Polycythemia Vera

A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.

Also Known As:

Networked: 1317 relevant articles (55 outcomes, 133 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Essential Thrombocythemia
2.	Thrombocytosis (Thrombocythemia)
3.	Pruritus (Itching)
4.	Primary Myelofibrosis (Myelosclerosis)
5.	Thrombosis (Thrombus)

Experts

1.	Tefferi, Ayalew: 31 articles (04/2015 - 04/2002)
2.	Verstovsek, Srdan: 27 articles (09/2015 - 10/2006)
3.	Vannucchi, Alessandro M: 25 articles (05/2015 - 01/2007)
4.	Barbui, Tiziano: 22 articles (10/2014 - 01/2003)
5.	Finazzi, Guido: 18 articles (10/2014 - 01/2003)
6.	Rambaldi, Alessandro: 16 articles (10/2014 - 08/2010)
7.	Levine, Ross L: 16 articles (08/2013 - 04/2005)
8.	Prchal, Josef T: 15 articles (10/2015 - 06/2004)
9.	Guglielmelli, Paola: 15 articles (05/2015 - 11/2007)
10.	Silver, Richard T: 14 articles (10/2015 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Polycythemia Vera:

1.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 01/08/2004 - "We enrolled 518 patients with polycythemia vera, no clear indication for aspirin treatment, and no contraindication to such treatment in a double-blind, placebo-controlled, randomized trial to assess the safety and efficacy of prophylaxis with low-dose aspirin (100 mg daily). " 01/01/2003 - "In Section III, Dr. Gianni Tognoni discusses the role of aspirin therapy in PV based on the recently completed European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Study, a multi-country, multicenter project aimed at describing the natural history of PV as well as the efficacy of low-dose aspirin. " 12/01/2012 - "This issue was investigated using the database of the European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Study. " 03/15/2007 - "Thus, we exploited the large database collected by the observational study of the European Collaboration on Low-Dose Aspirin in Polycythemia Vera (ECLAP) to investigate the association of hematologic variables and cardiovascular risk factors with the thrombotic risk. " 04/01/2005 - "The European Collaboration on Low-Dose Aspirin in Polycythemia Vera study documents that large international collaborative studies are feasible in this field, in which few epidemiologic data are available. "
2.	Hydroxyurea (Hydrea)FDA LinkGeneric 02/01/1998 - "To manage the current case of polycythemia vera, hydroxyurea was effective for the control of hematocrit and transfusion of platelet concentrates was effective for hemostasis." 03/01/1987 - "Because hydroxyurea is safe and effective in the treatment of polycythemia vera, it should be considered as first-line therapy. " 08/01/2006 - "The cytoreductive agent of choice for these disorders is currently hydroxyurea, emerging from randomized trials beginning with those of the Polycythemia Vera Study Group. " 11/01/2015 - "Assessing The Level Of Resistance/Intolerance To Hydroxyurea Therapy Amongst Patients With Polycythemia Vera In Europe." 11/01/2015 - "Symptoms, Concomitant Conditions And Cardiac Risk In European Hydroxyurea Treated Polycythemia Vera Patients."
3.	ErythropoietinFDA Link 01/01/2005 - "Erythropoietin (EPO) level may help to separate diseases with high EPO (Chuvash, or yet unclassified), or with normal/low EPO (congenital with truncation of the EPO receptor, polycythemia vera-Vaquez disease-, or currently with unknown mechanism)." 01/01/2009 - "Having eliminated the common entity polycythemia vera, further direction for investigation is guided by the erythropoietin level. " 09/01/2014 - "Cytogenetic studies for polycythemia vera were negative, but the patient's erythropoietin (EPO) was elevated. " 02/01/1999 - "Furthermore, according to the Polycythemia Vera Study Group guidelines, the demonstration of an increased red blood cell mass is an absolute criterion for the diagnosis of PV. This article discusses the use of the serum erythropoietin level and endogenous erythroid colony assay as a potential alternative in the diagnosis of PV." 04/01/1994 - "Since the definition given in 1975 by the Polycythemia Vera Study Group (PVSG), some examinations became obsolete, and new ones (erythropoietin, stem cell culture) did appear, so that it seems useful to re-define the criteria of the disease. "
