Myeloproliferative Disorders (Myeloproliferative Disorder)

Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.

Also Known As:

Networked: 1177 relevant articles (44 outcomes, 88 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombocytosis (Thrombocythemia)
2.	BCR-ABL Positive Chronic Myelogenous Leukemia (Chronic Myelogenous Leukemia)
3.	Essential Thrombocythemia
4.	Lymphoma (Lymphomas)
5.	Polycythemia Vera

Experts

1.	Tefferi, Ayalew: 23 articles (03/2010 - 01/2003)
2.	Gilliland, D Gary: 16 articles (01/2007 - 01/2002)
3.	Cross, Nicholas C P: 15 articles (01/2008 - 01/2002)
4.	Kantarjian, Hagop: 10 articles (02/2008 - 09/2002)
5.	Verstovsek, Srdan: 9 articles (09/2013 - 09/2003)
6.	Vainchenker, William: 9 articles (02/2010 - 04/2005)
7.	Cortes, Jorge: 9 articles (02/2008 - 10/2002)
8.	Reiter, Andreas: 8 articles (01/2012 - 09/2005)
9.	Giles, Francis: 8 articles (11/2007 - 09/2002)
10.	Kreipe, Hans: 8 articles (01/2007 - 09/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Myeloproliferative Disorders:

1.	Interferon-alpha (Interferon Alfa)FDA Link 06/01/2006 - "Conventional interferon-alpha (IFN) is an effective treatment for patients with myeloproliferative disorders. " 11/01/2000 - "Interferon-alpha and the pathogenesis of myeloproliferative disorders." 03/01/2000 - "Interferon alpha (IFN) inhibits the growth of the abnormal clone in patients with myeloproliferative disorders, leading to a reduction of the clinical and laboratory signs of the pathologic myeloproliferation. " 12/01/1999 - "The role of interferon-alpha in the treatment of myeloproliferative disorders." 06/15/1999 - "Interferon-alpha (rIFN-alpha) is an established therapy for patients with myeloproliferative disorders. "
2.	anagrelide (Agrylin)FDA LinkGeneric 01/01/2007 - "A multicenter, open, phase II study of anagrelide treatment in 60 patients during 2 yr was performed by the Swedish Myeloproliferative Disorder Study Group. " 05/01/2004 - "A multicenter, open, phase II study of anagrelide treatment was performed by the Swedish Myeloproliferative Disorder Study Group. " 05/01/2008 - "This makes anagrelide adequate for the treatment of chronic myeloproliferative disorders characterized by marked thrombocytemia. " 06/01/2006 - "Anagrelide (ANA) and hydroxycarbamide (HC) are two distinct pharmacological agents used to treat thrombocythaemia associated with myeloproliferative disorders. " 05/01/2006 - "Aim of patient register is to monitor medical effect of anagrelide therapy and incidence of adverse effects in patients with ET and other myeloproliferative disorders and subsequent analysis of collected data. "
3.	imatinib (Gleevec)FDA Link 12/01/2004 - "Recently, STI (imatinib mesylate) has been shown to be effective in treating patients with chronic myeloproliferative disorder (CMPD) displaying the translocation of the PDGFbetaR gene. " 09/01/2005 - "Further studies investigating the effects of imatinib on normal hematopoiesis are of interest as they might lead to a better understanding of the clinically observed side effects and also might help identify new therapeutic applications of the drug, possibly in Bcr-Abl-negative myeloproliferative disorders and potentially as an immunomodulatory agent." 06/01/2005 - "Studies investigating the effects of imatinib on normal hematopoiesis are of interest because they might help us better understand the side effects observed clinically and might lead to the identification of novel therapeutic applications of the drug (e.g., in Bcr-Abl(-) myeloproliferative disorders and potentially as an immunomodulatory agent)." 12/01/2008 - "We studied 103 consecutive patients with chronic myeloid leukaemia on treatment with imatinib (IM) and 57 patients with chronic myeloproliferative disorders not treated with IM in order to evaluate its cardiotoxicity. " 01/01/2008 - "A key ongoing challenge is to define the molecular pathogenesis of the great majority of atypical myeloproliferative disorders for whom the causative lesion remains unknown, since very few of these cases gain any benefit from imatinib or other second-generation inhibitors."
