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Polycytemia vera

S Ruberti, E Bianchi, P Guglielmelli, S Rontauroli, G Barbieri, L Tavernari, T Fanelli, R Norfo, V Pennucci, G Corbizi Fattori, C Mannarelli, N Bartalucci, B Mora, L Elli, M A Avanzini, C Rossi, S Salmoiraghi, R Zini, S Salati, Z Prudente, V Rosti, F Passamonti, A Rambaldi, S Ferrari, E Tagliafico, A M Vannucchi, R Manfredini
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hyperplastic megakaryopoiesis and myelofibrosis. We recently described the upregulation of MAF (v-maf avian musculoaponeurotic fibrosarcoma oncogene homolog) in PMF CD34+ hematopoietic progenitor cells (HPCs) compared to healthy donor. Here we demonstrated that MAF is also upregulated in PMF compared with the essential thrombocytemia (ET) and polycytemia vera (PV) HPCs. MAF overexpression and knockdown experiments shed some light into the role of MAF in PMF pathogenesis, by demonstrating that MAF favors the megakaryocyte and monocyte/macrophage commitment of HPCs and leads to the increased expression of proinflammatory and profibrotic mediators...
February 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Massimo Cajozzo, Vincenzo Davide Palumbo, Salvatore Buscemi, Giuseppe Damiano, Ada Maria Florena, Daniela Cabibi, Francesco Raffaele, Antonino Alessio Anzalone, Federica Fatica, Gerlando Cocchiara, Salvatore Dioguardi, Antonio Bruno, Francesco Paolo Caronia, Attilio Ignazio Lo Monte
BACKGROUND: Plasmoblastic lymphoma is a rare and aggressive subtype of diffuse large B cell lymphoma, which occurs usually in the jaw of immunocompromised subjects. CASE PRESENTATION: We describe the occurrence of plasmoblastic lymphoma in the mediastinum and chest wall skin of an human immunodeficiency virus-negative 63-year-old Caucasian man who had had polycytemia vera 7 years before. At admission, the patient showed a superior vena cava syndrome, with persistent dyspnoea, cough, and distension of the jugular veins...
March 21, 2017: Journal of Medical Case Reports
Roberto Castelli, Massimo Cugno, Umberto Gianelli, Alessandro Pancrazzi, Alessandro Maria Vannucchi
BACKGROUND: In a small subset of polycytemia vera (PV), neutrophilia not secondary to reactive conditions or treatment can develop and persist. Clinical significance and morphogenetic alterations associated with this uncommon phenomenon are not well defined. CASE REPORT: An 81-year-old Caucasian woman, affected by polycytemia vera lasting 17 years, presented in March 2012 with hyperleukocytosis, absolute neutrophilia, and thrombocytosis despite hydroxyurea treatment...
April 2015: Pathology, Research and Practice
Miguel Gallardo, Santiago Barrio, Marisol Fernandez, Alberto Paradela, Alicia Arenas, Oscar Toldos, Rosa Ayala, Enriqueta Albizua, Ana Jimenez, Santiago Redondo, Rosa Maria Garcia-Martin, Florinda Gilsanz, Juan Pablo Albar, Joaquin Martinez-Lopez
JAK-STAT signaling through the JAK2V617F mutation is central to the pathogenesis of myeloproliferative neoplasms (MPN). However, other events could precede the JAK2 mutation. The aim of this study is to analyze the phenotypic divergence between polycytemia vera (PV) and essential thrombocytemia (ET) to find novel therapeutics targets by a proteomic and functional approach to identify alternative routes to JAK2 activation. Through 2D-DIGE and mass spectrometry of granulocyte protein from 20 MPN samples, showed differential expression of HSP70 in PV and ET besides other 60 proteins...
November 19, 2013: Molecular Cancer
J-J Lehot, B Was, L Dendeleu, O Jegaden
A 55-year-old male with a history of positive HIV serology and polycythemia vera underwent coronary artery bypass graft surgery with normothermic extracorporeal circulation. Following heparin administration the activated clotting time (ACT) was 633 seconds (Hemocron with kaolin). Lower than expected arterial and venous oxygen partial pressures together with high pressure (350 mmHg) in the arterial line upstream of the oxygenator were observed. Because of these signs the oxygenator was changed during the procedure...
