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: 1419 relevant articles (56 outcomes, 110 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Tefferi, Ayalew: 26 articles (01/2021 - 01/2003)
2.	Gilliland, D Gary: 18 articles (11/2008 - 01/2002)
3.	Cross, Nicholas C P: 15 articles (01/2008 - 01/2002)
4.	Vainchenker, William: 12 articles (08/2020 - 04/2005)
5.	Kantarjian, Hagop: 11 articles (06/2008 - 09/2002)
6.	Verstovsek, Srdan: 10 articles (09/2013 - 09/2003)
7.	Cortes, Jorge: 10 articles (06/2008 - 10/2002)
8.	Bock, Oliver: 9 articles (06/2007 - 09/2003)
9.	Kreipe, Hans: 9 articles (06/2007 - 09/2003)
10.	Mesa, Ruben A: 8 articles (01/2019 - 08/2003)

Drugs and Biologics

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

1.	Interferon-alpha (Interferon Alfa)IBA 06/01/2006 - "Conventional interferon-alpha (IFN) is an effective treatment for patients with myeloproliferative disorders. " 03/01/1996 - "Cytogenetic remission induced by interferon-alpha in a myeloproliferative disorder with trisomy 8." 01/01/2020 - "Interferon alfa (IFNα) has been used in the treatment of myeloproliferative disorders for more than 30 years. " 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. "
2.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 07/06/2023 - "Aspirin has been shown effective in Myeloproliferative Disorders." 03/01/2017 - "Aspirin is a useful treatment of erythromelagia associated with myeloproliferative disorders. " 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. " 06/01/2003 - "The potential risk factors most often associated with haemorrhage were a diagnosis of a myeloproliferative disorder, aspirin therapy or both. "
3.	Imatinib Mesylate (Gleevec)FDA Link 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)." 01/01/2023 - "FIP1L1-PDGFRA-positive T-LBL and myeloproliferative disorders have excellent response to low-dose treatment with (TKI) imatinib. " 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." 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. "
4.	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. " 10/01/2019 - "Anagrelide is an oral imidazoquinazoline agent that is prescribed for reducing elevated platelet counts in patients with myeloproliferative disorders. " 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. "
5.	Hydroxyurea (Hydrea)FDA LinkGeneric 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. " 11/23/2023 - "Hydroxyurea is a cytotoxic drug commonly used to treat various myeloproliferative disorders. " 01/01/2006 - "Hydroxyurea is an antitumour agent used most commonly to treat myeloproliferative disorders. " 02/01/2005 - "Safety profile of hydroxyurea in the treatment of patients with Philadelphia-negative chronic myeloproliferative disorders."
6.	Janus Kinase InhibitorsIBA 07/01/2023 - "Janus kinase inhibitors (JAKi) have dramatically improved the treatment of various autoimmune and myeloproliferative disorders. " 06/01/2020 - "Gene knock-out studies together with evidence from patients carrying activating mutant forms of JAKs (eg, JAK2 V617F in myeloproliferative disorders) provided strong rationale for the development of JAK inhibitors. " 01/01/2019 - "However, compared to the large amount of clinical studies with JAK inhibitors that are currently widely used in the clinics to treat myeloproliferative disorders, the clinical trials with STAT5 inhibitors are very limited. " 01/01/2022 - "Furthermore, as clinical data regarding the limitations of approved JAK inhibitors in myeloproliferative disorders matures, there is a growing awareness of the need for alternative precision medicine approaches for specific JAK2 lesions. " 06/09/2021 - "The small molecules directed to inhibit JAK/STAT activation were assayed in vitro and in in vivo models of pulmonary fibrosis, and different JAK inhibitors are currently approved for myeloproliferative disorders. "
7.	Tyrosine Kinase InhibitorsIBA 10/01/2020 - "Pulmonary complications of tyrosine kinase inhibitors in myeloproliferative disorders." 05/01/2004 - "This case illustrates one of many myelodysplastic/myeloproliferative disorders that can be treated with this particular tyrosine kinase inhibitor." 08/01/2009 - "Myeloproliferative disorder with eosinophilia and ETV6-ABL gene rearrangement: efficacy of second-generation tyrosine kinase inhibitors." 12/31/2021 - "Chronic myeloid leukemia (CML), a myeloproliferative disorder caused by the over activity of BCR-ABL1 (breakpoint cluster region-Abelson), has been successfully treated by Tyrosine kinase inhibitors (TKIs). " 01/01/2013 - "Review of current classification, molecular alterations, and tyrosine kinase inhibitor therapies in myeloproliferative disorders with hypereosinophilia."
8.	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."
9.	Antineoplastic Agents (Antineoplastics)IBA 01/01/2018 - "NPV-BSK805, an Antineoplastic Jak2 Inhibitor Effective in Myeloproliferative Disorders, Causes Adiposity in Mice by Interfering With the Action of Leptin." 11/01/2012 - "Hydroxyurea (HU) is an antitumor agent effective in the treatment of myeloproliferative disorders. " 12/01/2019 - "Janus kinase 2 (JAK2) inhibitors represent a promising therapeutic class of anticancer agents against many myeloproliferative disorders. " 04/01/2016 - "Hydroxyurea (HU) is an antineoplastic drug used in chronic myeloproliferative disorders. " 03/01/2011 - "Hydroxyurea (HU) is an antineoplastic drug commonly used to treat chronic myeloproliferative disorders. "
10.	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. " 01/12/2016 - "Chronic myeloid leucaemia (CML) is a chronic myeloproliferative disorder characterised by a reciprocal translocation between the chromosomes 9 and 22 resulting in constitutionally active tyrosine kinase signalling. " 04/01/2014 - "Factors associated with a lack of clinical resolution included signs of portal hypertension⁄liver failure on presentation, complete vessel occlusion at diagnosis, presence of a myeloproliferative disorder or JAK2V617F tyrosine kinase mutation and the absence of a local⁄transient predisposing factor. " 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). "