4.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 09/01/1996 - "The rationale for the use of aspirin in patients with PV and ET is provided by the efficacy of this agent in the treatment of microcirculatory disturbances of thrombocythemic states associated with myeloproliferative disorders and by recent evidence that asymptomatic PV and ET patients have persistently increased thromboxane (TX) A2-biosynthesis. " 06/01/2003 - "The potential risk factors most often associated with haemorrhage were a diagnosis of a myeloproliferative disorder, aspirin therapy or both. " 08/01/2000 - "These findings led to the diagnosis of an underlying myeloproliferative disorder explaining both her cutaneous and liver abnormalities and institution of appropriate platelet directed anticoagulation with aspirin." 01/01/1986 - "It is suggested that patients with myeloproliferative disorders lack - to varying degrees - an aspirin-independent mechanism which amplifies the primary response to PAF. " 03/01/2007 - "Whatever the ET subgroup, antiplatelet therapy is largely used, based on the results of the ECLAP prospective controlled trial that showed a statistically significant reduction in thrombotic complications in patients receiving aspirin for polycythemia vera, a very similar myeloproliferative disorder."
5.	Hydroxyurea (Hydrea)FDA LinkGeneric 11/01/2012 - "Hydroxyurea (HU) is an antitumor agent effective in the treatment of myeloproliferative disorders. " 05/01/2005 - "Over the last 20 years a vast array of data has been accumulated on the efficacy of hydroxyurea (HU) in patients with Philadelphia-negative myeloproliferative disorders (MPD). " 02/01/2005 - "The efficacy of hydroxyurea (HU) in myeloproliferative disorders is well documented. " 05/01/2014 - "Hydroxyurea is an antimetabolite drug used in the treatment of myeloproliferative disorders. " 01/01/2014 - "Hydroxyurea is a cytotoxic agent widely used in the treatment of myeloproliferative disorders. "
6.	Protein-Tyrosine Kinases (Tyrosine Kinase)IBA 06/15/2008 - "Recent studies have demonstrated that patients with myeloproliferative disorders (MPDs) frequently have acquired activating mutations in the JAK2 tyrosine kinase. " 09/17/2015 - "CML is a myeloproliferative disorder that results from dysregulated tyrosine kinase activity of the fusion oncoprotein BCR-ABL. " 01/01/2013 - "Review of current classification, molecular alterations, and tyrosine kinase inhibitor therapies in myeloproliferative disorders with hypereosinophilia." 04/01/2012 - "Centrosomal targeting of tyrosine kinase activity does not enhance oncogenicity in chronic myeloproliferative disorders." 10/27/2011 - "In recent years, a single point mutation (V617F) in the tyrosine kinase JAK2 was found to be present with a high incidence in myeloproliferative disorders (MPDs). "
7.	Messenger RNA (mRNA)IBA 02/01/2009 - "To investigate the frequency and mutational status of JAK2V617F mutation in Chinese patients with chronic myeloproliferative disorders (CMPD) and to study the relative quantification of mutated JAK2 mRNA and the clinical significance. " 11/01/2007 - "In patients with myeloproliferative disorders CD177 mRNA overexpression is secondary to a gain-of-function mutation in JAK2, JAK2 V617F. " 09/01/2004 - "Clinical significance of neutrophil CD177 mRNA expression in Ph-negative chronic myeloproliferative disorders." 05/01/2004 - "Mpl mRNA expression was significantly increased up to 9-fold in total bone marrow cells (p < 0.001) and up to 4-fold in megakaryocytes in chronic myeloproliferative disorders (n = 73) compared to normal controls (n = 26, p = 0.01). " 01/01/1990 - "Analysis of c-sis/PDGF-B mRNA expression in megakaryocytic cells of patients with myeloproliferative disorders."
8.	ranimustineIBA 04/01/1987 - "Our study supports the contention that MCNU tablet is a useful agent against myeloproliferative disorders." 04/01/1987 - "A phase II study of the oral agent methyl 6-[3-(2-chloroethyl)-3-nitrosoureido]-6-deoxy-alpha-D-glucopyranoside (MCNU tablet) for myeloproliferative disorders was performed. " 05/01/1986 - "A phase II study of MCNU tablet has been performed on 70 patients with hematological disorders including mostly myeloproliferative disorders. " 02/01/1991 - "Seventeen patients with myeloproliferative disorders and one patient with chronic myelomonocytic leukemia (CMMoL) were treated with ranimustine++ (MCNU), and the efficacy was evaluated. " 02/01/1991 - "[Therapeutic effect of ranimustine(MCNU) on myeloproliferative disorder and chronic myelomonocytic leukemia]."
