JosefTPrchalMD

PubMed

• Novel germline JAK2mutation causing PV-like erythrocytosis in 3 generations. Amelioration by Ropeg-Interferon.
  Jihyun Song, Lucie Lanikova, Soo Jin Kim, Nicolas Papadopoulos, Stefan N Constantinescu, Brynn Parsegov, Jaroslav F Prchal, Jessica Meznarich, Josef T Prchal> ;American Journal of Hematology. 2024 Apr 17
• Measurement of red cell, plasma and blood volume: Essential components of diagnostic and research studies of oxygen transport.
  Lichtman, M., Prchal, J.> ;Blood Cells, Molecules & Diseases. 2024 Mar 1
• Biomarkers of erythropoiesis response to intravenous iron in a crossover pilot study in unexplained anemia of the elderly.
  Justin J Yoo, Harvey J Cohen, Andrew S Artz, Elizabeth Price, Jeffrey A Fill, Josef Prchal, Shelly Sapp, Huiman Barnhart> ;Hematology. 2023 Dec 1
• 1 citations
  Measurement of red cell, plasma, and blood volume: A perspective.
  Josef T Prchal, Marshall A Lichtman> ;American Journal of Hematology. 2024 Jan 1
• Publisher Correction: Gene expression changes in sickle cell reticulocytes and their clinical associations.
  Xu Zhang, Jihyun Song, Binal N Shah, Jin Han, Taif Hassan, Galina Miasniakova, Adelina Sergueeva, Sergei Nekhai, Roberto F Machado, Mark T Gladwin, Santosh L Saraf, Jo...> ;Scientific Reports. 2023 Sep 14
• Gene expression changes in sickle cell reticulocytes and their clinical associations.
  Xu Zhang, Jihyun Song, Binal N Shah, Jin Han, Taif Hassan, Galina Miasniakova, Adelina Sergueeva, Sergei Nekhai, Roberto F Machado, Mark T Gladwin, Santosh L Saraf, Jo...> ;Scientific Reports. 2023 Aug 8
• 2 citations
  Increased transferrin protects from thrombosis in Chuvash erythrocytosis.
  Binal N Shah, Xu Zhang, Adelina I Sergueeva, Galina Y Miasnikova, Tomas Ganz, Josef T Prchal, Victor R Gordeuk> ;American Journal of Hematology. 2023 Oct 1
• 3 citations
  Increased blood reactive oxygen species and hepcidin in obstructive sleep apnea precludes expected erythrocytosis.
  Jihyun Song, Krishna M Sundar, Monika Horvathova, Radhika Gangaraju, Karel Indrak, Robert D Christensen, Samuel Genzor, Carsten Lundby, Vladimir Divoky, Tomas Ganz, Jo...> ;American Journal of Hematology. 2023 Aug 1
• On either side of homeostasis:gain- and loss-offunction mutations.
  Josef T Prchal, Gregg L Semenza> ;Haematologica. 2023 Oct 1
• 2 citations
  EnvIRONment modifies polycythemia vera.
  Josef T Prchal, Brandi N Reeves> ;Blood. 2023 Apr 27
• 11 citations
  The transcriptional and regulatory identity of erythropoietin producing cells.
  Bjørt K Kragesteen, Amir Giladi, Eyal David, Shahar Halevi, Laufey Geirsdóttir, Olga M Lempke, Baoguo Li, Andreas M Bapst, Ken Xie, Yonatan Katzenelenbogen, Sophie L D...> ;Nature Medicine. 2023 May 1
• "What We Know and What We Do Not Know about Evolutionary Genetic Adaptation to High Altitude Hypoxia in Andean Aymaras".
  Ricardo Amaru, Jihyun Song, N Scott Reading, Victor R Gordeuk, Josef T Prchal> ;Genes. 2023 Mar 3
• Allogeneic stem cell transplant for multiple myeloma & myelofibrosis with split-dose busulfan, fludarabine & cyclophosphamide.
