Peter Valent

Last updated

Peter Valent
Born (1962-10-09) 9 October 1962 (age 60)
NationalityAustrian
Alma mater Medical University of Vienna
Scientific career
Fields Hematology

Peter Valent (born 9 October 1962 in Vienna, Austria) is an Austrian hematologist and stem cell researcher. Since 1990 he leads a research group at the Medical University of Vienna. From 2002 he coordinates the European Competence Network on Mastocytosis and since 2008 he is Scientific Director of the Ludwig Boltzmann Institute for Hematology and Oncology of the Ludwig Boltzmann Society in Austria. [1]

Contents

Life and education

Valent studied medicine at the Medical University of Vienna and graduated in 1987. He is a specialist in internal medicine and hematology and was promoted to Assistant Professor for Experimental Hematology in 1992 and Associate Professor for Internal Medicine in 1995. Valent visited several universities in Germany as Guest Scientist, including the Institute of Pathology of the University of Tübingen, the University of Schleswig-Holstein (Campus Lübeck) and the LMU Munich. [2]

Scientific contributions

Mast cell research and the classification of mastocytosis

Between 1989 and 1999 Valent examined the phenotype and growth characteristics of human mast cells. [3] He found that mast cells form a unique lineage in the hematopoietic cell system. [4] [5] In subsequent studies, neoplastic mast cells were characterized. [6] [7] The resulting data contributed essentially to the development of diagnostic criteria and the WHO classification of mastocytosis. [8] In 2000, Valent organized a Working Conference on Mastocytosis to implement this classification together with an international consensus group. [9] Since 2002 Valent coordinates the European Competence Network on Mastocytosis that expanded rapidly and provides a useful basis for the development and conduct of studies and activities in the field of mastocytosis. [10] In the forthcoming years, the diagnostic WHO criteria of mastocytosis were validated, adjusted and extended. [11] To discuss these developments, Valent and his team organized additional conferences in Vienna, including a Working Conference on Standards and Standardization of Diagnostic Criteria and Therapies in Mastocytosis [12] and one on the Global Classification of Mast Cell Disorders and Mast Cell Activation Syndromes (2010). [13]

Additional scientific contributions

One additional focus in Valent´s research is the neoplastic stem cell, also termed cancer stem cell in the context of cancer and leukemic stem cell in leukemia contexts. Valent investigates the phenotype of these cells in various hematologic neoplasms and develops concepts predicting the step-wise development of these cells from normal stem cells. The major aim in his research is to identify molecular targets in these cells and to develop more effective (curative) therapies by eliminating these cells in various blood cell disorders, including Acute Myeloid Leukemia, Chronic Myeloid Leukemia, [14] Systemic Mastocytosis [15] and Myelodysplastic Syndromes. [16] [17] These studies are primarily conducted in the Ludwig Boltzmann Institute for Hematology and Oncology at the Medical University of Vienna. [18]

Valent is a member of numerous scientific organizations and has published over 850 publications since 1988, including more than 500 original papers, over 200 review articles and numerous book contributions. He also published numerous textbook chapters. In 2001, 2008 and 2016 he drew book chapters on mastocytosis as the author of the WHO. Peter Valent is one of the most frequently cited scientists from German-speaking countries in the field of immunology from 2011 to 2015. In Austria he is one of the top 5 scientists in the field of medicine (as of June 2022 - https://research.com /scientists-rankings/medicine/at). In total, his work has been cited more than 35,000 times by March 2019 and his h-index is 103 (as of April 2022). Peter Valent is currently ranked #777 among the world's top scientists in Biology and Biochemistry as of April 2022, and #913 among the world's top scientists in Medicine as of June 2022 - https://research.com/scientists- rankings/medicine).

Awards

Valent received several national and international awards, including the Karl Landsteiner-Award of the Austrian Society for Allergy and Immunology, the Paracelsus Award of the Austrian Society for Internal Medicine, the Wilhelm Türk Award of the Austrian Society for Hematology and Oncology, the Theodor-Billroth Medal of the Austrian Medical Association, the Mac Forster Award of the European Society for Clinical Investigation and the Middle European Award for Interdisciplinary Cancer Research. [19]

Related Research Articles

<span class="mw-page-title-main">Mastocytosis</span> Medical condition

Mastocytosis, a type of mast cell disease, is a rare disorder affecting both children and adults caused by the accumulation of functionally defective mast cells and CD34+ mast cell precursors.

