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myeloproliferative syndromes

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https://www.readbyqxmd.com/read/28822056/patient-reported-outcomes-in-myelodysplastic-syndromes-and-mds-mpn-overlap-syndromes-stepping-onto-the-stage-with-changing-times
#1
REVIEW
Sagar S Patel, Aaron T Gerds
Quality of life (QOL) and symptom burden are important measures captured by patient-reported outcomes (PROs). Myelodysplastic and myelodysplastic/myeloproliferative (MDS/MPN) neoplasm overlap syndromes are notable for significant morbidity and mortality, including a wide spectrum of physical and psychosocial effects. Thus, the development and application of PROs can provide meaningful information to facilitate communication and assist in follow up care. Disease-specific measures can more accurately reflect the full breadth of functional restrictions and symptoms...
August 18, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28794380/tumor-lysis-syndrome-after-the-administration-of-ruxolitinib-in-a-patient-with-post-polycythemia-vera-myelofibrosis
#2
Megumi Koshiishi, Yuki Sueki, Ichiro Kawashima, Kei Nakajima, Toru Mitsumori, Keita Kirito
The development of tumor lysis syndrome (TLS) in association with treatment for myeloproliferative neoplasms (MPNs) is relatively rare. We herein present the case of a post-polycythemia vera (PV) myelofibrosis patient with massive splenomegaly who developed laboratory TLS after treatment with ruxolitinib, a potent JAK1/JAK2 inhibitor. She also exhibited a rapid reduction of spleen volume. Our present case suggests the potential risk of TLS development after ruxolitinib treatment, particularly in patients with massive splenomegaly...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28781891/vascular-occlusions-following-ocular-surgical-procedures-a-clinical-observation-of-vascular-complications-after-ocular-surgery
#3
Charlotte Fischer, Anne Bruggemann, Annette Hager, Josep Callizo Planas, Johann Roider, Hans Hoerauf
BACKGROUND: Ocular vascular occlusions following intraocular procedures are a rare complication. We report a case series of patients with retinal vascular occlusions or anterior ischemic optic neuropathy (AION) after anterior and posterior segment surgery and demonstrate possible risk factors. METHODS: Observational case series. RESULTS: In ten patients, vascular occlusions were observed within ten weeks after intraocular surgery: branch retinal arterial occlusion (BRAO) (n = 2), central retinal artery occlusion (CRAO) (n = 2), central retinal vein occlusion (CRVO) (n = 1), branch retinal vein occlusion (BRVO) (n = 1), anterior ischemic optic neuropathy (AION) (n = 3), and combined central artery and vein occlusion (n = 1)...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/28775205/therapy-related-hematological-malignancies-after-peptide-receptor-radionuclide-therapy-with-177-lu-dota-octreotate-incidence-course-predicting-factors-in-patients-with-gep-nets
#4
Hendrik Bergsma, Kirsten van Lom, Mark Konijnenberg, Boen Kam, Jaap Teunissen, Wouter de Herder, Eric Krenning, Dik Kwekkeboom
Peptide Receptor Radionuclide Therapy (PRRT) may induce long-term toxicity to the bone marrow (BM). The aim of this study was to analyze persistent dysfunction of the hematopoietic system after PRRT with (177)Lu-DOTATATE in patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Methods: The incidence and course of persistent hematological dysfunction (PHD) was analyzed in 274 (=GEP-NET) out of 367 patients with somatostatin receptor-positive tumors. PHD was defined as diagnosis of Myelodysplastic Syndrome (MDS), Acute Myeloid Leukemia (AML), Myeloproliferative Neoplasms (MPN), Myelodysplastic/Myeloproliferative neoplasms (MDS/MPN) or otherwise unexplained cytopenia (for more than 6 months)...