  Andrew D Trunk, Sagar S Patel, Josef T Prchal, Douglas W Sborov, Axel R Zander, Catherine J Lee> ;Leukemia Research Reports. 2023 Jan 1
• 4 citations
  Downregulated KLF2 in polycythemia vera and essential thrombocythemia induces prothrombotic gene expression.
  Jihyun Song, Soo Jin Kim, Jahnavi Gollamudi, Perumal Thiagarajan, Josef T Prchal> ;Blood Advances. 2023 Mar 14
• 4 citations
  Developmental changes in iron metabolism and erythropoiesis in mice with human gain-of-function erythropoietin receptor.
  Barbora Kralova, Lucie Sochorcova, Jihyun Song, Ondrej Jahoda, Katarina Hlusickova Kapralova, Josef T Prchal, Vladimir Divoky, Monika Horvathova> ;American Journal of Hematology. 2022 Oct 1
• 36 citations
  A randomized phase 3 trial of interferon-α vs hydroxyurea in polycythemia vera and essential thrombocythemia.
  John Mascarenhas, Heidi E Kosiorek, Josef T Prchal, Alessandro Rambaldi, Dmitriy Berenzon, Abdulraheem Yacoub, Claire N Harrison, Mary Frances McMullin, Alessandro M V...> ;Blood. 2022 May 12
• Perspective: Pivotal translational hematology and therapeutic insights in chronic myeloid hematopoietic stem cell malignancies.
  Tariq I Mughal, Naveen Pemmaraju, Rafael Bejar, Robert P Gale, Prithviraj Bose, Jean-Jacques Kiladjian, Josef Prchal, Daniel Royston, Daniel Pollyea, Peter Valent, Tim...> ;Hematological Oncology. 2022 Oct 1
• Summary of Joint European Hematology Association (EHA) and EuroBloodNet Recommendations on Diagnosis and Treatment of Methemoglobinemia.
  Achille Iolascon, Immacolata Andolfo, Roberta Russo, Wilma Barcellini, Elisa Fermo, Gergely Toldi, Stefano Ghirardello, Davis Rees, Richard van Wijk, Antonis Kattamis,...> ;Hemasphere. 2021 Nov 17
• 6 citations
  Tissue factor activity is increased in neutrophils from JAK2 V617F-mutated essential thrombocythemia and polycythemia vera patients.
  Brandi Reeves, Soo Jin Kim, Jihyun Song, Kathryn Wilson, Michael W. Henderson, Nigel S. Key, Rafal Pawlinski, Josef T. Prchal> ;American Journal of Hematology. 2021 Nov 8
• 2 citations
  Thirty-five males with severe (Class 1) G6PD deficiency (c.637G>T) in a North American family of European ancestry.
  Timothy M. Bahr, Archana M. Agarwal, Jessica Meznarich, Wende L. Prince, Tirzah W.P. Wait, Josef T. Prchal, Robert D. Christensen> ;Blood Cells, Molecules & Diseases. 2021 Nov 4
• 4 citations
  Safety and Efficacy of Ruxolitinib in Patients With Myelofibrosis and Low Platelet Counts (50–100 × 109/L): Final Analysis of an Open-label Phase 2 study
  Moshe Talpaz, Josef T. Prchal, Lawrence B. Afrin, Murat O. Arcasoy, Solomon I. Hamburg, Jason Clark, Deanna Kornacki, Philomena Colucci, Srdan Verstovsek> ;Clinical Lymphoma, Myeloma & Leukemia. 2021 Nov 2
• 10 citations
  α-Ketoglutarate Inhibits Thrombosis and Inflammation by Prolyl Hydroxylase-2 Mediated Inactivation of Phospho-Akt.
  Nishith M Shrimali, Sakshi Agarwal, Simrandeep Kaur, Sulagna Bhattacharya, Sankar Bhattacharyya, Josef T. Prchal, Prasenjit Guchhait> ;Ebiomedicine. 2021 Nov 2
• 2 citations
  Anticoagulation management post-transjugular intrahepatic portosystemic shunt in portal hypertension associated with myeloproliferative neoplasms.
