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Primary myelofibrosis

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https://www.readbyqxmd.com/read/28715625/-myelofibrosis-in-a-benzene-exposed-cleaning-worker
#1
Roser Bausà, Lydia Navarro, Imma Cortès-Franch
Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related...
July 2017: Archivos de Prevención de Riesgos Laborales
https://www.readbyqxmd.com/read/28710830/prognostic-significance-of-a-comprehensive-histologic-evaluation-of-reticulin-fibrosis-collagen-deposition-and-osteosclerosis-in-primary-myelofibrosis-patients
#2
Umberto Gianelli, Stefano Fiori, Daniele Cattaneo, Anna Bossi, Ivan Cortinovis, Arturo Bonometti, Giulia Ercoli, Cristina Bucelli, Nicola Orofino, Gaetano Bulfamante, Alessandra Iurlo
AIMS: to evaluate whether a comprehensive histological evaluation of reticulin fibrosis, collagen deposition and osteosclerosis in bone marrow trephine biopsies (BMBs) of primary myelofibrosis (PMF) patients may have prognostic implications. METHODS: reticulin fibrosis, collagen deposition and osteosclerosis were graded from 0 to 3 in a series of 122 base-line BMBs. Then, we assigned to each case a comprehensive score (RCO score, ranging from 0 to 9) that allowed us to distinguish two groups of patients, with low-grade (RCO score 0-4) and high-grade (RCO score 5-9) stromal changes...
July 15, 2017: Histopathology
https://www.readbyqxmd.com/read/28700486/progression-of-primary-myelofibrosis-to-polycythemia-vera-a-case-report
#3
Yan Guo, Wenwei Xu, Lin Dong, Ning Huang, Kehong Bi
RATIONALE: This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms. PATIENT CONCERNS: The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months. DIAGNOSIS: A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JAK2 V617F mutation was positive before and after the diagnosis of PV; however, new chromosomal abnormalities were detected during the progression...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28699671/primary-myelofibrosis-presenting-in-acute-promyelocytic-transformation
#4
Joyce H Y Kwong, Rock Y Y Leung, Michael L G Wong, Harinder Gill, Yok-Lam Kwong
No abstract text is available yet for this article.
July 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28690072/identity-of-gli1-cells-in-the-bone-marrow
#5
REVIEW
Isadora Sena, Pedro Prazeres, Gabryella Santos, Isabella Borges, Patrick Azevedo, Julia Andreotti, Viviani Almeida, Ana Paiva, Daniel Guerra, Luiza Lousado, Luanny Souto, Akiva Mintz, Alexander Birbrair
Bone marrow fibrosis is a critical component of primary myelofibrosis in which normal bone marrow tissue and blood-forming cells are gradually replaced with scar tissue. The specific cellular and molecular mechanisms that cause bone marrow fibrosis are not understood. A recent study by using state-of-the-art techniques including in vivo lineage-tracing provides evidence that Gli1+ cells are the cells responsible for fibrotic disease in the bone marrow. Strikingly, genetic depletion of Gli1+ cells rescues bone marrow failure and abolishes myelofibrosis...
July 6, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28688466/leukemic-transformation-in-myeloproliferative-neoplasms-a-literature-review-on-risk-characteristics-and-outcome
#6
REVIEW
Meera Yogarajah, Ayalew Tefferi
Myeloproliferative neoplasms (MPNs) operationally include essential thrombocythemia, polycythemia vera, primary myelofibrosis (PMF), and prefibrotic PMF. All 4 MPN variants might progress into blast-phase disease (MPN-BP). For essential thrombocythemia, reported risk factors for leukemic transformation include advanced age, extreme thrombocytosis, anemia, leukocytosis, and sequence variants/mutations involving TP53 and EZH2 (for expansion of gene symbols, see www.genenames.org); for polycythemia vera, advanced age, leukocytosis, abnormal karyotype, mutations involving SRSF2 and IDH2, and treatment with pipobroman, chlorambucil, or P32; and for PMF, increased blast percentage, thrombocytopenia, abnormal karyotype, triple-negative driver mutational status, and sequence variants/mutations involving SRSF2, RUNX1, CEBPA, and SH2B3...
July 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#7
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28681787/intracranial-extramedullary-hematopoiesis-in-primary-myelofibrosis
#8
Ravikanth Reddy
No abstract text is available yet for this article.
