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Original Article

Clinical and Biologic Diversity of Leukemias Occurring in Patients with Mastocytosis

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Pages 473-486 | Received 10 Oct 1999, Published online: 01 Jul 2009

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Read on this site (5)

Wolfgang R Sperr & Peter Valent. (2012) Diagnosis, progression patterns and prognostication in mastocytosis. Expert Review of Hematology 5:3, pages 261-274.
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Catherine Fong & Michael Tong. (2010) Systemic mastocytosis associated with acute myeloid leukaemia. Pathology 42:5, pages 485-487.
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Karoline Sonneck, Stefan Florian, Alexandra Böhm, Maria-Theresa Krauth, Rudin Kondo, Alexander W. Hauswirth, Karoline V. Gleixner, Karl J. Aichberger, Sophia Derdak, Winfried F. Pickl, Wolfgang R. Sperr, Lawrence B. Schwartz & Peter Valent. (2006) Evaluation of biologic activity of tryptase secreted from blast cells in acute myeloid leukemia. Leukemia & Lymphoma 47:5, pages 897-906.
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Peter Valent, Cem Akin, Wolfgang Sperr, Matthias Mayerhofer, Manuela Födinger, Robert Fritsche-Polanz, Karl Sotlar, Luis Escribano, Michel Arock, Hans-Peter Horny & Dean Metcalfe. (2005) Mastocytosis: Pathology, genetics, and current options for therapy. Leukemia & Lymphoma 46:1, pages 35-48.
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Wolfgang R. Sperr, Alexander W. Hauswirth & Peter Valent. (2002) Tryptase a Novel Biochemical Marker of Acute Myeloid Leukemia. Leukemia & Lymphoma 43:12, pages 2257-2261.
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Magda Zanelli, Martina Quintini, Salvatore Magnasco, Lara Aprile, Andrea Palicelli, Maurizio Zizzo, Francesca Sanguedolce, Stefano Ricci, Saverio Pancetti, Valeria Zuccalà, Veronica Martino, Giuseppe Broggi, Rosario Caltabiano, Alberto Cavazza, Paola Parente, Cristina Mecucci, Giovanni Martino & Stefano Ascani. (2023) Mast Cell Leukemia: An Update with a Practical Review. Cancers 15:6, pages 1664.
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Peter Valent, Cem Akin, Wolfgang R. Sperr, Hans-Peter Horny, Michel Arock, Dean D. Metcalfe & Stephen J. Galli. (2023) New Insights into the Pathogenesis of Mastocytosis: Emerging Concepts in Diagnosis and Therapy. Annual Review of Pathology: Mechanisms of Disease 18:1, pages 361-386.
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Peter Valent, Karin Hartmann, Juliana Schwaab, Ivan Alvarez-Twose, Knut Brockow, Patrizia Bonadonna, Olivier Hermine, Marek Niedoszytko, Melody C. Carter, Gregor Hoermann, Wolfgang R. Sperr, Joseph H. Butterfield, Celalettin Ustun, Roberta Zanotti, Deepti H. Radia, Mariana Castells, Massimo Triggiani, Lawrence B. Schwartz, Alberto Orfao, Tracy I. George, Karl Sotlar, Jason Gotlib, Andreas Reiter, Hans-Peter Horny, Michel Arock, Cem Akin & Dean D. Metcalfe. (2022) Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User’s Guide for Daily Clinical Practice. The Journal of Allergy and Clinical Immunology: In Practice 10:8, pages 1999-2012.e6.
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M. B. Günay, N. Büyükbabani, A. S. Yavuz, İ. Yılmaz & C. Baykal. (2021) Association of systemic mastocytosis with primary cutaneous marginal zone lymphoma; first case. Journal of the European Academy of Dermatology and Venereology 36:4.