  Mouhamed Yazan Abou-Ismail, Josef T. Prchal, Michael W. Deininger, Charles J. Parker, Ming Y. Lim> ;Blood Coagulation & Fibrinolysis. 2021 Oct 4
• 1 citations
  Novel, de novo, beta-globin variant with decreased oxygen affinity (HBB:c.317T>A, "Hemoglobin St. George") in a healthy child with low oxygen saturations and anemia.
  Jessica Meznarich, Anton Rets, Archana M. Agarwal, Robert D. Christensen, N. Scott Reading, Frans A. Kuypers, Josef T. Prchal> ;American Journal of Hematology. 2021 Sep 29
• 39 citations
  Recommendations for diagnosis and treatment of methemoglobinemia
  Achille Iolascon, Paola Bianchi, Immacolata Andolfo, Roberta Russo, Wilma Barcellini, Elisa Fermo, Gergely Toldi, Stefano Ghirardello, Davis Rees, Richard van Wijk, An...> ;American Journal of Hematology. 2021 Sep 23
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Journal Articles

• Hypoxia and Thrombosis  
  Josef Prchal, MD, Blood
• Effects of hydroxyurea on frequency of painful crises in SCD.  
  Charache, S et al, N Engl J Med
• Triosephosphate Isomerase Deficiency: Repetitive Occurrence of Point Mutation in Amino Acid 104 in Multiple Apparently Unrelated Families.  
  Schneider A, Westwood B, Yim C, Prchal JT, Berkow R, Labotka R, Warrier R, Beutler, E, Amer J Hematology
• Clonal Stability analysis of all blood lineages indicated by X-Chromosomal transcriptional polymorphism.  
  Prchal JT, Prchal JF, Belickova M, Chen S, Guan YL, Gartland L, Cooper MD, J Exp Med
• Sickle Cell Acute Chest Syndrome Associated with Parvovirus B19 Infection: Case Series and Review.  
  Elizabeth A. Lowenthal, D.O., Alan Wells, M.D., DMS, Peter D. Emanuel, M.D., Rick Player, M.D.and Prchal, JT, Amer J Hemat
• Benign erythrocytosis’ and other familial and congenital polycythemias (invited Editorial).  
  Prchal JT, Sokol L, Eur. J. Haematol
• Unusual Patterns of Exon Skipping in Bruton Tyrosine Kinase Are Associated with Mutations Involving the Intron 17 3’ Splice Site.  
  Haire RN, Ohta Y, Strong SJ, Litman RT, Liu Y, Prchal JT, Cooper MD, Litman GW, Am J Hum Genet
• Rapid Determination of Clonality by Detection of Two Closely-Linked X-Chromosome Exonic Polymorphisms Using Allele-Specific PCR.  
  Liu Y, Phelan JT, Go RCP, Prchal JF, Prchal JT, Journal of Clinical Investigation
• Angiotensin II Facilitates Erythropoietin Mediated Proliferation of Normal Early Erythroid Progenitors.  
  Mrug M, Stopka T, Julian BA, Prchal JF, Prchal JT, J. Clin. Invest
• Absence of Polycythemia Phenotype in a Child in with a unique EPO Receptor Mutation.  
  Kralovics R, Sokol L, Prchal JT, J Clin Invest
• Losartan, Angiotensin II Type I receptor antagonist, lowers hematocrit in possttransplant erythrocytosis.  
  Julian BA, Brantley RR, Barker CV, Stopka T, Gaston RS, Curtis JJ, Lee JY, Prchal JT, J Am Soc Nephrol
• A Polymorphism of the X-Linked Gene IDS Increases the Number of Females Informative for Transcriptional Clonality Assays.  
  Xylina T. Gregg, Robert Kralovics, and Josef T. Prchal, Am J Hemat
• Non-anemic Homozygous o Thalassemia in an African-American Family: Association of High Fetal Hemoglobin Levels with Thalassemia Alleles.  
  Vladimir Divoky, Michal Mrug, Denyse Thornley-Brown, Martina Divoka, and Josef T. Prchal, Am J Hemat
• Genetic heterogeneity of primary familial and congenital polycythemia.  