July 2017: Neurology India
https://www.readbyqxmd.com/read/28680586/pulmonary-extra-medullary-hematopoiesis-and-pulmonary-hypertension-from-underlying-polycythemia-vera-a-case-series
#9
Inderjit Singh, Geoffrey Mikita, Daniel Green, Cristobal Risquez, Abraham Sanders
Myeloproliferative neoplasia (MPN)-associated pulmonary hypertension (PH) is included in group five of the most recent clinical classification of PH.(1) The MPNs are a heterogeneous group of disorders that includes disorders with primary expression of a myeloid phenotype and disorders characterized by expression of the Janus Kinase 2 (JAK2) mutation, p.V617F. The latter includes essential thrombocytosis, polycythemia vera, and idiopathic myelofibrosis.(2) Pulmonary extra-medullary hematopoiesis (EMH) refers to the presence of hematopoietic precursor cells in the lung...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28676668/the-calreticulin-calr-exon-9-mutations-are-promising-targets-for-cancer-immune-therapy
#10
M O Holmström, E Martinenaite, S M Ahmad, Ö Met, C Friese, L Kjær, C H Riley, P Thor Straten, I M Svane, H C Hasselbalch, M H Andersen
The CALR exon 9 mutations are found in approximately 30% of patients with essential thrombocythemia and primary myelofibrosis. Recently, we reported spontaneous immune responses against the CALR mutations. Here, we describe that CALR-mutant (CALRmut)-specific T-cells are able to specifically recognize CALRmut cells. First, we established a T-cell culture specific for a CALRmut epitope. These specific T-cells were able to recognize several epitopes in the CALRmut C-terminus. Next, we established a CALRmut specific CD4(+) T-cell clone by limiting dilution...
July 5, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28667351/efficacy-and-safety-of-eltrombopag-in-persistent-and-newly-diagnosed-itp-in-clinical-practice
#11
Tomás José González-López, Fernando Fernández-Fuertes, José Angel Hernández-Rivas, Blanca Sánchez-González, Violeta Martínez-Robles, María Teresa Alvarez-Román, Gloria Pérez-Rus, Cristina Pascual, Silvia Bernat, Esther Arrieta-Cerdán, Carlos Aguilar, Abelardo Bárez, María Jesús Peñarrubia, Pavel Olivera, Angeles Fernández-Rodríguez, Erik de Cabo, Luis Javier García-Frade, José Ramón González-Porras
Eltrombopag is safe and effective in primary chronic ITP. However, lack of clinical trials avoids a clear demonstration of its utility in newly diagnosed and persistent ITP. Our aim here is to report Spanish results for this type of patients. We retrospectively evaluated 220 adult primary ITP patients. According to standard definition, patients were allocated to newly diagnosed (n = 30), persistent (n = 30), and chronic (n = 160) ITP. Groups were homogenous regarding most relevant parameters. 180 (90%) of 220 patients achieved a platelet response (R) with 167 (75...
June 30, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28660085/dermatomyositis-associated-with-myelofibrosis-following-polycythemia-vera
#12
Naomi Fei, Sarah Sofka
Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28628689/aggressive-skin-cancers-occurring-in-patients-treated-with-the-janus-kinase-inhibitor-ruxolitinib
#13
Adam B Blechman, Christine E Cabell, Christine H Weinberger, Anna Duckworth, Justin J Leitenberger, Fiona O Zwald, Mark A Russell
<p>The Food and Drug Administration approved Ruxolitinib in 2011 for the treatment of primary myelofibrosis. Five-year safety data showed a higher incidence of skin cancer in patients treated with Ruxolitinib compared to best available therapy for myelofibrosis. This report presents a series of five patients with history of myelofibrosis treated with Ruxolitinib who subsequently developed numerous skin cancers with aggressive biological behavior. Each patient in this report was treated by a Mohs surgeon affiliated with an academic institution...
May 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28622624/primary-myelofibrosis-older-age-and-high-jak2v617f-allele-burden-are-associated-with-elevated-plasma-high-sensitivity-c-reactive-protein-levels-and-a-phenotype-of-progressive-disease
#14
Giovanni Barosi, Margherita Massa, Rita Campanelli, Gabriela Fois, Paolo Catarsi, Gianluca Viarengo, Laura Villani, Valentina Poletto, Tiziana Bosoni, Umberto Magrini, Robert P Gale, Vittorio Rosti
We measured plasma levels of high-sensitivity C-reactive protein (hs-CRP) in 526 subjects with primary myelofibrosis (PMF). Thirty-eight percent had an elevated hs-CRP level (≥0.3mg/dL). Elevated hs-CRP levels were associated with a progressive disease phenotype, including anemia, high white blood cell count, low platelet count, increased splenomegaly, increased risk of blast transformation, and worse survival. Age≥52years, but no other demographic characteristics, was associated with an elevated hs-CRP level in multivariable logistic regression (odds ratio [OR], 4...