4.	Busulfan (Busulfex)FDA Link 05/01/1996 - "The study was aimed to assess the efficacy of busulphan treatment in patients with polycythemia rubra vera (PRV). " 01/01/1994 - "However, busulfan also seems to have some role in the modification of polycythemia vera. " 12/01/1992 - "A 57-year-old man, diagnosed as Polycythemia vera (PV), had been treated with administrations of Busulfan since 1984. " 01/01/1977 - "[Case of myelosan-induced hematopoietic aplasia in erythremia]." 04/01/1974 - "Two cases of primary erythremia treated with Busulfan."
5.	Recombinant Interferon Type IIBA 08/15/1990 - "Recombinant interferon alfa, a natural product with growth inhibitory capabilities, has been demonstrated for the first time to have significant therapeutic efficacy in controlling the red cell mass in patients with the myeloproliferative disease polycythemia vera. " 01/01/1998 - "The therapeutic efficacy of recombinant interferon-alpha (rIFN-alpha) has been evaluated in 7 patients with polycythaemia vera (PV), diagnosed according to the criteria of the Polycythemia Vera Study Group. " 08/15/1990 - "A new treatment for polycythemia vera: recombinant interferon alfa." 08/01/2006 - "Long-term effects of the treatment of polycythemia vera with recombinant interferon-alpha." 01/01/1996 - "In previous researches recombinant interferon alpha (IFN-alpha) has been demonstrated to significantly control red cell mass and thrombocytemia in patients with polycythemia vera (PV). "
6.	InterferonsIBA 03/01/2009 - "Pursuing clonal remission in polycythemia vera by interferon needs new evidence. " 01/01/2012 - "Thus, further study is warranted to elucidate the treatment of both polycythemia vera and interferon-induced impending AION." 01/01/2014 - "Combination therapy with interferon and JAK1-2 inhibitor is feasible: Proof of concept with rapid reduction in JAK2V617F-allele burden in polycythemia vera." 09/01/2013 - "Are all interferons the same for therapy in polycythemia vera?" 10/11/2012 - "Interferon (IFN-α) is effective therapy for polycythemia vera (PV) patients, but it is frequently interrupted because of adverse events. "
7.	Interferon-alpha (Interferon Alfa)FDA Link 12/01/1993 - "Interferon-alpha 2b: a new treatment for polycythemia vera." 02/01/1993 - "Morphometric study of the bone marrow in polycythemia vera following interferon-alpha therapy." 06/01/2010 - "Interferon-alpha (IFNalpha) therapy leads to hematological remissions and a reduction of the JAK2V617F allele burden in patients with polycythemia vera (PV). " 10/15/2008 - "Interferon-alpha (IFN-alpha) is a nonleukemogenic treatment of polycythemia vera (PV) able to induce cytogenetic remissions. " 04/01/2007 - "Biomarker analysis in polycythemia vera under interferon-alpha treatment: clonality, EEC, PRV-1, and JAK2 V617F."
8.	imatinib (Gleevec)FDA Link 07/01/2013 - "Effects of imatinib mesylate in patients with polycythemia vera: results of a phase II study." 07/01/2009 - "Imatinib mesylate therapy for polycythemia vera: final result of a phase II study initiated in 2001." 12/01/2008 - "As a result we began a phase II, open label trial of imatinib in patients with polycythemia vera. " 12/01/2008 - "Phase II open label trial of imatinib in polycythemia rubra vera." 07/01/2012 - "Long term follow-up of Polycythemia Vera patients treated with imatinib mesylate."
9.	Pipobroman (Vercyte)FDA Link 10/01/2000 - "Efficacy of pipobroman in the treatment of polycythemia vera: long-term results in 163 patients." 01/01/2003 - "Long-term outcomes of polycythemia vera patients treated with pipobroman as initial therapy." 05/01/1982 - "Between 1971 and 1981, 74 patients with polycythemia vera were treated with pipobroman using a high-dose induction, low-dose maintenance regimen. " 05/01/1982 - "Pipobroman therapy of polycythemia vera." 10/01/1972 - "[Clinical trial of pipobroman (N,N'-bis-(3-bromopropionyl) piperazine) in eight patients with polycythemia vera]."