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Javier I Muñoz-González, Iván Álvarez-Twose, María Jara-Acevedo, Roberta Zanotti, Cecelia Perkins, Mohamad Jawhar, Wolfgang R Sperr, Khalid Shoumariyeh, Juliana Schwaab, Georg Greiner, Ana Henriques, Carolina Caldas, Carlos Fernández-Giménez, Laura Sánchez-Muñoz, Andrea Mayado, Alba Pérez-Pons, Annette Schmitt-Graeff, Justus Duyster, Ilaria Tanasi, Francesco Olivieri, Elvira Mora-Casterá, Irene Luna, Leonor Senent, Maria-Helena Bañas, Amanda Nuñez-García, Manuel Jurado-Chacón, Guillermo Martín-Sánchez, Enrique Colado, Blanca Xicoy, Georgina Gener-Ricós, Jason Gotlib, Patrizia Bonadonna, Andreas Reiter, Peter Valent, Andrés C García-Montero & Alberto Orfao. (2021) Proposed global prognostic score for systemic mastocytosis: a retrospective prognostic modelling study. The Lancet Haematology 8:3, pages e194-e204.
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Aleksandra S Orlova, Karina M Shkurlatovskaia, Ekaterina V Silina, Polina M Pyatilova, Natalya P Teplyuk, Evgeniya A Kogan, Sergej I Vorobyev, Stefani S Bolevich, Valida A Dadaeva, Bella I Tachieva & Sergej B Bolevich. (2020) Laboratory Tests in Diagnosis of Mastocytosis: Literature Review and Case Report. Serbian Journal of Experimental and Clinical Research 21:2, pages 171-178.
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Peter Valent. (2020) Oligo-monocytic CMML and other pre-CMML states: Clinical impact, prognostication and management. Best Practice & Research Clinical Haematology 33:2, pages 101137.
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Alice Boilève, Adrien Contejean, Sylvain Barreau, Élise Sourdeau, Chloé Friedrich, Olivier Kosmider, Barbara Burroni, Nicolas Dupin, Coralie Lheure, Julien Rossignol, Didier Bouscary & Éric Grignano. (2020) Mastocytosis onset in a patient with treated hairy cell leukemia: Just a coincidence?. Blood Cells, Molecules, and Diseases 81, pages 102392.
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Roos Leguit, Konnie Hebeda, Marcus Kremer, Jon van der Walt, Umberto Gianelli, Alexandar Tzankov & Attilio Orazi. (2020) The Spectrum of Aggressive Mastocytosis: A Workshop Report and Literature Review. Pathobiology 87:1, pages 2-19.
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Barbara Frossi, Francesca Mion, Claudio Tripodo, Mario P. Colombo & Carlo E. Pucillo. (2017) Rheostatic Functions of Mast Cells in the Control of Innate and Adaptive Immune Responses. Trends in Immunology 38:9, pages 648-656.
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Barbara J. Bain. 2017. Leukaemia Diagnosis. Leukaemia Diagnosis 69 134 .
Dean D. MetcalfeYoseph A. Mekori. (2017) Pathogenesis and Pathology of Mastocytosis. Annual Review of Pathology: Mechanisms of Disease 12:1, pages 487-514.
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Feryal Abbas Ibrahim Hilmi, Ahmad Al-Sabbagh, Dina Sameh Soliman, Hesham Al SabahOmar Mohammad IsmailMohamed YassinHalima El-Omri. (2017) Acute Myeloid Leukemia With Inv(16)(p13q22) Associated With Hidden Systemic Mastocytosis: Case Report and Review of Literature. Clinical Medicine Insights: Blood Disorders 10, pages 1179545X1770085.
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Douglas Tremblay, Nicole Carreau, Marina Kremyanskaya & John Mascarenhas. (2015) Systemic Mastocytosis: Clinical Update and Future Directions. Clinical Lymphoma Myeloma and Leukemia 15:12, pages 728-738.
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Phillip Lieberman, Richard A. Nicklas, Christopher Randolph, John Oppenheimer, David Bernstein, Jonathan Bernstein, Anne Ellis, David B.K. Golden, Paul Greenberger, Steven Kemp, David Khan, Dennis Ledford, Jay Lieberman, Dean Metcalfe, Anna Nowak-Wegrzyn, Scott Sicherer, Dana Wallace, Joann Blessing-Moore, David Lang, Jay M. Portnoy, Diane Schuller, Sheldon Spector & Stephen A. Tilles. (2015) Anaphylaxis—a practice parameter update 2015. Annals of Allergy, Asthma & Immunology 115:5, pages 341-384.