August 3, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28774880/a-randomized-phase-ii-study-of-low-dose-decitabine-versus-low-dose-azacitidine-in-lower-risk-mds-and-mds-mpn
#5
Elias Jabbour, Nicholas J Short, Guillermo Montalban-Bravo, Xuelin Huang, Carlos Bueso-Ramos, Wei Qiao, Hui Yang, Chong Zhao, Tapan Kadia, Gautam Borthakur, Naveen Pemmaraju, Koji Sasaki, Zeev Estrov, Jorge Cortes, Farhad Ravandi, Yesid Alvarado, Rami Komrokji, Mikkael A Sekeres, David P Steensma, Amy DeZern, Gail Roboz, Hagop Kantarjian, Guillermo Garcia-Manero
BACKGROUND: Hypomethylating agents (HMAs) improve survival in patients with higher-risk myelodysplastic syndromes (MDS) but are less well-studied in lower-risk disease. We compared the safety and efficacy of low-dose decitabine versus low-dose azacitidine in this group of patients. METHODS: Adults with low- or intermediate-1-risk MDS or MDS/myeloproliferative neoplasm (MPN), including chronic myelomonocytic leukemia, by the International Prognostic Scoring System were randomized using a Bayesian adaptive design to receive either azacitidine 75 mg/m(2) IV/SC daily or decitabine 20 mg/m(2) IV daily for 3 consecutive days on a 28-day cycle...
August 3, 2017: Blood
https://www.readbyqxmd.com/read/28771058/myelodysplastic-and-myeloproliferative-neoplasms-updates-on-the-overlap-syndromes
#6
Swapna Thota, Aaron T Gerds
Myelodysplastic and myeloproliferative neoplasms (MDS/MPN) is a rare and distinct group of myeloid neoplasms with overlapping MDS and MPN features. Next generation sequencing studies have led to an improved understanding of MDS/MPN disease biology by identifying recurrent somatic mutations. Combining the molecular findings to patho-morphologic features has improved the precision of diagnosis and prognostic models in MDS/MPN. We discuss and highlight these updates in MDS/MPN nomenclature and diagnostic criteria per revised 2016 WHO classification of myeloid neoplasms in this article...
August 3, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28760304/diagnostic-prognostic-and-predictive-utility-of-recurrent-somatic-mutations-in-myeloid-neoplasms
#7
REVIEW
Umang Patel, Rajyalakshmi Luthra, L Jeffrey Medeiros, Keyur P Patel
The classification and risk stratification of myeloid neoplasms, including acute myeloid leukemia, myelodysplastic syndromes, myelodysplastic syndromes/myeloproliferative neoplasms, and myeloproliferative neoplasms, have increasingly been guided by molecular genetic abnormalities. Gene expression analysis and next-generation sequencing have led to the ever increasing discovery of somatic gene mutations in myeloid neoplasms. Mutations have been identified in genes involved in epigenetic modification, RNA splicing, transcription factors, DNA repair, and the cohesin complex...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28760301/the-challenge-of-treating-myelodysplastic-syndromes-myeloproliferative-neoplasms
#8
REVIEW
Brittany Knick Ragon, Michael R Savona
Myelodysplastic syndromes/myeloproliferative neoplasms (MDS/MPN) comprise a spectrum of myeloid disorders with both dysplastic and proliferative features, arising from hematopoietic stem cells. MDS/MPN are neither MDS nor MPN but a distinct World Health Organization-defined subclassification of diseases that, by definition, do not arise from a pre-existing MDS or MPN, inherently creating diagnostic and therapeutic challenges. Appropriately recognizing and classifying MDS/MPN is paramount for appropriately formulating treatment strategies and accurate prognostication...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28737134/nephrotic-syndrome-in-primary-myelofibrosis-with-renal-extramedullary-hematopoiesis-and-glomerulopathy-in-the-jak-inhibitor-era
#9
Rachele Del Sordo, Rachele Brugnano, Carla Covarelli, Gioia Fiorucci, Franca Falzetti, Giorgio Barbatelli, Emidio Nunzi, Angelo Sidoni
Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure...