10.	INCB018424IBA 10/01/2015 - "One patient with polycythemia vera achieved a partial response and received 18 cycles of ruxolitinib. " 08/01/2015 - "Ruxolitinib for the treatment of patients with polycythemia vera." 04/23/2015 - "Ruxolitinib versus standard therapy for the treatment of polycythemia vera." 04/23/2015 - "Ruxolitinib versus standard therapy for the treatment of polycythemia vera." 03/01/2015 - "Jakafi (Ruxolitinib): First FDA-Approved Medication for the Treatment of Patients with Polycythemia Vera."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 04/01/2013 - "Polycythemia vera: current pharmacotherapy and future directions." 10/01/2003 - "This is in contrast to the statement, "Thus chemotherapy treatment of [polycythemia vera] patients is not as easy, innocuous, and well tolerated as it is generally believed". " 07/01/1984 - "One hundred thirty-five polycythemia vera (PV) patients (30 untreated by chemotherapy and 105 treated) were studied cytogenetically . " 12/01/1979 - "From 1967 to 1978 38 patients with polycythemia vera received a long term cytostatic chemotherapy. " 12/01/1979 - "[A contribution to the cytostatic long-term chemotherapy of polycythemia vera (author's transl)]."
2.	Bloodletting 01/01/1987 - "[Erythron in patients with polycythemia vera during treatment by erythrocytapheresis and bloodletting]." 06/01/1986 - "[Chalone content of the blood of polycythemia vera patients following bloodletting]." 04/01/1968 - "[I-131 absorption in patients with polycythemia vera and change in this index after treatment by bloodletting and with P32]." 10/01/1967 - "[Bloodletting with return blood transfusion at the time of gastric resection of erythremia]." 09/01/1991 - "[Effect of bloodletting on the proliferative activity of erythroid progenitor cells from the bone marrow of patients with polycythemia and polycythemia vera]."
3.	Stem Cell Transplantation 01/01/2008 - "Stem cell transplantation for polycythemia vera." 05/01/2014 - "Allogeneic stem cell transplantation is currently the only potentially curative treatment for advanced polycythemia vera or essential thrombocythemia. " 11/01/2009 - "Successful treatment with reduced-intensity stem cell transplantation for secondary myelofibrosis following polycythemia vera." 07/01/2002 - "Curative therapy of advanced essential thrombocythemia or polycythemia vera by hemopoietic stem cell transplantation." 06/01/2014 - "We correlate regression of bone marrow fibrosis (BMF) on day 30 and 100 after dose- reduced allogeneic stem cell transplantation (allo-SCT) in 57 patients with primary or post-essential thrombocythemia/polycythemia vera myelofibrosis with graft function and survival. "
4.	Transplantation (Transplant Recipients) 07/31/2014 - "Treatment of polycythemia vera (PV) and primary myelofibrosis (PMF) CD34(+) cells with low doses of RG7112 and Peg-IFNα 2a before their transplantation into immune-deficient mice decreased the degree of donor-derived chimerism as well as the JAK2V617F allele burden, indicating that these drugs can each alone or in combination deplete MPN HSCs. " 10/01/2010 - "We evaluated the NOD/SCID engraftment of CD34(+) cells from polycythemia vera (PV) and secondary polycythemia patients (SP) and the JAK2V617F clone before and after transplantation. " 01/01/2012 - "Neutrophils from Healthy Blood Donors (HBD), patients with AASV most in complete remission, Polycythemia Vera (PV), Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and renal transplant recipients (TP) were incubated in vitro, and the rate of spontaneous apoptosis was measured by FACS. " 05/01/2014 - "This large retrospective study confirms that transplantation is potentially curative for patients with end-stage polycythemia vera/essential thrombocythemia progressing to myelofibrosis or acute myeloid leukemia. " 05/01/2014 - "We analyzed 250 consecutive patients with an initial diagnosis of polycythemia vera (n=120) or essential thrombocythemia (n=130), who underwent transplantation due to progression to myelofibrosis (n=193) or acute myeloid leukemia (n=57) and who were reported to the European Group for Blood and Marrow Transplantation registry between 1994 and 2010. "
5.	Photochemotherapy (Photodynamic Therapy) 08/01/1996 - "Efficacy of photochemotherapy on severe pruritus in polycythemia vera." 10/01/1985 - "Photochemotherapy treatment of pruritus associated with polycythemia vera." 04/01/1993 - "Oral psoralen photochemotherapy (PUVA) for pruritus associated with polycythemia vera and myelofibrosis."