  Kralovics R, Prchal JT, Am J Hemat
• Mutations of von Hippel-Lindau tumor suppressor gene and congenital polycythemia.  
  Pastore Y, Jedlickova K, Guan YL, Liu E, James Fahner J, Hasle H, Prchal JF , and Prchal JT, Am J Hum Genet
• The in vitro bleeding time.  
  Blakely JA, Prchal JT, Glynn M, J Lab Clin Med
• Gamma-heavy chain disease in a young man.  
  Pruzanski W, Prchal JT, Chan E, Parr D, Annals of the Royal College of Physicians & Surgeons of Canada
• Erythropoietin and erythropoiesis: polycythemias due to disruption of oxygen homeostasis.  
  Prchal JT and Pastore YD, Hematology J
• Urinary acidification in a patient with glucose-6-phosphate dehydrogenase deficiency: A reevaluation of the role of the hexose monophosphate shunt in renal acid secre...  
  Rostand SG, Culpepper RM, Prchal JT, Amer J of Med
• CNS extramedullary blast transformation in idiopathic myelofibrosis.  
  Barnes HM, Prchal JT, Scott CW, Amer J. Hemat
• Cataracts and riboflavin deficiency.  
  Skalka H, Prchal J, American Journal of Clinical Nutrition
• Self-association of spectrin in abnormal red blood cells.  
  Liu SC, Palek J, Prchal J, Castleberry R, J Supramol Struct Cell Biochem
• Two siblings with unusually mild homozygous ß-thalassemia: a didactic example of the effect of a non allelic modifier gene on the expressivity of a monogenic disorder.  
  Prchal J, Papayannopoulou T, Stamatoyannopoulos G, Amer J Med Genetics
• Altered spectrin dimer-dimer association and erythrocyte membrane skeleton instability in hereditary pyropoikilocytosis.  
  Liu SC, Palek J, Prchal J, Castleberry RP, J of Clin Invest
• HIF-1alpha -deficiency results in dysregulated EPO signaling and iron homeostasis in mouse development.  
  Yoon D, Pastore YD, Divoky V, Liu E, Mlodnicka AE, Rainey K, Ponka P, Semenza GL, Schumacher A, Prchal JT, J Biol Chem
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Books/Book Chapters

• Alterations in the NK-Cell population: Phenotypic Heterogeneity of Granular Lymphocytes in Lymphoproliferative Disorders. 
  Chapter: Contributions of Modern Biology to Medicine 
  Page 139 - 149Grossi CE, Velardi A, Prchal JT, Cooper MDEditors: U. Bertazzoni, F.J. Bollum, M. Ghione.Raven Press, Serono Symposia

Abstracts/Posters

• Hypoxia Inducible FactorsÍ Transcriptional Activity in Polycythemia Vera
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/9/2019
• Results from a Phase 2 Study of Navitoclax in Combination with Ruxolitinib in Patients with Primary or Secondary Myelofibrosis
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/9/2019
• Pegylated Interferon-Alfa Suppresses Clonal Hematopoiesis in Polycythemia Vera and Essential Thrombocythemia and Is Associated with Immune Responses to Tumor Antigens
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/7/2019
• Hematological Changes in Chronic Sustained Hypoxia and Chronic Intermittent Hypoxia in a Mouse Model
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/9/2019
• Final Results of Prospective Treatment with Pegylated Interferon Alfa-2a for Patients with Polycythemia Vera and Essential Thrombocythemia in First and Second-Line Set...
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/8/2019
• Normal Hemoglobin Concentrations in Obstructive Sleep Apnea and Associated Neocytolysis-Mediated Hemolysis and Inflammation Mediated Suppression of Expected Elevated H...