May 2012: Annales Françaises D'anesthèsie et de Rèanimation
I Ben Ghorbel, T Ben Salem, M Lamloum, M Khanfir, A Braham, M Miled, N Ben Romdhane, M H Houman
Neurological manifestations in polycytemia vera are common. However, chorea is an exceptionally revealing feature of this disease. We report a 78-year-old man who presented with headache and an abnormal movement disorder corresponding to chorea. Laboratory findings showed increased levels of hemoglobin at 20 g/dl and hematocrit at 62.3%. An elevated erythrocyte mass to twice the normal value demonstrated the absolute erythrocytosis. A JAK2 V617F gene mutation was identified. A diagnosis of polycytemia vera-associated chorea was obtained...
June 2011: La Revue de Médecine Interne
Salvatore Lentini, Mario Barone, Filippo Benedetto, Francesco Spinelli
Polycythemia vera is a myeloproliferative disorder characterized by thrombotic complications both in the arterial and venous systems. We report the case of a 55-year-old patient affected by polycythemia vera, presenting with acute superior vena cava syndrome due to thrombosis of the upper part of the superior vena cava. Diagnosis was done clinically and by computed tomography scan and showed an unusual finding: an air bubble trapped in the brachiocephalic venous trunk. The patient underwent emergency surgery...
2010: Cardiology Research and Practice
Claudia Buzas, Zeno Sparchez, Andrei Cucuianu, Simona Manole, Ioana Lupescu, Monica Acalovschi
Budd-Chiari syndrome still represents a challenge for the hepatologist with regard to its causes and its most effective therapy. Polycythemia vera is considered to be the most frequent condition causing the Budd-Chiari syndrome (10-40% of cases). We present a 34-year-old patient in post-partum who was admitted for right upper abdominal quadrant pain and asthenia. Laboratory data, abdominal echography and angioMRI all raised the suspicion of BCS, but it was in the haematological department that polycytemia vera was diagnosed as the cause of the hepatic condition...
September 2009: Journal of Gastrointestinal and Liver Diseases: JGLD
Magnus Tobiasson, Bassam Alyass, Susanne Söderlund, Gunnar Birgegård
In order to examine whether symptoms of iron deficiency anemia are due to the iron deficiency itself or the associated anemia, 34 patients with polycytemia vera (PV) treated with venesectio, who had iron deficiency but normal hemoglobin (Hb) levels, were given a questionnaire covering symptoms of iron deficiency including the international RLS-scale and the Fact-fatigue quality of life scale (QoL). We found a prevalence of pica of 11.7%, mouth paresthesias of 5.8% and rest-less legs 29.6% (RLS "normal" prevalence 10%)...
March 2010: Medical Oncology
V Tutaeva, A V Misurin, J J Michiels, J M Rozenberg, M A Sokolova, V L Ivanova, T I Kolosheinova, T E Manakova, A A Levina, E A Semenova, N D Khoroshko
Increased PRV-1 mRNA expression and the presence of Jak2(V617F) mutation in peripheral blood granulocytes are specific markers for chronic myeloproliferative disorders (MPD), which facilitate the differential diagnosis between polycythemia vera (PV) and secondary erythrocytosis (SE) and may be helpful for monitoring treatment efficacy in MPD patients. We evaluated the presence of the Jak2V617F mutation and increased PRV-1 mRNA expression along with previously established markers - erythropoietin (EPO) independent colony formation (EEC) and erythropoietin level for diagnosis of PV and assessment of treatment efficiency...
December 2007: Hematology (Amsterdam, Netherlands)
Naoyuki Sata, Yasuhiro Tanaka, Katsunori Toufuku, Katsurou Kashima, Kenkichi Miyahara
No abstract text is available yet for this article.
July 2005: Journal of Cardiology
Oscar Norbeck, Thomas Tolfvenstam, Laurence E Shields, Magnus Westgren, Kristina Broliden
OBJECTIVE: To evaluate the capacity of parvovirus B19 capsid protein VP2 to inhibit hematopoiesis in vitro and in vivo. If effective, a VP2-derived construct may have therapeutic and prophylactic utility in diseases associated to overproduction of hematopoietic cells. METHODS: The effect on hematopoiesis in vitro of recombinant VP2, intact and enzymatically fragmented, was evaluated in a colony formation assay, using cells from fetal liver and macaque bone marrow...