June 7, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28622623/a-phase-2-study-of-momelotinib-a-potent-jak1-and-jak2-inhibitor-in-patients-with-polycythemia-vera-or-essential-thrombocythemia
#15
Srdan Verstovsek, Stephane Courby, Martin Griesshammer, Ruben A Mesa, Carrie Baker Brachmann, Jun Kawashima, Julia D Maltzman, Lixin Shao, Yan Xin, Daniel Huang, Ashish Bajel
Momelotinib is a potent inhibitor of JAK1 and JAK2 that demonstrated efficacy in patients with primary and secondary myelofibrosis. This phase 2, open-label, randomized study evaluated the efficacy and safety of oral once-daily momelotinib (100mg and 200mg) for the treatment of polycythemia vera (PV) and essential thrombocythemia (ET). The primary endpoint for PV was overall response rate (ORR), defined as the proportion of patients with hematocrit <45%, white blood cell count <10×10(9)/L, platelet count ≤400×10(9)/L, and resolution of palpable splenomegaly, each lasting ≥4 weeks...
May 30, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28615140/imaging-features-of-myeloproliferative-neoplasms
#16
REVIEW
I G Murphy, E L Mitchell, L Raso-Barnett, A L Godfrey, E M Godfrey
Myeloproliferative neoplasms (MPNs) are a heterogeneous group of haematological disorders including polycythaemia vera (PV), essential thrombocythaemia (ET), primary myelofibrosis (PMF), and chronic myeloid leukaemia (CML). These disorders show large overlap in genetic and clinical presentations, and can have many different imaging manifestations. Unusual thromboses, embolic events throughout the systemic or pulmonary vasculature, or osseous findings can often be clues to the underlying disease. There is limited literature about the imaging features of these disorders, and this may result in under-diagnosis...
June 11, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28603913/a-common-complication-of-myelofibrosis-presenting-as-a-rare-finding-in-cerebrospinal-fluid-cytology
#17
Roberto Ruiz-Cordero, Jeffrey L Jorgensen, Savitri Krishnamurthy, Gene Landon
Herein, we present a rare case of intracranial extramedullary hematopoiesis (EMH) diagnosed by cerebrospinal fluid (CSF) cytology and describe the clinical presentation, radiologic, and pathologic findings. A 65 year-old man with a history of progressing primary myelofibrosis was admitted for headaches and right facial numbness. A brain MRI revealed focal abnormalities that were suspicious for leptomeningeal involvement of acute leukemia. Cytologic examination of CSF demonstrated a hypercellular specimen composed of hematopoietic cells including few blasts, as well as maturing red blood cells and granulocytic cells...
June 11, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28602585/janus-kinase-2-inhibitor-fedratinib-in-patients-with-myelofibrosis-previously-treated-with-ruxolitinib-jakarta-2-a-single-arm-open-label-non-randomised-phase-2-multicentre-study
#18
Claire N Harrison, Nicolaas Schaap, Alessandro M Vannucchi, Jean-Jacques Kiladjian, Ramon V Tiu, Pierre Zachee, Eric Jourdan, Elliott Winton, Richard T Silver, Harry C Schouten, Francesco Passamonti, Sonja Zweegman, Moshe Talpaz, Joanne Lager, Zhenming Shun, Ruben A Mesa
BACKGROUND: Myelofibrosis is a chronic myeloproliferative neoplasm characterised by splenomegaly, cytopenias, bone marrow fibrosis, and debilitating symptoms including fatigue, weight loss, and bone pain. Mutations in Janus kinase-2 (JAK2) occur in approximately 50% of patients. The only approved JAK2 inhibitor for myelofibrosis is the dual JAK1 and JAK2 inhibitor, ruxolitinib. 58-71% of patients treated with ruxolitinib in clinical trials so far have not achieved the primary endpoint of 35% or more reduction in spleen volume from baseline assessed by MRI or CT...
July 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28601551/patients-with-post-essential-thrombocythemia-and-post-polycythemia-vera-differ-from-patients-with-primary-myelofibrosis
#19
Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Kate J Newberry, Taghi Manshouri, Jorge Cortes, Hagop M Kantarjian, Srdan Verstovsek
Prognostic scoring systems for primary myelofibrosis (PMF) are not accurate in patients with post-essential thrombocythemia and post-polycythemia vera myelofibrosis (PET-MF; PPV-MF). Given the paucity of data describing the clinical characteristics, disease course and outcomes of these patients, we sought to describe and compare the clinical characteristics and outcomes of 755 patients with PMF, 181 with PPV-MF, and 163 with PET-MF referred to our institution between 1984 and 2013. The median follow-up was 31 months, and 56% (n=616) patients had died...
June 2, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28592432/up-regulation-of-lysyl-oxidase-and-adhesion-to-collagen-of-human-megakaryocytes-and-platelets-in-primary-myelofibrosis
#20
Vittorio Abbonante, Vipul Chitalia, Vittorio Rosti, Orly Leiva, Shinobu Matsuura, Alessandra Balduini, Katya Ravid
No abstract text is available yet for this article.
June 7, 2017: Blood
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