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/9/2019
• Hypoxia Dependent and Independent Dysregulation of the Transcriptome in Sickle Cell Anemia
  Josef T. Prchal, 61st Annual American Society of Hematology Meeting, Orlando, FL, 12/8/2019
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Lectures

• Tibetan Enriched PKLR Variant Is Beneficial to High Altitude Adaption By Improving Oxygen Delivery 
  2018 ASH Annual Meeting - San Diego, CA - 12/1/2018
• Pathophysiology of Obstructive Sleep Apnea (OSA) - Blood Cells` Reactive Oxygen Species and Inflammation Prevent Polycythemia 
  2018 ASH Annual Meeting - San Diego, CA - 12/1/2018
• Lunch Meeting and Case presentations with Dr Charles Chuah and Haematology Group at SGH, host Drs. 
  David Virshup and Charles Chuah, University of Singapore
• Germline and Somatic Mutations Causing Polycythemic States. 
  Prof. Daniel G. TENEN, Cancer Science Institute of Singapore
• Philadelphia Chromosome Negative Myeloproliferative Disorders in 2009. Pathogenesis and Treatment Guidelines. 
  Hong Medical School, host Dr. James Chim
• Germline and Somatic Mutations Causing Polycythemias, with emphasis on Tibetan adaptation to hypoxia. 
  Xining Chinese Institute of High altitude Medicine
• Clinical Features and Molecular basis of Myeloproliferative disorders. 
  Monday-Wednesday Beijing and Tianjien Hematology Hospital, hosts president PUMC LINg
• Many Genes, Many Phenotypes. 
  Zhejiang University School of Medicine in Hangzhou
• Many Genes, Many Phenotypes. Third Annual Arthur Haut Lecture, and Medical Grand Round. 
  University of Arkansas, Little Rock, AK
• Inherited and Somatic Mutations causing Polycythemias. 
  NYUSM, New York
• Lunch Debate: Is the new WHO classification of MPD a blessing? 
  Opponent Dr. Aylew Tefferi, Berlin, Germany
• Where are We Now? 
  Turkish Hematology Society, Anatalya Turkey
• Germline and somatic mutations in polycythemia vera. 
  5th International meeting on Myeloproliferative and Myelodysplastic disorders, Brooklyn NewYork
• Is JAK2 the Whole Story? 
  Chicago
• Molecular markers of MPNs. 
  Association for Molecular Pathology, Orlando, FL
• Is JAK2 V617F whole story in PV? 
  Fourth International Workshop on CML and MPN, Natchez, Louisiana
• Germline and Somatic Genomic Changes in Polycythemia Vera. 
  John Hopkins University Baltimore
• Hypoxia and Polycythemia. 
  West China University and Tibetan Hospital
• Congenital and acquired Polycythemic Disorders. 
  Department of Hematology, The Third Hospital, Peking University, Beijing
• Biomoarkers of polycythemia vera. 
  Genomic Medicine, Troy Michigan
• Is JAK2 the Whole Story in polycythemia vera? 
  Albufeira, Portugal
• Genetic Regulation of Erythropoiesis. 
  Jerusalem, Israel
• Germline and Somatic Mutations Causing Polycythemias. 
  Myrexis Pharmaceuticals: MPDs
• JAK2 mutation in Polycythemia. Is that the whole Story? 
  Marriott SLC
• JAK2V617 Mutation in PV. 
  Salt Lake City, Utah
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Other

• A Frameshift Mutation in the Erythropoietin Receptor Gene and Increased Sensitivity of Erythroid Progenitors to Erythropoietin. 
  Sokol L, Luhovy M, Prchal JF, Guan YL, Semenza GL, Prchal JT
• Current Opinion in Hematology. 
  Prchal JT
• A Taq l Polymorphism in the Human Erythroid ß Spectrin Gene.Human Genetics. 
  Beeton L., Prchal JT, Coetzer TL
• Familial Polycythemia, Science. 
  Prchal JT, Prchal JF, Semenza GL, Sokol L
• Hb Bibba (a2136(h19)LEU-PROb2) in a Caucasian family from Alabama. 
  Prchal JT, Adler B, Wilson JB, Kazaznetz EG, Huisman,THT
• A novel clonality assay based on transcriptional polymorhism of X chromosome gene p55. 
