SOHO state-of-the-art update and next questions: MPN

The discovery of the activating JAK2V617F mutation in 2005 in the majority of patients with the classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) spurred intense interest in research into these disorders, culminating in the identification of activating mutations in MPL in 2006 and indels in CALR in 2013, thus providing additional mechanistic explanations for the universal activation of Janus kinase-signal transducer and activator of transcription (JAK-STAT) observed in these conditions, and the success of the JAK1/2 inhibitor ruxolitinib, which first received regulatory approval in 2011.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Review Article Source Type: research

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Publication date: Available online 29 November 2018Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): S. Venugopal, J. MascarenhasAbstractChronic neutrophilic leukemia (CNL) is a BCR–ABL1 negative myeloproliferative (MPN) neoplasm with notably dismal survival. The current 2016 WHO classification of myeloid neoplasms enables clinicians to unequivocally differentiate CNL from its comparable MDS/MPN overlap syndromes. Additionally, the gradual emergence of next-generation sequencing has progressively expanded our evolving understanding of the molecular pathogenesis of CNL and its therapeutic potential. Hematopoiet...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
Publication date: Available online 23 November 2018Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Brianna Smith, Michael Savona, Rami S. KomrokjiAbstractMyelodysplastic/Myeloproliferative neoplasms (MDS/MPN) are hybrid group of chronic myeloid neoplasms combining features of both MDS and MPN. The world health organization (WHO) classification coined this group designation in 2008 to include chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia (aCML), juvenile myelomoncoytic leukemia (JMML), refractory anemia with ring sideroblasts and thrombocytosis (RARS-T) as provisional entity, and MDS/MPN...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research
IntroductionChronic graft-versus-host disease (cGVHD) affects up to 50% of the long-term survivors of allogeneic hematopoietic stem cell transplantation (HCT), and is the leading cause of mortality in patients who survive to two years post-transplant. Unlike acute GVHD (aGVHD), the primary pathology in cGVHD is fibrotic, affecting the skin, and lacrimal and salivary glands, and shares many features with the autoimmune conditions Sjogrens syndrome and systemic sclerosis. Certain gastrointestinal microbiota compositions have been associated with these autoimmune conditions, and we thus hypothesized that the configuration of ...
Source: Blood - Category: Hematology Authors: Tags: 722. Clinical Allogeneic Transplantation: Acute and Chronic GVHD, Immune Reconstitution: Biomarkers and the Microbiome Source Type: research
MF is a myeloproliferative neoplasm characterized by abnormal megakaryocytes and elevated proinflammatory cytokines which results in bone marrow fibrosis, progressive hepatosplenomegaly due to extramedullary hematopoiesis, and debilitating constitutional symptoms. Current treatments, including ruxolitinib (the only approved drug for MF), provide symptomatic relief but have limited effects on the underlying disease. Effective therapies with potential MF disease course modification and second line therapies are urgently needed.CPI-0610 has been evaluated in 3 Phase 1 studies in> 140 patients with lymphoma, multiple myelom...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical Source Type: research
INTRODUCTIONUsually attributed to allergies or parasites (Seifert et. al Medline 2008) eosinophila is often overlooked. However hypereosinophilia (absolute eosinophil count>1.5 X 109/L on two separate exams one month apart or with pathologic confirmation) can have serious manifestations. When hypereosinophilia is associated with eosinophil-mediated organ damage or dysfunction, a hypereosinophilic syndrome (HES) exists.With an unpredictable course, eosinophilic infiltration commonly affects the skin (eczema), lung (dyspnea), &GIT (gastritis). However life-threatening damage to the CVS (myocarditis) or the CNS may occ...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical Source Type: research
Conclusion. Due to the good toxicity profile and the oral administration, combined therapy with momelotinib and citarinostat may represent a promising novel therapeutic modality for hematological malignancies. The study is ongoing and further investigation is required.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 802. Chemical Biology and Experimental Therapeutics: Poster II Source Type: research
CONCLUSIONThe considered clinical and biological features, at MPN diagnosis, were not different in cases with SC and controls. During the course of the disease, three factors significantly and independently affected the risk of SC in these MPN patients: 1) patients with PV had a 77% higher risk than those with ET, 2) patients with MPN duration of more than 5 years had a twice higher risk than those with lower duration, 3) for the first time, we documented that in non-skin solid cancers, aspirin treatment reduced SC risk of 38%. Exposure to HU and other cytoreductive drugs was confirmed as a risk factor for non-melanoma ski...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster III Source Type: research
The co-occurrence of myeloproliferative (MPN) and lymphoproliferative neoplasms (LPN) is rare and many publications have been limited to small case reports. Others have involved a considerable number of patients, but the coexistence remains underreported and inadequately studied. A recent retrospective review reported that a MPN patients have a 2.8-fold higher relative risk of developing LPN.A database developed at Weill Cornell Medicine (WCM) was queried for patients with ≥3 visits between 1998-2018 with a diagnostic code for a MPN and lymphoma or myeloma subtype. Patients identified were verified to ensure that study ...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster III Source Type: research
Conclusion: Combing AI "software" and the human expert "hardware" will allow for the quick delivery of comprehensive information needed for patient care that outperforms what either can achieve individually in the field of hematological disease.Figure1. Comparison of potential driver mutations between human curation and Watson for Genomics. The size of the gene symbol indicates the total number of mutations identifiedDisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 902. Health Services Research-Malignant Diseases: Poster I Source Type: research
Conclusions: This study provides evidence that: 1) the cumulative incidence of ST is about 1% person-year of follow up in SMF patients; 2) JAK inhibitors given during ET/PV or SMF phase are neutral for ST development within the limit of current follow up; 3) developing SMF in patients with PV or ET does not imply a higher risk of ST. These findings highlight the need of studies aimed at identifying patients at higher risk of ST occurrence.DisclosuresRambaldi: Roche: Consultancy; Celgene: Consultancy; Novartis: Consultancy; Italfarmaco: Consultancy; Omeros: Consultancy; Amgen Inc.: Consultancy; Pfizer: Consultancy. Komrokji...
Source: Blood - Category: Hematology Authors: Tags: 634. Myeloproliferative Syndromes: Clinical: Poster II Source Type: research
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