scholarly journals The polycythemia vera stem cell

2014 ◽  
Vol 3 (S1) ◽  
pp. S23-S24 ◽  
Author(s):  
J L Spivak
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera

scholarly journals Stem cell transplantation for polycythemia vera

2008 ◽  
Vol 50 (1) ◽  
pp. 124-126 ◽  
Author(s):  
Harald Reinhard ◽  
Thomas Klingebiel ◽  
Peter Lang ◽  
Peter Bader ◽  
Dietrich Niethammer ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Polycythemia Vera ◽  
Cell Transplantation

Curative Therapy of Advanced Essential Thrombocythemia or Polycythemia Vera by Hemopoietic Stem Cell Transplantation

2002 ◽  
Vol 43 (7) ◽  
pp. 1409-1414 ◽  
Author(s):  
Uwe Platzbecker ◽  
Ted Gooley ◽  
Claudio Anasetti ◽  
Frederick R. Appelbaum ◽  
Bruce Clurman ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Polycythemia Vera ◽  
Cell Transplantation ◽  
Essential Thrombocythemia ◽  
Hemopoietic Stem Cell ◽  
Curative Therapy ◽  
Hemopoietic Stem Cell Transplantation

scholarly journals JAK2 Negative Polycythemia Vera

2010 ◽  
Vol 2 (02) ◽  
pp. 114-116 ◽  
Author(s):  
Geetha J P. ◽  
Arathi C A. ◽  
Shalini M. ◽  
Srinivasa Murthy A G.
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera ◽  
Intracellular Signaling ◽  
Janus Kinase ◽  
Jak2 Mutation ◽  
Myeloproliferative Diseases ◽  
British Committee ◽  
Clonal Mutation ◽  
Serum Erythropoietin ◽  
Cell Disorder

ABSTRACTPolycythemia vera (PV) is a stem cell disorder, characterized as a panhyperplastic, malignant, and neoplastic marrow disorder. Several reasons suggest that a mutation on the Janus kinase-2 gene (JAK2) is the most probable candidate gene involved in PV pathogenesis, as JAK2 is directly involved in intracellular signaling, following its exposure to cytokines, to which PV progenitor cells display hypersensitivity. A recurrent unique acquired clonal mutation in JAK2 was found in most patients with PV and other myeloproliferative diseases (MPDs). A female patient of age 50 years, presented with hemiplegia, diplopia, and had a consistent rise in hemoglobin and hematocrit. Serum Erythropoietin (Epo) was decreased. JAK2 mutation analysis was found to be negative. A diagnosis of polycythemia vera was made on the basis of the British Committee for Standards in Hematology (BCSH) guidelines.

scholarly journals Polycythemia

2020 ◽  
pp. 38-43
Author(s):  
Stanley Kim ◽  
Ricardo Saca ◽  
Pamela Harford
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera ◽  
Myeloproliferative Neoplasm ◽  
Hematocrit Level ◽  
Cell Disease ◽  
Erythroid Progenitors ◽  
Cell Numbers ◽  
Erythropoietin Level ◽  
Secondary Polycythemia ◽  
Rbc Count

Polycythemia is a disease state in which the red blood cell numbers are increased in the blood (erythrocytosis), which in turn makes blood thicker and can cause circulatory problems. Polycythemia Vera is a stem cell disease belonged to a group of myeloproliferative neoplasm in which the erythroid progenitors are overly proliferated by acquired mutation of the JAK2 gene, resulting in excessive erythrocytosis. Secondary Polycythemia refers erythrocytosis due to underlying conditions. It is usually associated with increased blood erythropoietin levels as a compensatory reaction to tissue hypoxia, which can be seen in patients with chronic lung disease or sleep apnea or living at high altitudes. Certain tumors produce the erythropoiet ©Win and testosterestern University of Heone incralth Scienceseases the blood erythropoietin level, resulting in secondary polycythemia. Relative polycythemia is the consequence of plasma volume contraction, falsely raising the RBC count and hemoglobin/hematocrit level in CBC. Two cases of polycythemia are presented: 1) a patient with polycythemia vera and 2) a patient with secondary polycythemia. Various types of polycythemia are discussed with an updated review covering the etiology, clinical manifestation, diagnostic approach and treatment.

