosomal dominant gain-of-function mutations in CXC chemokine receptor 4 (CXCR4). Myelokathexis is neutropenia caused by neutrophil retention in bone marrow. Patients with WHIM syndrome are often treated with granulocyte colony-stimulating factor (G-CSF), which can increase neutrophil counts but does not affect cytopenias other than neutropenia. Read More

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http://dx.doi.org/10.1056/NEJMoa1808575DOI Listing
January 2019
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Mean platelet component and mean platelet volume as useful screening markers for myelodysplastic syndrome.

Health Sci Rep 2018 May 2;1(5):e50. Epub 2018 May 2.

Department of Central Clinical Laboratory Osaka Medical College Hospital Takatsuki Osaka Japan.

Background: Hematologic disorders, including myelodysplastic syndrome (MDS), are difficult to identify in routine hematologic examinations using automated hematology analyzers. However, the practical uses of mean platelet component and mean platelet volume (MPV) measured by these analyzers as screening markers for MDS, remain unclear.

Methods: Mean platelet component and MPV values were measured in the peripheral blood of patients with MDS, aplastic anemia, idiopathic thrombocytopenic purpura, myeloproliferative neoplasms, and in healthy controls using an automated hematologic analyzer. Read More

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http://dx.doi.org/10.1002/hsr2.50DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6266360PMC
May 2018
3 Reads

Cutaneous mastocytosis treatment: strategies, limitations and perspectives.

Postepy Dermatol Alergol 2018 Dec 13;35(6):541-545. Epub 2018 Aug 13.

Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland.

Mastocytosis is a rare myeloproliferative disease, characterized by excessive proliferation and accumulation of mast cells in the tissues. In cutaneous mastocytosis (CM), mast cells infiltration is limited to the skin, whereas in systemic mastocytosis (SM) internal organs are involved. The first-line treatment in CM is antimediator therapy (mainly H1 and H2 antihistamines) and short-term topical corticosteroids. Read More

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http://dx.doi.org/10.5114/ada.2018.77605DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320483PMC
December 2018
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Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis: "2019 Update on Diagnosis, Risk-stratification, and Management".

Am J Hematol 2019 Jan 7. Epub 2019 Jan 7.

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thrombocytosis (RARS-T); now called myelodysplastic/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T).

Diagnosis: MDS-RS is a lower-risk MDS, with single or multilineage dysplasia (SLD/MLD), <5% bone marrow (BM) blasts and ≥ 15% BM RS (≥5% in the presence of SF3B1 mutations). Read More

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http://doi.wiley.com/10.1002/ajh.25397
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http://dx.doi.org/10.1002/ajh.25397DOI Listing
January 2019
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Understanding the structural features of JAK2 inhibitors: a combined 3D-QSAR, DFT and molecular dynamics study.

Mol Divers 2019 Jan 7. Epub 2019 Jan 7.

Computational Biology Lab, Department of Genetic Engineering, School of Bioengineering, SRM Institute of Science and Technology, SRM Nagar, Kattankulathur, Chennai, 603203, India.

JAK2 plays a critical role in JAK/STAT signaling pathway and in patho-mechanism of myeloproliferative disorders and autoimmune diseases. Thus, effective JAK2 inhibitors provide a promising opportunity for the pharmaceutical intervention of many diseases. In this work, 3D-QSAR study was performed on a series of 1-amino-5H-pyrido-indole-4-carboxamide derivatives as JAK2 inhibitors to obtain reliable comparative molecular field analysis (CoMFA) and comparative molecular similarity analysis (CoMSIA) models with three different alignment methods. Read More

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http://dx.doi.org/10.1007/s11030-018-09913-4DOI Listing
January 2019
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Splenic irradiation before allogeneic stem cell transplantation for myelofibrosis.

Med Oncol 2019 Jan 8;36(2):16. Epub 2019 Jan 8.

Department of Hematology and Bone Marrow Transplantation, School of Medicine in Katowice, Medical University of Silesia, Dąbrowski Street 25, 40-032, Katowice, Poland.

Splenectomy before allogeneic stem cell transplantation (ASCT) for patients with myelofibrosis (MF) remains a matter of debate, and conflicting results have been reported to date. The procedure seems to fasten post-transplant hematological recovery, but it does not have an impact on survival. The role of pre-transplant splenic irradiation (SI) is much more difficult to evaluate. Read More

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http://dx.doi.org/10.1007/s12032-019-1245-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326000PMC
January 2019
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[Monitoring the chronic myeloid leukemia patients between 2008 and 2018; the experience of the Hematology and Bone Marrow Transplantation Unit Târgu-Mureș].

Orv Hetil 2019 Jan;160(2):67-72

Hematológiai és Csontvelő-átültetési Klinika, Marosvásárhelyi Orvosi és Gyógyszerészeti Egyetem Târgu Mureș, Str. Revoluției nr. 35, Jud. Mureș, cod 540042 Romania.

Introduction And Aim: Chronic myeloid leukemia is a clonal myeloproliferative disorder characterized by the BCR-ABL gene rearrangement with translocation between chromosomes 9 and 22. The constitutively active BCR-ABL tyrosine kinase inhibitor became the standard frontline therapy. The molecular monitoring is essential. Read More

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https://www.akademiai.com/doi/10.1556/650.2019.31250
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http://dx.doi.org/10.1556/650.2019.31250DOI Listing
January 2019
6 Reads

WT1 gene is overexpressed in myeloproliferative neoplasms, especially in myelofibrosis.

Blood Cells Mol Dis 2019 Mar 20;75:35-40. Epub 2018 Dec 20.

Université Angers, UFR Santé, Angers, France; CHU Angers, Laboratoire d'Hématologie, Angers, France; INSERM, CRCINA, Université de Nantes, Université d'Angers, France; Fédération Hospitalo-Universitaire 'Grand Ouest Against Leukemia' (FHU GOAL), France. Electronic address:

Classical Philadelphia-negative myeloproliferative neoplasms include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF). They are characterized by the presence of driver mutations of JAK2, CALR or MPL genes. Overexpression of WT1 is used as a marker of minimal residual disease in acute myeloid leukemia, especially after allogeneic stem cell transplantation (SCT). Read More

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http://dx.doi.org/10.1016/j.bcmd.2018.12.004DOI Listing
March 2019
3 Reads