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Peter Valent, Karl Sotlar, Wolfgang R. Sperr, Andreas Reiter, Michel Arock & Hans-Peter Horny. (2015) Chronic mast cell leukemia: A novel leukemia-variant with distinct morphological and clinical features. Leukemia Research 39:1, pages 1-5.
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Amy Rich, Jinlan Sun, Abdulrahman S. Aldayel, C. Cameron Yin, L. Jeffrey Medeiros & Sergej Konoplev. (2014) Myelomastocytic Leukemia With Aberrant CD25 Expression: Case Report and Review of the Literature. Clinical Lymphoma Myeloma and Leukemia 14:5, pages e173-e177.
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Theodoros Iliakis, Niki Rougkala, Panagiotis T. Diamantopoulos, Vasiliki Papadopoulou, Fani Kalala, Konstantinos Zervakis, Nefeli Giannakopoulou, Polixeni Chatzinikolaou, Georgia Levidou, Eleftheria Lakiotaki, Penelope Korkolopoulou, Efstratios Patsouris, Eleni Variami & Nora-Athina Viniou. (2014) An Adult Patient with Systemic Mastocytosis and B-Acute Lymphoblastic Leukemia. Case Reports in Medicine 2014, pages 1-5.
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K.M. Meyer, M. Landthaler, C. Hafner & E. Geissinger. (2013) Systemic mastocytosis associated with cutaneous B-cell lymphoma. British Journal of Dermatology 169:5, pages 1165-1167.
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Sheeja T. Pullarkat, Vinod Pullarkat, Anand Lagoo, Russell Brynes, Lawrence M. Weiss, Victoria Bedell, Wengang Chen, Qin Huang, Karl Gaal, Dennis D. Weisenburger & Young S. Kim. (2013) Characterization of bone marrow mast cells in acute myeloid leukemia with t(8;21) (q22;q22); RUNX1-RUNX1T1. Leukemia Research 37:11, pages 1572-1575.
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Stephen J. Fuller. (2012) New Insights into the Pathogenesis, Diagnosis, and Management of Mastocytosis. Hematology/Oncology Clinics of North America 26:6, pages 1143-1168.
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T. Gülen, B. Sander, G. Nilsson, J. Palmblad, K. Sotlar, H.-P. Horny & H. Hägglund. (2012) Systemic mastocytosis: progressive evolution of an occult disease into fatal mast cell leukemia: unique findings on an unusual hematological neoplasm. Medical Oncology 29:5, pages 3540-3546.
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Celalettin Ustun, Natasha M. Savage, Jason Gotlib, Kapil Bhalla, Elizabeth Manaloor & Tracy I. George. (2011) Systemic mastocytosis with associated clonal hematological non‐mast‐cell lineage disease: A case review. American Journal of Hematology 87:2, pages 191-193.
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Hans-Peter Horny, Karl Sotlar & Peter Valent. 2012. Bone Marrow Lymphoid Infiltrates. Bone Marrow Lymphoid Infiltrates 271 279 .
Tracy I. George & Hans-Peter Horny. (2011) Systemic Mastocytosis. Hematology/Oncology Clinics of North America 25:5, pages 1067-1083.
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R. Fernández-Torres, M. M. Verea, A. Álvarez, P. Torres & E. Fonseca. (2011) Systemic Mastocytosis Associated with Splenic Marginal Zone Lymphoma with Villous Lymphocytes. Dermatology Research and Practice 2011, pages 1-4.
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Tracy I. George & Hans-Peter Horny. (2010) Systemic Mastocytosis. Surgical Pathology Clinics 3:4, pages 1185-1202.