Long-Term Survival Data in 652 Patients with Primary Myelofibrosis or Polycythemia Vera— Trends in Recent Years.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2910-2910
Author(s):  
Rakhee Vaidya ◽  
Naseema Gangat ◽  
Sergio Siragusa ◽  
Susan Schwager ◽  
Curtis A. Hanson ◽  
...  
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera ◽  
Median Survival ◽  
Survival Data ◽  
Leukocyte Count ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
Significant Difference

Abstract Abstract 2910 Poster Board II-886 Background: Polycythemia vera (PV) and primary myelofibrosis (PMF) are stem cell-derived myeloproliferative neoplasms characterized clinically by increased red cell volume and bone marrow fibrosis, respectively. Phlebotomy is the cornerstone of treatment in PV whereas hydroxyurea has been the traditional drug of choice in both PV and PMF. Over the last 20 years, several new treatment approaches have been introduced and promoted (e.g. interferon-alpha, anagrelide, thalidomide, allogeneic stem cell transplantation) but their benefit in terms of survival has not been confirmed in controlled studies. Methods: Study patients were recruited form the Mayo Clinic database for myeloproliferative neoplasms. Diagnosis was based on 2001 WHO criteria. Follow-up information was updated in July, 2009. In order to assess changes in survival over time, patients were stratified into two groups by year-of-diagnosis: 1970-1990 and 1991-2005. Survival analysis was performed by the Kaplan-Meier method and comparisons made by the log-rank test. Cox regression model was used for multivariable analysis. Results: 652 patients were studied, including 314 with PMF (median age 57 years, range 18-88; 193 males) and 338 with PV (median age 63 years, range 18-93; 183 males). Overall median survivals were 6.5 years for PMF and 12.2 years for PV (p<0.0001); ten- and twenty-year survival rates were 37% and 13% in PMF and 60% and 18% in PV (Figure). Both the International Prognostic Scoring System for PMF (IPSS; Cervantes et al. Blood 2009;113:2895) and the age/leukocyte count-based PSS for PV (Gangat et al. BJH 2007;138:354) were validated in the current series of patients. Considering all patients, there was no significant difference in survival when either PMF (p=0.91) or PV (p=0.73) patients were stratified according to year-of-diagnosis (Figure); median survivals were 4.7 and 6.6 years in PMF patients diagnosed 1970-1990 (n=54) and 1991-2005 (n=260), respectively; the corresponding median survival figures for PV were 11.8 (n=170) and 12.2 (n=168) years. These results did not change when adjusted for age and sex and, in PV, for age/leukocyte count-based PSS. In PMF, however, borderline significance (p=0.05) was noted when IPSS scores were introduced into the Cox model with further analysis showing improved survival in Int-2/high risk patients with PMF diagnosed after 1990 (p=0.01). Conclusion: The current study provides mature survival data in PMF and PV using large cohorts of consecutive patients seen at a single institution. Although median survival is significantly better in PV compared to PMF, their survival curves converge after 20 years of follow-up, underscoring the poor long-term prognosis in PV patients. The study also suggests improved survival for high/intermediate-2 risk PMF in recent years. Disclosures: Off Label Use: hydroxyurea use in myelofibrosis.

Clonal hematopoiesis in familial polycythemia vera suggests the involvement of multiple mutational events in the early pathogenesis of the disease

Blood ◽  
2003 ◽  
Vol 102 (10) ◽  
pp. 3793-3796 ◽  
Author(s):  
Robert Kralovics ◽  
David W. Stockton ◽  
Josef T. Prchal
Keyword(s):  
Stem Cell ◽  
Polycythemia Vera ◽  
Cell Clone ◽  
Incomplete Penetrance ◽  
Erythroid Progenitors ◽  
Familial Predisposition ◽  
Clonal Hematopoiesis ◽  
Healthy Family ◽  
Cell Defect ◽  
Autosomal Dominant Inheritance Pattern

AbstractFamilial clustering of malignancies provides a unique opportunity to identify molecular causes of cancer. Polycythemia vera (PV) is a myeloproliferative disorder due to an unknown somatic stem cell defect that leads to clonal myeloid hyperproliferation. We studied 6 families with PV. The familial predisposition to PV appears to follow an autosomal dominant inheritance pattern with incomplete penetrance. All examined females informative for a transcriptional clonality assay had clonal hematopoiesis. We excluded linkage between PV and a number of previously proposed candidate disease loci (c-mpl, EPOR, 20q, 13q, 5q, 9p). Therefore, mutations at these loci are unlikely primary causes of familial PV. The finding of erythropoietin-independent erythroid progenitors in healthy family members indicated the presence of the PV stem cell clone in their hematopoiesis. This finding, together with clonal hematopoiesis in the affected individuals, supports the hypothesis of multiple genetic defects involved in the early pathogenesis of PV. (Blood. 2003;102:3793-3796)

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