July 24, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28724539/racial-and-ethnic-disparities-in-hematologic-malignancies
#10
Kedar Kirtane, Stephanie J Lee
Racial and ethnic disparities in patients with solid malignancies have been well documented. Less is known about these disparities in patients with hematologic malignancies. With the advent of novel chemotherapeutics and targeted molecular, cellular, and immunologic therapies, it is important to identify differences in care that may lead to disparate outcomes. This review provides a critical appraisal of the empirical research on racial and ethnic disparities in incidence, survival, and outcomes in patients with hematologic malignancies...
July 19, 2017: Blood
https://www.readbyqxmd.com/read/28710306/ruxolitinib-treatment-in-an-infant-with-jak2-polycythaemia-vera-associated-budd-chiari-syndrome
#11
Mehmet Enes Coskun, Sue Height, Anil Dhawan, Nedim Hadzic
Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction commonly seen with myeloproliferative neoplasms (MPNs). Polycythaemia vera (PV) is a very rare MPN in childhood. This is the youngest reported patient diagnosed with PV and BCS secondary to JAK V617F mutation.A 26-month-old girl was admitted with a 5-month history of abdominal distension, hepatosplenomegaly and ascites. Imaging studies revealed occlusion of the right hepatic vein and marked attenuation of the middle and left hepatic veins...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28698883/anti-platelet-factor-4-heparin-antibody-formation-occurs-endogenously-and-at-unexpected-high-frequency-in-polycythemia-vera
#12
Sara C Meyer, Eva Steinmann, Thomas Lehmann, Patricia Muesser, Jakob R Passweg, Radek C Skoda, Dimitrios A Tsakiris
BACKGROUND: Myeloproliferative neoplasms (MPN) encounter thromboses due to multiple known risk factors. Heparin-induced thrombocytopenia (HIT) is a thrombotic syndrome mediated by anti-platelet factor 4 (PF4)/heparin antibodies with undetermined significance for thrombosis in MPN. We hypothesized that anti-PF4/heparin Ab might occur in MPN and promote thrombosis. METHODS: Anti-PF4/heparin antibodies were analyzed in 127 MPN patients including 76 PV and 51 ET. Screening, validation testing, and isotype testing of anti-PF4/heparin Ab were correlated with disease characteristics...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28685840/european-leukemianet-study-on-the-reproducibility-of-bone-marrow-features-in-masked-polycythemia-vera-and-differentiation-from-essential-thrombocythemia
#13
Hans Michael Kvasnicka, Attilio Orazi, Juergen Thiele, Giovanni Barosi, Carlos E Bueso-Ramos, Alessandro M Vannucchi, Robert P Hasserjian, Jean-Jacques Kiladjian, Umberto Gianelli, Richard Silver, Tariq I Mughal, Tiziano Barbui
The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-called masked/prodromal polycythemia vera (mPV) from JAK2-mutated essential thrombocythemia (ET). The six members of the hematopathology panel evaluated 98 BM specimens independently and in a blinded fashion without knowledge of clinical data...
July 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28685257/budd-chiari-syndrome-hepatic-venous-outflow-tract-obstruction
#14
REVIEW
Dominique-Charles Valla
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO). METHODS: Recent literature has been analyzed for this narrative review. RESULTS: Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes...