9.	Phosphotransferases (Kinase)IBA 05/01/2006 - "This study demonstrates the utility of screening for PDGFRA kinase domain overexpression in patients with IHES and has identified a third PDGFRA fusion partner in chronic myeloproliferative disorders." 05/01/2013 - "Small molecule inhibitors of Janus kinase (JAK) family members (JAK1, JAK2, JAK3, and Tyk2) are currently being pursued as potential new modes of therapy for a variety of diseases, including the inhibition of JAK2 for the treatment of myeloproliferative disorders. " 08/01/2011 - "Chronic Myeloid Leukaemia (CML) is a myeloproliferative disorder characterized by the expression of the oncoprotein, Bcr-Abl kinase. " 01/01/2011 - "Constitutive MAP kinase activation in hematopoietic stem cells induces a myeloproliferative disorder." 01/01/2011 - "JAK2 is an obligatory kinase for the proliferation and differentiation of erythroid cells and megakaryocytes thus representing a relevant therapeutic target for agents that specifically inhibit its activity particularly in myeloproliferative disorders (MPD) harboring JAK2(V617F) mutations. "
10.	Janus Kinase 2IBA 08/27/2009 - "Ongoing clinical trials of Janus kinase 2 (JAK2) inhibitors in myeloproliferative disorder patients use small molecules targeting both wild-type and mutated JAK2. " 06/01/2015 - "Herein we report a rapid, accurate and robust UHPLC-MS/MS assay for the quantitation of BMS-911453, a Janus kinase 2 inhibitor under clinical development for the treatment of myeloproliferative disorders, in human plasma. " 02/15/2014 - "Janus Kinase 2 (JAK2) gene single point mutations, which have been reported to be associated with myeloproliferative disorders, are usually detected through conventional methods such as melting curve assays, allele-specific and quantitative Polymerase Chain Reactions (PCRs). " 09/01/2012 - "An activating mutation of Janus kinase 2 (JAK2-V617F) was previously described in chronic myeloproliferative disorders (MPD). " 02/01/2012 - "Janus kinase 2 (JAK2) V617F mutation testing has revolutionized the classification of myeloproliferative disorders, for which several tests have been introduced for qualitative and quantitative diagnostics including the MutaScreen and MutaQuant kits by IPSOGEN. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 11/01/2014 - "From 2001 to 2011, patients with lymphoproliferative or myeloproliferative disorders treated with chemotherapy were retrospectively identified. " 01/01/2009 - "Clonal switch of leukemic myeloblasts after chemotherapy in a patient with chronic myeloproliferative disorder." 02/01/2002 - "However, to date the pattern of replication has not been studied in myeloproliferative disorders nor has the effect of chemotherapy been systematically evaluated. " 05/01/1989 - "The outcome of treatment with standard first line therapy of 66 patients with acute myeloid leukaemia (AML) secondary to preceding chemotherapy (Group 1), a myelodysplastic state (Group 2) or a myeloproliferative disorder (Group 3) was analysed in relation to the preceding disorder, the cytogenetic pattern where available, and the cytology and cytochemistry of blood and bone marrow. " 09/01/1990 - "Radiotherapy of the pelvic lesion and chemotherapy to control the myeloproliferative disorder gave rise to significant improvement in neuropathy. "
2.	Splenectomy 11/01/1996 - "The myeloproliferative disorders constitute a spectrum of disease potentially improved by splenectomy, but preoperative management should be modified in this group. " 09/03/1990 - "Four patients with myeloproliferative disorders had impaired platelet aggregation before splenectomy that improved in only one patient after surgery. " 10/01/2015 - "The incidence of PVT after splenectomy in patients with myeloproliferative disorders is high (40%). " 04/25/2003 - "Flow cytometry applied to sFNAB corroborates the cytologic diagnosis in lymphoid and myeloproliferative disorders of the spleen and allows therapeutic decisions avoiding splenectomy." 09/01/2000 - "Patients with a myeloproliferative disorder or haemolytic anaemia are at higher risk; they might benefit from early detection and could have routine Doppler ultrasonography after splenectomy."