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Robert Fritsche-Polanz, Marika Fritz, Andrea Huber, Karl Sotlar, Wolfgang R. Sperr, Christine Mannhalter, Manuela Födinger & Peter Valent. (2010) High frequency of concomitant mastocytosis in patients with acute myeloid leukemia exhibiting the transforming KIT mutation D816V . Molecular Oncology 4:4, pages 335-346.
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Angela R. Arredondo, Jason Gotlib, Luke Shier, Bruno Medeiros, Kathleen Wong, Athena Cherry, Christopher Corless, Daniel A. Arber, Peter Valent & Tracy I. George. (2010) Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: A diagnostic challenge. American Journal of Hematology 85:8, pages 600-606.
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H. David Pettigrew, Suzanne S. Teuber, James S. Kong & M. Eric Gershwin. (2009) Contemporary Challenges in Mastocytosis. Clinical Reviews in Allergy & Immunology 38:2-3, pages 125-134.
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Karl Sotlar, Sema Colak, Anja Bache, Sabina Berezowska, Manuela Krokowski, Burkhard Bültmann, Peter Valent & Hans‐Peter Horny. (2010) Variable presence of KIT D816V in clonal haematological non‐mast cell lineage diseases associated with systemic mastocytosis (SM–AHNMD) . The Journal of Pathology 220:5, pages 586-595.
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Barbara J. Bain. 2010. Leukaemia Diagnosis. Leukaemia Diagnosis 64 113 .
Hans-Peter Horny. (2009) Mastocytosis. American Journal of Clinical Pathology 132:3, pages 438-447.
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Luis Escribano, Iván Álvarez-Twose, Laura Sánchez-Muñoz, Andres Garcia-Montero, Rosa Núñez, Julia Almeida, Maria Jara-Acevedo, Cristina Teodósio, Mónica García-Cosío, Carmen Bellas & Alberto Orfao. (2009) Prognosis in adult indolent systemic mastocytosis: A long-term study of the Spanish Network on Mastocytosis in a series of 145 patients. Journal of Allergy and Clinical Immunology 124:3, pages 514-521.
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Sheeja T. Pullarkat, Vinod Pullarkat, Steven H. Kroft, Carla S. Wilson, Arshad N. Ahsanuddin, Karen P. Mann, Maung Thein, Wayne W. Grody & Russell K. Brynes. (2009) Systemic mastocytosis associated with t(8;21)(q22;q22) acute myeloid leukemia. Journal of Hematopathology 2:1.
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Alexander W. Hauswirth, Manuela Födinger, Marika Fritz, Leonhard Müllauer, Ingrid Simonitsch-Klupp, Berthold Streubel, Andreas Chott, Wolfgang R. Sperr, Ulrich Jäger & Peter Valent. (2008) Indolent systemic mastocytosis associated with atypical small lymphocytic lymphoma: a rare form of concomitant lymphoproliferative disease. Human Pathology 39:6, pages 917-924.
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Animesh Pardanani & Srdan Verstovsek. (2007) Hypereosinophilic Syndrome, Chronic Eosinophilic Leukemia, and Mast Cell Disease. The Cancer Journal 13:6, pages 384-391.
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Caron Steinmetz, Ahmed Shabaik & Farnaz Hasteh. (2007) Systemic mastocytosis associated with small lymphocytic lymphoma: An incidental finding in a patient with invasive gastric adenocarcinoma. Diagnostic Cytopathology 35:11, pages 728-733.
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Alexandra Böhm, Manuela Födinger, Friedrich Wimazal, Oskar A. Haas, Matthias Mayerhofer, Wolfgang R. Sperr, Harald Esterbauer & Peter Valent. (2007) Eosinophilia in systemic mastocytosis: Clinical and molecular correlates and prognostic significance. Journal of Allergy and Clinical Immunology 120:1, pages 192-199.
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P. Valent, C. Akin, L. Escribano, M. Födinger, K. Hartmann, K. Brockow, M. Castells, W. R. Sperr, H. C. Kluin-Nelemans, N. A. T. Hamdy, O. Lortholary, J. Robyn, J. van Doormaal, K. Sotlar, A. W. Hauswirth, M. Arock, O. Hermine, A. Hellmann, M. Triggiani, M. Niedoszytko, L. B. Schwartz, A. Orfao, H.-P. Horny & D. D. Metcalfe. (2007) Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria. European Journal of Clinical Investigation 37:6, pages 435-453.