Therapies and Procedures

1.	Therapeutics 11/01/2012 - "These recent advances in this enigmatic disorder, now known as Transient Myeloproliferative Disorder, are reviewed here in order to bring clarity to the breadth of organ involvement, the range of severity, the risk factors for mortality, the therapeutic options for severe manifestations, the natural course of spontaneous resolution regardless of therapy, and the elucidation of the subsequent risk for myeloid leukaemia." 10/31/2019 - "The Myeloproliferative Disorders Research Consortium (MPD-RC)-111 study was an investigator-initiated, international, multicenter, phase 2 trial evaluating the ability of PEG therapy to induce complete (CR) and partial (PR) hematologic responses in patients with high-risk ET or PV who were either refractory or intolerant to HU. " 06/01/2009 - "This study was aimed to investigate the JAK2V617F mutation in myeloproliferative disorders (MPD) and to evaluate the significance of JAK2V617F in diagnosis and therapy of MPD. " 01/01/2008 - "JAK2 inhibitor therapy in myeloproliferative disorders: rationale, preclinical studies and ongoing clinical trials." 11/01/2007 - "PEG-IFN-alpha-2b therapy in BCR-ABL-negative myeloproliferative disorders: final result of a phase 2 study."
2.	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. " 09/01/1990 - "Radiotherapy of the pelvic lesion and chemotherapy to control the myeloproliferative disorder gave rise to significant improvement in neuropathy. " 09/01/2014 - "The primary objectives of the conference included: (1) to present advances in Allo-SCT in pediatric ALL and novel pre and post-transplant immunotherapy; (2) to highlight new strategies in alternative allogeneic stem cell donor sources for children and adolescents with non-malignant hematological disorders; (3) to discuss timing of immune reconstitution after Allo-SCT and methods of facilitating more rapid recovery of immunity; (4) to identify strategies of utilizing Allo-SCT in pediatric myeloproliferative disorders; (5) to develop diagnostic and therapeutic approaches to hematological complications post pediatric Allo-SCT; (6) to enhance the understanding of new novel cellular therapeutic approaches to pediatric malignant and non-malignant hematological disorders; and (7) to discuss optimizing drug therapy in pediatric recipients of Allo-SCT. "
3.	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." 09/15/1993 - "Increased platelet count (> 5 x 10(11)/L) after splenectomy was associated with vWF abnormalities indistinguishable from those detected in patients with myeloproliferative disorders. "
4.	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. " 05/01/1995 - "He was diagnosed with hypereosinophilic syndrome (HES) probably due to myeloproliferative disorder, and underwent allogeneic bone marrow transplantation (allo-BMT) from an HLA-identical sibling. "
5.	Hematopoietic Stem Cell Transplantation 01/01/2022 - "Allogeneic Hematopoietic Stem Cell Transplantation for Mixed or Overlap Myelodysplastic/Myeloproliferative Disorders." 11/01/2018 - "Early human cytomegalovirus reactivation is associated with lower incidence of relapse of myeloproliferative disorders after allogeneic hematopoietic stem cell transplantation." 01/01/2016 - "Chronic myeloid leukemia is a myeloproliferative disorder that may be treated with hematopoietic stem cell transplantation (HSCT). " 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. "