<span class="mw-page-title-main">Myelodysplastic syndrome</span> Diverse collection of blood-related cancers

A myelodysplastic syndrome (MDS) is one of a group of cancers in which immature blood cells in the bone marrow do not mature, and as a result, do not develop into healthy blood cells. Early on, no symptoms typically are seen. Later, symptoms may include fatigue, shortness of breath, bleeding disorders, anemia, or frequent infections. Some types may develop into acute myeloid leukemia.

Aplastic anemia (AA) is a severe hematologic condition in which the body fails to make blood cells in sufficient numbers. Aplastic anemia is associated with cancer and various cancer syndromes. Blood cells are produced in the bone marrow by stem cells that reside there. Aplastic anemia causes a deficiency of all blood cell types: red blood cells, white blood cells, and platelets.

<span class="mw-page-title-main">Eosinophilia</span> Blood condition

Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5×108/L (500/μL). Hypereosinophilia is an elevation in an individual's circulating blood eosinophil count above 1.5 × 109/L (i.e. 1,500/μL). The hypereosinophilic syndrome is a sustained elevation in this count above 1.5 × 109/L (i.e. 1,500/μL) that is also associated with evidence of eosinophil-based tissue injury.

<span class="mw-page-title-main">Mast cell</span> Cell found in connective tissue

A mast cell is a resident cell of connective tissue that contains many granules rich in histamine and heparin. Specifically, it is a type of granulocyte derived from the myeloid stem cell that is a part of the immune and neuroimmune systems. Mast cells were discovered by Paul Ehrlich in 1877. Although best known for their role in allergy and anaphylaxis, mast cells play an important protective role as well, being intimately involved in wound healing, angiogenesis, immune tolerance, defense against pathogens, and vascular permeability in brain tumors.

<span class="mw-page-title-main">Tumors of the hematopoietic and lymphoid tissues</span> Tumors that affect the blood, bone marrow, lymph, and lymphatic system

Tumors of the hematopoietic and lymphoid tissues or tumours of the haematopoietic and lymphoid tissues are tumors that affect the blood, bone marrow, lymph, and lymphatic system. Because these tissues are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making aplasia, myeloproliferation and lymphoproliferation closely related and often overlapping problems. While uncommon in solid tumors, chromosomal translocations are a common cause of these diseases. This commonly leads to a different approach in diagnosis and treatment of hematological malignancies. Hematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. In some centers "hematology/oncology" is a single subspecialty of internal medicine while in others they are considered separate divisions. Not all hematological disorders are malignant ("cancerous"); these other blood conditions may also be managed by a hematologist.

<span class="mw-page-title-main">Lenalidomide</span> Pair of enantiomers

Lenalidomide, sold under the brand name Revlimid among others, is a medication used to treat multiple myeloma, smoldering myeloma, and myelodysplastic syndromes (MDS). For multiple myeloma, it is used after at least one other treatment and generally with dexamethasone. It is taken by mouth.

<span class="mw-page-title-main">Tryptase</span> Class of enzymes

Tryptase is the most abundant secretory granule-derived serine proteinase contained in mast cells and has been used as a marker for mast cell activation. Club cells contain tryptase, which is believed to be responsible for cleaving the hemagglutinin surface protein of influenza A virus, thereby activating it and causing the symptoms of flu.

<span class="mw-page-title-main">Chronic myelomonocytic leukemia</span> Medical condition

Chronic myelomonocytic leukemia (CMML) is a type of leukemia, which are cancers of the blood-forming cells of the bone marrow. In adults, blood cells are formed in the bone marrow, by a process that is known as haematopoiesis. In CMML, there are increased numbers of monocytes and immature blood cells (blasts) in the peripheral blood and bone marrow, as well as abnormal looking cells (dysplasia) in at least one type of blood cell.