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Alfonso Quintas‐Cardama, Ahmed Aribi, Jorge Cortes, Francis J. Giles, Hagop Kantarjian & Srdan Verstovsek. (2006) Novel approaches in the treatment of systemic mastocytosis. Cancer 107:7, pages 1429-1439.
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A. Pardanani, C. Akin & P. Valent. (2006) Pathogenesis, clinical features, and treatment advances in mastocytosis. Best Practice & Research Clinical Haematology 19:3, pages 595-615.
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Joseph H. Butterfield. (2006) Systemic Mastocytosis: Clinical Manifestations and Differential Diagnosis. Immunology and Allergy Clinics of North America 26:3, pages 487-513.
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Peter Valent. (2006) Diagnostic Evaluation and Classification of Mastocytosis. Immunology and Allergy Clinics of North America 26:3, pages 515-534.
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P Samorapoompichit, G-H Schernthaner, C Worda, F Wimazal, M-T Krauth, W R Sperr & P Valent. (2006) Evaluation of neoplastic human mast cells by tryptase-immunoelectron microscopy. Histopathology 48:3, pages 247-257.
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Jamie Robyn & Dean D. Metcalfe. 2006. 169 243 .
Wolfgang R. Sperr, Johannes Drach, Alexander W. Hauswirth, Jutta Ackermann, Margit Mitterbauer, Gerlinde Mitterbauer, Manuela Foedinger, Christa Fonatsch, Ingrid Simonitsch-Klupp, Peter Kalhs & Peter Valent. (2005) Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation. Clinical Cancer Research 11:19, pages 6787-6792.
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Hermine Agis, Karl Sotlar, Peter Valent & Hans-Peter Horny. (2005) Ph-Chromosome-positive chronic myeloid leukemia with associated bone marrow mastocytosis. Leukemia Research 29:10, pages 1227-1232.
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Knut Brockow, Cem Akin, Mary Huber & Dean D. Metcalfe. (2005) IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis. Clinical Immunology 115:2, pages 216-223.
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Peter Valent, Wolfgang R Sperr, Lawrence B Schwartz & Hans-Peter Horny. (2004) Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non–mast cell hematopoietic neoplasms. Journal of Allergy and Clinical Immunology 114:1, pages 3-11.
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Luis Escribano, Andrés Garcı́a-Montero, Rosa Núñez-López, Javier López-Jiménez, Julia Almeida, Aranzazu Prados & Alberto Orfao. (2004) Systemic mastocytosis associated with acute myeloid leukemia: case report and implications for disease pathogenesis. Journal of Allergy and Clinical Immunology 114:1, pages 28-33.
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Alexander W Hauswirth, Ingrid Simonitsch-Klupp, Martin Uffmann, Elisabeth Koller, Wolfgang R Sperr, Klaus Lechner & Peter Valent. (2004) Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: report of five cases and review of the literature. Leukemia Research 28:3, pages 249-257.
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Stephen O'Brien, Ayalew Tefferi & Peter Valent. (2004) Chronic Myelogenous Leukemia and Myeloproliferative Disease. Hematology 2004:1, pages 146-162.
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Peter Valent, Cem Akin, Wolfgang R Sperr, Hans-Peter Horny & Dean D Metcalfe. (2003) Mast cell proliferative disorders: current view on variants recognized by the World Health Organization. Hematology/Oncology Clinics of North America 17:5, pages 1227-1241.
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Peter Valent, Cem Akin, Wolfgang R. Sperr, Hans-P. Horny, Michel Arock, Klaus Lechner, John M. Bennett & Dean D. Metcalfe. (2003) Diagnosis and treatment of systemic mastocytosis: state of the art. British Journal of Haematology 122:5, pages 695-717.
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Hans G Drexler & Roderick A.F MacLeod. (2003) Malignant hematopoietic cell lines: in vitro models for the study of mast cell leukemia. Leukemia Research 27:8, pages 671-676.