3.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 04/01/2003 - "Long-term complete haematological and molecular remission after allogeneic bone marrow transplantation in a patient with a stem cell myeloproliferative disorder associated with t(8;13)(p12;q12)." 12/01/2008 - "Murine retroviral bone marrow transplantation models for the study of human myeloproliferative disorders." 06/01/2007 - "In murine bone marrow transplantation studies, HoxA10 overexpression induces a myeloproliferative disorder with accumulation of mature phagocytes in the peripheral blood and tissues. " 11/21/2013 - "Here we demonstrate that the most prevalent, activating mutation, CSF3R T618I, is sufficient to drive a lethal myeloproliferative disorder in a murine bone marrow transplantation model. " 02/01/1999 - "Chronic myelogenous leukemia (CML), a myeloproliferative disorder characterized by the clonal proliferation of a hematopoietic stem cell, is a malignancy for which allogeneic bone marrow transplantation (BMT), when available, constitutes a mainstay of treatment. "
4.	Hematopoietic Stem Cell Transplantation 10/01/2011 - "The results showed that (1) out of 46 newly diagnosed as chronic myeloproliferative disease or myelodysplastic and myeloproliferative disorders, 22 cases were diagnosed as CML, the FISH detection showed all positive (100%), while cytogenetic test showed 86.4% (19/22) positive, in the other 24 patients who were diagnosed as other chronic myeloproliferative disease or myelodysplastic and myeloproliferative disorders, BCR/ABL fusion gene all were be detected as negative 100% by FISH, while the cytogenetic test of bone marrow in 3 cases supported the diagnosis of CML, and the diagnosis of myelodysplastic disorder in 1 case; (2) in 3 out of 7 acute lymphocytic leukemia cases the BCR/ABL fusion gene could not be detected by FISH; (3) the BCR/ABL fusion gene could be detected by FISH in 2 cases of CML received allogeneic hematopoietic stem cell transplantation, with abnormal threshold 6.5% and 1.2% respectively. " 06/01/2007 - "Juvenile myelomonocytic leukemia (JMML) is a rapidly fatal myeloproliferative disorder of early childhood for which no effective treatment other than hematopoietic stem cell transplantation is currently available. " 10/01/2003 - "Allogeneic hematopoietic stem cell transplantation for myeloproliferative disorders and myelodysplastic syndromes." 01/01/2011 - "Children with Noonan syndrome (NS) are at increased risk of developing juvenile myelomonocytic leukemia (JMML) or a myeloproliferative disorder associated with NS (MPD/NS) resembling JMML in the first weeks of life; whereas JMML is an aggressive disorder requiring hematopoietic stem cell transplantation, MPD/NS may resolve without treatment and cases with spontaneous remission have also been reported. " 10/01/2011 - "The conventional cytogenetic test and detection of BCR/ABL fusion gene by FISH for bone marrow of patients with newly diagnosed chronic myeloproliferative disease or myelodysplastic and myeloproliferative disorders, acute lymphocytic leukemia and chronic myelogenous leukemia (CML) after allogeneic hematopoietic stem cell transplantation were carried out. "
5.	Radiotherapy 09/01/1990 - "Radiotherapy of the pelvic lesion and chemotherapy to control the myeloproliferative disorder gave rise to significant improvement in neuropathy. " 01/01/2015 - "Adaptive splenic radiotherapy for symptomatic splenomegaly management in myeloproliferative disorders." 10/15/2001 - "In the present study, the frequencies of isolated +8 in relation to gender, age, previous treatment with chemo- or radiotherapy, and morphologic subtype were ascertained in published, as well as in our own unpublished, cases of acute myeloid leukemia (AML; n=4,246), myelodysplastic syndromes (MDS; n=1,817), and chronic myeloproliferative disorders (MPD; n=530). " 08/01/1980 - "Radiotherapy has little role in the treatment of chest wall tumors except for the myeloproliferative disorders and possibly some cases of Ewing's sarcoma. "