<span class="mw-page-title-main">KIT (gene)</span> Mammalian protein and protein-coding gene

Proto-oncogene c-KIT is the gene encoding the receptor tyrosine kinase protein known as tyrosine-protein kinase KIT, CD117 or mast/stem cell growth factor receptor (SCFR). Multiple transcript variants encoding different isoforms have been found for this gene. KIT was first described by the German biochemist Axel Ullrich in 1987 as the cellular homolog of the feline sarcoma viral oncogene v-kit.

Juvenile myelomonocytic leukemia (JMML) is a rare form of chronic leukemia that affects children, commonly those aged four and younger. The name JMML now encompasses all diagnoses formerly referred to as juvenile chronic myeloid leukemia (JCML), chronic myelomonocytic leukemia of infancy, and infantile monosomy 7 syndrome. The average age of patients at diagnosis is two (2) years old. The World Health Organization has included JMML as a subcategory of myelodysplastic and myeloproliferative disorders.

<span class="mw-page-title-main">Mast cell leukemia</span> Medical condition

Mast cell leukemia is an extremely aggressive subtype of acute myeloid leukemia that usually occurs de novo but can, rarely, evolve from transformation of chronic myeloid leukemia into the more aggressive acute myeloid leukemia. In a small proportion of cases, acute mast cell leukemia may evolve from a more progressive form of systemic mastocytosis. The diagnosis of acute mast cell leukemia by the WHO criteria includes the requirement for a prevalence of 20% neoplastic mast cells in marrow and 10% in blood. If the mast cells represent less than 10% of blood cells, the tumor is called "aleukemic" mast cell leukemia.

Ulrich Rudolph Mahlknecht is an internationally renowned German/Italian physician scientist.

<span class="mw-page-title-main">Midostaurin</span> Chemical compound

Midostaurin, sold under the brand name Rydapt & Tauritmo both by Novartis, is a multi-targeted protein kinase inhibitor that has been investigated for the treatment of acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and advanced systemic mastocytosis. It is a semi-synthetic derivative of staurosporine, an alkaloid from the bacterium Streptomyces staurosporeus.

Mast cell activation syndrome (MCAS) is a term referring to one of two types of mast cell activation disorder (MCAD); the other type is idiopathic MCAD. MCAS is an immunological condition in which mast cells inappropriately and excessively release chemical mediators, resulting in a range of chronic symptoms, sometimes including anaphylaxis or near-anaphylaxis attacks. Primary symptoms include cardiovascular, dermatological, gastrointestinal, neurological and respiratory problems.

Azra Raza is the Chan Soon-Shiong Professor of Medicine and Director of Myelodysplastic Syndrome (MDS) Center at Columbia University. She has previously held positions at Roswell Park Comprehensive Cancer Center, University of Cincinnati, Rush University, and the University of Massachusetts. Raza's research focuses on myelodysplastic syndrome and acute myeloid leukemia.

Clonal hypereosinophilia, also termed primary hypereosinophilia or clonal eosinophilia, is a grouping of hematological disorders all of which are characterized by the development and growth of a pre-malignant or malignant population of eosinophils, a type of white blood cell that occupies the bone marrow, blood, and other tissues. This population consists of a clone of eosinophils, i.e. a group of genetically identical eosinophils derived from a sufficiently mutated ancestor cell.

<span class="mw-page-title-main">Emberger syndrome</span> Medical condition

The Emberger syndrome is a rare, autosomal dominant, genetic disorder caused by familial or sporadic inactivating mutations in one of the two parental GATA2 genes. The mutation results in a haploinsufficiency in the levels of the gene's product, the GATA2 transcription factor. This transcription factor is critical for the embryonic development, maintenance, and functionality of blood-forming, lympathic-forming, and other tissues. The syndrome includes as its primary symptoms: serious abnormalities of the blood such as the myelodysplastic syndrome and acute myeloid leukemia; lymphedema of the lower limbs, and sensorineural hearing loss. However, the anomalies caused by GATA2 mutations are highly variable with some individuals showing little or no such symptoms even in old age while others exhibit non-malignant types of hematological anomalies; lymphedema in areas besides the lower limbs, little or no hearing loss; or anomalies in other tissues. The syndrome may present with relatively benign signs and/or symptoms and then progress rapidly or slowly to the myelodysplastic syndrome and/or acute myeloid leukemia. Alternatively, it may present with one of the latter two life-threatening disorders.