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Vinod A. Pullarkat, Carlos Bueso‐Ramos, Raymond Lai, Steven Kroft, Carla S. Wilson, Sheeja T. Pullarkat, Xiangdong Bu, Maung Thein, M. Lee & Russell K. Brynes. (2003) Systemic mastocytosis with associated clonal hematological non‐mast‐cell lineage disease: Analysis of clinicopathologic features and activating c‐kit mutations . American Journal of Hematology 73:1, pages 12-17.
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Francis J. Giles, Maureen A. Cooper, Lewis Silverman, Judith E. Karp, Jeffrey E. Lancet, Maurizio Zangari, Paul J. Shami, Khuda D. Khan, Alison L. Hannah, Julie M. Cherrington, Deborah A. Thomas, Guillermo Garcia‐Manero, Maher Albitar, Hagop M. Kantarjian & Alison T. Stopeck. (2003) Phase II study of SU5416—a small‐molecule, vascular endothelial growth factor tyrosine‐kinase receptor inhibitor—in patients with refractory myeloproliferative diseases. Cancer 97:8, pages 1920-1928.
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Luis Escribano, Jaime Pérez de Oteyza, Rosa Núñez & Alberto Orfao. (2002) Cladribine induces immunophenotypical changes in bone marrow mast cells from mastocytosis. Leukemia Research 26:11, pages 1043-1046.
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Alexander W. Hauswirth, Wolfgang R. Sperr, Minoo Ghannadan, Gerit-Holger Schernthaner, John-Hendrik Jordan, Robert Fritsche-Polanz, Ingrid Simonitsch-Klupp, Manuela Födinger, Klaus Lechner & Peter Valent. (2002) A case of smouldering mastocytosis with peripheral blood eosinophilia and lymphadenopathy. Leukemia Research 26:6, pages 601-606.
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Knut Brockow & Dean D. Metcalfe. (2001) Mastocytosis. Current Opinion in Allergy and Clinical Immunology 1:5, pages 449-454.
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Wolfgang R Sperr, Luis Escribano, John-Hendrik Jordan, Gerit-Holger Schernthaner, Michael Kundi, Hans-Peter Horny & Peter Valent. (2001) Morphologic properties of neoplastic mast cells: delineation of stages of maturation and implication for cytological grading of mastocytosis. Leukemia Research 25:7, pages 529-536.
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Peter Valent, Wolfgang R. Sperr, Puchit Samorapoompichit, Klaus Geissler, Klaus Lechner, Hans-Peter Horny & John M. Bennett. (2001) Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis. Leukemia Research 25:7, pages 595-602.
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Gianni Marone, Giuseppe Spadaro, Francescopaolo Granata & Massimo Triggiani. (2001) Treatment of mastocytosis: pharmacologic basis and current concepts. Leukemia Research 25:7, pages 583-594.
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Peter Valent, Hans-P Horny, Luis Escribano, B.Jack Longley, Chin Y Li, Lawrence B Schwartz, Gianni Marone, Rosa Nuñez, Cem Akin, Karl Sotlar, Wolfgang R Sperr, Klaus Wolff, Richard D Brunning, Reza M Parwaresch, K.Frank Austen, Karl Lennert, Dean D Metcalfe, James W Vardiman & John M Bennett. (2001) Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leukemia Research 25:7, pages 603-625.
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Hans-Peter Horny & Peter Valent. (2001) Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings. Leukemia Research 25:7, pages 543-551.
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John-Hendrik Jordan, Robert Fritsche-Polanz, Wolfgang R. Sperr, Gerlinde Mitterbauer, Manuela Födinger, Gerit-Holger Schernthaner, Hans Christian Bankl, Walter Gebhart, Andreas Chott, Klaus Lechner & Peter Valent. (2001) A case of ‘smouldering’ mastocytosis with high mast cell burden, monoclonal myeloid cells, and C-KIT mutation Asp-816-Val. Leukemia Research 25:7, pages 627-634.
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