July 6, 2017: Hepatology International
https://www.readbyqxmd.com/read/28679734/high-prevalence-of-myeloid-neoplasms-in-adults-with-non-langerhans-cell-histiocytosis
#15
Matthias Papo, Eli L Diamond, Fleur Cohen-Aubart, Jean-François Emile, Damien Roos-Weil, Nishant Gupta, Benjamin H Durham, Neval Ozkaya, Ahmet Dogan, Gary A Ulaner, Raajit Rampal, Jean-Emmanuel Kahn, Thomas Sené, Frédéric Charlotte, Baptiste Hervier, Caroline Besnard, Olivier A Bernard, Catherine Settegrana, Nathalie Droin, Zofia Hélias-Rodzewicz, Zahir Amoura, Omar Abdel-Wahab, Julien Haroche
Erdheim-Chester Disease (ECD) is a rare non-Langerhans Cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in BRAF, MAP2K1, and kinases promoting MAP kinase signaling. Due to the relative rarity of ECD, key clinical features of the disease may not be well defined. Across a multi-institutional cohort of 189 patients with ECD and ECD overlapping with Langerhans Cell Histiocytosis (so-called Mixed Histiocytosis (MH)), we identified an unexpected and heretofore undescribed frequent occurrence of myeloid neoplasms amongst patients with ECD and MH...
July 5, 2017: Blood
https://www.readbyqxmd.com/read/28675622/early-radiological-intervention-and-haematology-screening-is-associated-with-excellent-outcomes-in-budd-chiari-syndrome
#16
Allison Mo, Adam Testro, Janine French, Marcus Robertson, Peter Angus, Andrew Grigg
BACKGROUND: Budd Chiari Syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments including pharmacological, radiological and surgical interventions have been used. AIM: We aimed to retrospectively describe our institution's experience with management of patients with BCS. RESULTS: Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1...
July 4, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28675575/clinical-implications-of-transient-myeloproliferative-disorder-in-a-neonate-without-down-syndrome-features
#17
Vickyanne Carruthers, Mario Nicola, Parvathy Venugopal, Christopher N Hahn, Hamish S Scott, Tamas Revesz
No abstract text is available yet for this article.
July 4, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28642604/turning-the-tide-in-myelodysplastic-myeloproliferative-neoplasms
#18
REVIEW
Michael W N Deininger, Jeffrey W Tyner, Eric Solary
Myelodysplastic syndromes/myeloproliferative neoplasms (MDS/MPN) are aggressive myeloid malignancies recognized as a distinct category owing to their unique combination of dysplastic and proliferative features. Although current classification schemes still emphasize morphology and exclusionary criteria, disease-defining somatic mutations and/or germline predisposition alleles are increasingly incorporated into diagnostic algorithms. The developing picture suggests that phenotypes are driven mostly by epigenetic mechanisms that reflect a complex interplay between genotype, physiological processes such as ageing and interactions between malignant haematopoietic cells and the stromal microenvironment of the bone marrow...
June 23, 2017: Nature Reviews. Cancer
https://www.readbyqxmd.com/read/28640953/overview-of-transgenic-mouse-models-of-myeloproliferative-neoplasms-mpns
#19
REVIEW
Andrew Dunbar, Abbas Nazir, Ross Levine
Myeloproliferative neoplasms (MPNs) are a class of hematologic diseases characterized by aberrant proliferation of one or more myeloid lineages and progressive bone marrow fibrosis. In 2005, seminal work by multiple groups identified the JAK2V617F mutation in a significant fraction of MPN patients. Since that time, murine models of JAK2V617F have greatly enhanced the understanding of the role of aberrant JAK-STAT signaling in MPN pathogenesis and have provided an in vivo pre-clinical platform that can be used to develop novel therapies...
June 22, 2017: Current Protocols in Pharmacology
https://www.readbyqxmd.com/read/28637621/gata2-deficiency-and-related-myeloid-neoplasms
#20
Marcin W Wlodarski, Matthew Collin, Marshall S Horwitz
The GATA2 gene codes for a hematopoietic transcription factor that through its two zinc fingers (ZF) can occupy GATA-DNA motifs in a countless number of genes. It is crucial for the proliferation and maintenance of hematopoietic stem cells. During the past 5 years, germline heterozygous mutations in GATA2 were reported in several hundred patients with various phenotypes ranging from mild cytopenia to severe immunodeficiency involving B cells, natural killer cells, CD4(+) cells, monocytes and dendritic cells (MonoMAC/DCML), and myeloid neoplasia...
April 2017: Seminars in Hematology
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