  Luhovy M, Yunying Liu, Belickova M, Prchal JF, Prchal JT
• Flt3 Ligand Plasma/Serum Levels are Low in Normal Individuals and are Highly Elevated in Patients with Fanconi Anemia and Acquired Aplastic Anemia. 
  Lyman SD, Seaberg M, Hanna R, Zappone JD, Brasel k, Abkowitz JL, Prchal JT, Schultz JC, Shahidi NT
• Design of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. 
  Charache S., at.el
• Stable transduction of recombinant adeno-associated virus into hematopoietic stem cells from normal and sickle cell patients. 
  Luhovy M, McCune S, Dong JY, Guan YL, Prchal JF, Townes, TM, Prchal JT
• A novel mutation found in the 3’ domain of NADH-cytochrome b5 reductase in an African-American family with type 1 congenital methemoglobinemia. 
  Jenkins MM, and Prchal JT
• Melrose, V Brabec, and J Palek Characterization of 13 novel band 3 gene defects in hereditary spherocytosis with band 3 deficiency. 
  P Jarolim, JL Murray, HL Rubin, WM Taylor, JT Prchal, SK Ballas, LM Snyder, L Chrobak, WD
• Terrin, Samuel Charache, George J. Dover, and the Multicenter Study of Hydroxyurea Fetal Hemoglobin in Sickle Cell Anemia: Determinants of Response to Hydroxyurea. 
  Martin H. Steinberg, Zhi-Hong Lu, Franca B. Barton, Michael L
• A high frequency polymorphism of NADH-cytochrome b5 reductase in African-Americans. 
  Jenkins, M M,.and Prchal, JT
• Congenital Polycythemia in Chuvashia. 
  Sergeyeva A, Gordeuk VR, Tokarev YN, Sokol L, Prchal JF, Prchal JT
• . Erythropoietin Receptor Mutations and Human Disease. 
  Gregg XT, Prchal JT
• Fetal hemoglobin in Sickle Cell Anemia: Determinants of response to Hydroxyurea. 
  Steinberg M, Lu ZH, Barton FB, Terrin ML, Charache S et al
• Clinical features, diagnosis, and treatment of methemoglobinemia 
  Prchal JT
  http://www.uptodate.com/contents/clinical-features-diagnosis-and-treatment-of-methemoglobinemia
  UpToDate, Wolters Kluwer Health - 2012-05-30
• Genetics and pathogenesis of methemoglobinemia 
  Prchal JT
  http://www.uptodate.com/contents/genetics-and-pathogenesis-of-methemoglobinemia
  UpToDate, Wolters Kluwer Health - 2012-08-22
• Trilineage Extramedullary Myeloid Cell Tumor in Myelodysplastic Syndrome. 
  Hancock JC, Prchal JT, Bennett JM, Linstinsky CM
• Two new EPO receptor mutations: Truncated EPO receptors are most frequently associated with primary familial and congenital polycythemias. 
  Kralovics R, Indrak K, Stopka T, Berman BW, Prchal JF, Prchal JT
• The erythropoietin receptor gene is not linked with the polycythemia phenotype in a family with autosomal dominant primary polycythemia. 
  Kralovics R, Sokol L, Broxson E, Prchal JT
• Human Hematopoietic Progenitors Express Erythropoietin. 
  T. Stopka, J.H. Zivny, P. Stopkova, J.F. Prchal and J.T. Prchal
• Guinea Pig Serum Erythropoietin (EPO) Selectively Stimulates Guinea Pig Erythroid Progenitors: Human or Mouse Erythroid Progenitors Do Not Form BFU-E Colonies in Respo... 
  T Stopka,, E Necas, JH Zivny, E Goldwasser, JF Prchal and JT Prchal
• Full-length human L1 insertion retain the capacity for high frequency retrotransposition in cultured cells. 
  Kimberland ML, Divoky V, Prchal JT, Schwahn U, Berger W, and Kazazian HG
• Congenital and Inherited Polycythemia. 
  Kralovics R, Prchal JT
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