GATA2 deficiency is a grouping of several disorders caused by common defect, namely, familial or sporadic inactivating mutations in one of the two parental GATA2 genes. Being the gene haploinsufficient, mutations that cause a reduction in the cellular levels of the gene's product, GATA2, are autosomal dominant. The GATA2 protein is a transcription factor critical for the embryonic development, maintenance, and functionality of blood-forming, lymphatic-forming, and other tissue-forming stem cells. In consequence of these mutations, cellular levels of GATA2 are deficient and individuals develop over time hematological, immunological, lymphatic, or other presentations that may begin as apparently benign abnormalities but commonly progress to severe organ failure, opportunistic infections, virus infection-induced cancers, the myelodysplastic syndrome, and/or leukemia. GATA2 deficiency is a life-threatening and precancerous condition.

<span class="mw-page-title-main">Transfusion-dependent anemia</span>

Transfusion-dependent anemia is a form of anemia characterized by the need for continuous blood transfusion. It is a condition that results from various diseases, and is associated with decreased survival rates. Regular transfusion is required to reduce the symptoms of anemia by increasing functional red blood cells and hemoglobin count. Symptoms may vary based on the severity of the condition and the most common symptom is fatigue. Various diseases can lead to transfusion-dependent anemia, most notably myelodysplastic syndromes (MDS) and thalassemia. Due to the number of diseases that can cause transfusion-dependent anemia, diagnosing it is more complicated. Transfusion dependence occurs when an average of more than 2 units of blood transfused every 28 days is required over a period of at least 3 months. Myelodysplastic syndromes is often only diagnosed when patients become anemic, and transfusion-dependent thalassemia is diagnosed based on gene mutations. Screening for heterozygosity in the thalassemia gene is an option for early detection.