November 2004: Experimental Hematology
J-J Lehot, B Waz, L Dendeleu, P Gaudon, O Jegaden
A 55-year-old male with a history of positive HIV serology and Polycytemia vera underwent coronary artery bypass graft surgery with normothermic extracorporeal circulation. Following heparin administration the activated clotting time (ACT) was 633 seconds (Hemocron) with kaolin). Lower than expected arterial and venous oxygen partial pressures together with high pressure (350 mmHg) in the arterial line upstream of the oxygenator were observed. Because of these signs the oxygenator was changed during the procedure...
March 2004: Annales Françaises D'anesthèsie et de Rèanimation
No abstract text is available yet for this article.
October 1, 1952: Klinische Wochenschrift
G W Ickenstein, J M Klotz, H D Langohr
UNLABELLED: As polycythemia vera is a myeloproliferative syndrome, it is based on an autonomic increase in the proliferation of all hematopoetic cells--mostly of erythropoesis. An increase in blood viscosity induces disturbed microcirculation, resulting in headaches with clinical symptoms. So far, the headache in polycythemia vera has not been classified. METHODS: We would like to demonstrate a case of a female patient aged 58 years who had been complaining of persistent hemicranial right-sided headaches for the last 16 days, alternating between throbbing and stabbing pain as well as a retrobulbar feeling of pressure...
August 19, 1999: Der Schmerz
C V Vignal, D M Lourenço, M A Noguti, M de L Chauffaille, J Kerbauy
OBJECTIVE: To correlate the incidence of hemorrhage and thrombosis to bleeding time (BT) and platelet aggregation in 27 consecutive patients with myeloproliferative diseases (MPD). DESIGN: Retrospective study. SETTING: Public tertiary referral center. PATIENTS: Eighteen patients with chronic myelogenous leukemia (CML), 5 with polycytemia vera (PV), 2 with essential thrombocytemia (ET) and 2 with idiopathic myelofibrosis (MF)...
November 1997: São Paulo Medical Journal, Revista Paulista de Medicina
S Shiga, T Furukawa, I Koyanagi, M Yamagishi, Y Yoshida, T Takahashi, R Kannagi, T Mori
To establish a simple computer program for the laboratory diagnosis of anemia and related diseases, multivariate analyses were applied to the results of routine hematological laboratory tests obtained from 48 patients and 51 healthy volunteers. The patients studied were limited to those who had not been treated hematologically by the time of their first visit to our hospital, and their first data obtained in our laboratory were analyzed. Final diagnoses were aplastic anemia (AA) in 21, myelodysplastic syndrome (MDS) in 14, iron deficiency anemia (IDA) in 3, polycytemia vera (PV)in 3, and idiopathic thrombocytopenic purpura (ITP) in 7...
February 1997: American Journal of Hematology
K Nagy, K Hunyadi, I Fehér, J Tamáska, J Zsíros, R Simkó, I Vámosi
The case of an 11 year old girl with three line type of polycythaemia vera, with 4 cm splenomegaly and a plethoric complexion is presented. Peripheral blood values were as follows: RBC: 7.75 x 10(12)/l, Hb: 18.8 g/l, WBC: 15.2 x 10(9)/l, platelets: 920 x 10(9)/l. Serum erythropoietin level: < 1 mU/ml. In vitro, erythroid colonies developed from the bone marrow in the absence of added erythropoetin. For three years the haematocrit value has been controlled by regular venesections. Since extreme thrombocytosis developed, the treatment was continued with interferon alpha...
January 7, 1996: Orvosi Hetilap
M L Randi, C Rossi, F Fabris, P Zerbinati, A Girolami
BACKGROUND: Thrombocytosis can be present in patients with myeloproliferative disorders or can accompany various conditions, in particular chronic inflammatory diseases, namely chronic bowel diseases, rheumatoid arthritis, and nephritis. OBJECTIVE: We report our experience in 55 patients younger than 45 years of age with increased platelet counts (over 500 X 10(9)/L). Thirty-three were affected by essential thrombocytemia in agreement with polycytemia vera study group criteria and 22 by reactive thrombocytosis...
December 1995: Annals of Allergy, Asthma & Immunology
E Ponte, G Mustacchi
No abstract text is available yet for this article.
June 9, 1972: Minerva Medica
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