References

  1. "Peter Valent". Ludwig Boltzmann Institute. 10 December 2008. Retrieved 13 March 2020.
  2. "Peter Valent - Scientific CV". Medizinische Universität Wien. Retrieved 13 March 2020.
  3. Valent, Peter; Bettelheim, P. (1992). "Cell Surface Structures on Human Basophils and Mast Cells: Biochemical and Functional Characterization". Advances in Immunology Volume 52. Vol. 52. pp. 333–423. doi:10.1016/s0065-2776(08)60879-2. ISBN   9780120224524. PMID   1332448.
  4. Valent, Peter; Bettelheim, P. (1992). "Cell Surface Structures on Human Basophils and Mast Cells: Biochemical and Functional Characterization". Advances in Immunology Volume 52. Vol. 52. pp. 333–423. doi:10.1016/s0065-2776(08)60879-2. ISBN   9780120224524. PMID   1332448.
  5. Valent, Peter (1994). "The riddle of the mast cell: kit(CD117)-ligand as the missing link? Immunol Today.". Immunology Today. 15 (3): 111–114. doi:10.1016/0167-5699(94)90153-8. PMID   7513517.
  6. Valent, Peter; Schernthaner, GH; Sperr, WR; Fritsch, G; Agis, H; Willheim, M; Bühring, HJ; Orfao, A; Escribano, L (2001). "Variable expression of activation-linked surface antigens on human mast cells in health and disease.". Immunol Rev. 179 (179): 74–81. doi:10.1034/j.1600-065x.2001.790108.x. PMID   11292030. S2CID   45298998.
  7. Valent, Peter; Cerny-Reiterer, S; Herrmann, H; Mirkina, I; George, TI; Sotlar, K; Sperr, WR; Horny, HP (2010). "Phenotypic heterogeneity, novel diagnostic markers, and target expression profiles in normal and neoplastic human mast cells". Best Pract Res Clin Haematol. 23 (3): 369–378. doi:10.1016/j.beha.2010.07.003. PMID   21112036.
  8. Valent, Peter; Horny, HP; Escribano, L; Longley, BJ; Li, CY; Schwartz, LB; Marone, G; Nuñez, R; Akin, C; Sotlar, K; Sperr, WR; Wolff, K; Parwaresch, RM; Brunning, RD; Austen, KF; Lennert, K; Metcalfe, DD; Vardiman, JW; Bennett, JM (2001). "Diagnostic criteria and classification of mastocytosis: a consensus proposal". Leuk Res. 25 (7): 603–625. doi:10.1016/s0145-2126(01)00038-8. PMID   11377686.
  9. Valent, Peter (1 June 2010). "Classification and response criteria in mastocytosis: is there a need to revise?". Expert Review of Hematology. 3 (3): 247–249. doi:10.1586/ehm.10.20. ISSN   1747-4086. PMID   21082975. S2CID   36767409.
  10. Valent, Peter; Arock, Michel; Bonadonna, Patrizia; Brockow, Knut; Broesby-Olsen, Sigurd; Escribano, Luis; Gleixner, Karoline V.; Grattan, Clive; Hadzijusufovic, Emir; Hägglund, Hans; Hermine, Olivier (1 December 2012). "European Competence Network on Mastocytosis (ECNM): 10-year jubilee, update, and future perspectives". Wiener klinische Wochenschrift. 124 (23–24): 807–814. doi:10.1007/s00508-012-0293-z. ISSN   0043-5325. PMID   23179435. S2CID   940450.
  11. "Peter VALENT, MD". PhD MCCA. Retrieved 8 April 2020.
  12. Valent, P.; Akin, C.; Escribano, L.; Födinger, M.; Hartmann, K.; Brockow, K.; Castells, M.; Sperr, W. R.; Kluin-Nelemans, H. C.; Hamdy, N. A. T.; Lortholary, O. (1 June 2007). "Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria". European Journal of Clinical Investigation. 37 (6): 435–453. doi: 10.1111/j.1365-2362.2007.01807.x . ISSN   0014-2972. PMID   17537151.
  13. Valent, Peter; Akin, Cem; Arock, Michel; Brockow, Knut; Butterfield, Joseph H.; Carter, Melody C.; Castells, Mariana; Escribano, Luis; Hartmann, Karin; Lieberman, Philip; Nedoszytko, Boguslaw (2012). "Definitions, Criteria and Global Classification of Mast Cell Disorders with Special Reference to Mast Cell Activation Syndromes: A Consensus Proposal". International Archives of Allergy and Immunology. 157 (3): 215–225. doi:10.1159/000328760. ISSN   1423-0097. PMC   3224511 . PMID   22041891.
  14. Valent, Peter; Sadovnik, Irina; Eisenwort, Gregor; Bauer, Karin; Herrmann, Harald; Gleixner, Karoline V.; Schulenburg, Axel; Rabitsch, Werner; Sperr, Wolfgang R.; Wolf, Dominik (29 August 2019). "Immunotherapy-Based Targeting and Elimination of Leukemic Stem Cells in AML and CML". International Journal of Molecular Sciences. 20 (17): 4233. doi: 10.3390/ijms20174233 . ISSN   1422-0067. PMC   6747233 . PMID   31470642.
  15. Valent, Peter; Sperr, Wolfgang R.; Akin, Cem (23 December 2010). "How I treat patients with advanced systemic mastocytosis". Blood. 116 (26): 5812–5817. doi: 10.1182/blood-2010-08-292144 . ISSN   0006-4971. PMID   20855864.
  16. Proposed minimal diagnostic criteria for myelodysplastic syndromes (MDS) and potential pre-MDS conditions, retrieved 15. April 2020 in Ncbi.nlm.nih.gov
  17. Valent, Peter; Orazi, Attilio; Steensma, David P.; Ebert, Benjamin L.; Haase, Detlef; Malcovati, Luca; van de Loosdrecht, Arjan A.; Haferlach, Torsten; Westers, Theresia M.; Wells, Denise A.; Giagounidis, Aristoteles (26 September 2017). "Proposed minimal diagnostic criteria for myelodysplastic syndromes (MDS) and potential pre-MDS conditions". Oncotarget. 8 (43): 73483–73500. doi:10.18632/oncotarget.19008. ISSN   1949-2553. PMC   5650276 . PMID   29088721.
  18. Bockwinkel, Stefanie. "Professor Peter Valent, MD". International CML Foundation. Retrieved 8 April 2020.
  19. "Univ.-Prof. Dr. Peter Valent". Medizinische Universität Wien. Retrieved 8 April 2020.