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Chapter 13 - Myeloid and Lymphoid Neoplasms Associated with Eosinophilia
- Jon van der Walt, Attilio Orazi, Daniel A. Arber, University of Chicago
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- Book:
- Diagnostic Bone Marrow Haematopathology
- Published online:
- 12 November 2020
- Print publication:
- 21 January 2021, pp 200-230
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Summary
Myeloid and lymphoid neoplasms with eosinophilia (MLNE) and rearrangements of PDGFRA, PDGFRB and FGFR1 were recognized as a standalone category in the 2008 WHO classification. PCM1-JAK2 was added to this family as a new provisional entity in the 2016 WHO classification [1, 2]. The features shared by neoplasms in this category include a common presentation with eosinophilia or hypereosinophilia in peripheral blood and an increased number of eosinophilic forms in bone marrow (BM). Some cases present as acute leukaemia. Some cases may lack hypereosinophilia. The underlying mechanism is the overexpression of an aberrant tyrosine kinase as a result of a fusion gene, or rarely of a mutation, and a diagnosis and classification requires the demonstration of the specific gene fusions. The cell of origin is a mutated pluripotent stem cell that has the potential to involve myeloid, lymphoid or both lineages, concomitantly or sequentially, leading to clinically complex and heterogeneous manifestations. A common scenario is the presentation as a chronic myeloproliferative neoplasm (MPN), usually with eosinophilia followed within a variable time period and depending on the gene fusion involved, by a progression to acute myeloid leukaemia (AML) or mixed phenotype acute leukaemia (usually in the BM), and B- or T-lymphoblastic leukaemia/lymphoma (B-/T-ALL) in BM or in an extramedullary site. Thus it is critical to recognize the clinicopathologic features of these neoplasms, identify the molecular genetic lesions and classify them accordingly. An accurate diagnosis and classification have important therapeutic and prognostic implications.
Contributors
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- By Aakash Agarwala, Linda S. Aglio, Rae M. Allain, Paul D. Allen, Houman Amirfarzan, Yasodananda Kumar Areti, Amit Asopa, Edwin G. Avery, Patricia R. Bachiller, Angela M. Bader, Rana Badr, Sibinka Bajic, David J. Baker, Sheila R. Barnett, Rena Beckerly, Lorenzo Berra, Walter Bethune, Sascha S. Beutler, Tarun Bhalla, Edward A. Bittner, Jonathan D. Bloom, Alina V. Bodas, Lina M. Bolanos-Diaz, Ruma R. Bose, Jan Boublik, John P. Broadnax, Jason C. Brookman, Meredith R. Brooks, Roland Brusseau, Ethan O. Bryson, Linda A. Bulich, Kenji Butterfield, William R. Camann, Denise M. Chan, Theresa S. Chang, Jonathan E. Charnin, Mark Chrostowski, Fred Cobey, Adam B. Collins, Mercedes A. Concepcion, Christopher W. Connor, Bronwyn Cooper, Jeffrey B. Cooper, Martha Cordoba-Amorocho, Stephen B. Corn, Darin J. Correll, Gregory J. Crosby, Lisa J. Crossley, Deborah J. Culley, Tomas Cvrk, Michael N. D'Ambra, Michael Decker, Daniel F. Dedrick, Mark Dershwitz, Francis X. Dillon, Pradeep Dinakar, Alimorad G. Djalali, D. John Doyle, Lambertus Drop, Ian F. Dunn, Theodore E. Dushane, Sunil Eappen, Thomas Edrich, Jesse M. Ehrenfeld, Jason M. Erlich, Lucinda L. Everett, Elliott S. Farber, Khaldoun Faris, Eddy M. Feliz, Massimo Ferrigno, Richard S. Field, Michael G. Fitzsimons, Hugh L. Flanagan Jr., Vladimir Formanek, Amanda A. Fox, John A. Fox, Gyorgy Frendl, Tanja S. Frey, Samuel M. Galvagno Jr., Edward R. Garcia, Jonathan D. Gates, Cosmin Gauran, Brian J. Gelfand, Simon Gelman, Alexander C. Gerhart, Peter Gerner, Omid Ghalambor, Christopher J. Gilligan, Christian D. Gonzalez, Noah E. Gordon, William B. Gormley, Thomas J. Graetz, Wendy L. Gross, Amit Gupta, James P. Hardy, Seetharaman Hariharan, Miriam Harnett, Philip M. Hartigan, Joaquim M. Havens, Bishr Haydar, Stephen O. Heard, James L. Helstrom, David L. Hepner, McCallum R. Hoyt, Robert N. Jamison, Karinne Jervis, Stephanie B. Jones, Swaminathan Karthik, Richard M. Kaufman, Shubjeet Kaur, Lee A. Kearse Jr., John C. Keel, Scott D. Kelley, Albert H. Kim, Amy L. Kim, Grace Y. Kim, Robert J. Klickovich, Robert M. Knapp, Bhavani S. Kodali, Rahul Koka, Alina Lazar, Laura H. Leduc, Stanley Leeson, Lisa R. Leffert, Scott A. LeGrand, Patricio Leyton, J. Lance Lichtor, John Lin, Alvaro A. Macias, Karan Madan, Sohail K. Mahboobi, Devi Mahendran, Christine Mai, Sayeed Malek, S. Rao Mallampati, Thomas J. Mancuso, Ramon Martin, Matthew C. Martinez, J. A. Jeevendra Martyn, Kai Matthes, Tommaso Mauri, Mary Ellen McCann, Shannon S. McKenna, Dennis J. McNicholl, Abdel-Kader Mehio, Thor C. Milland, Tonya L. K. Miller, John D. Mitchell, K. Annette Mizuguchi, Naila Moghul, David R. Moss, Ross J. Musumeci, Naveen Nathan, Ju-Mei Ng, Liem C. Nguyen, Ervant Nishanian, Martina Nowak, Ala Nozari, Michael Nurok, Arti Ori, Rafael A. Ortega, Amy J. Ortman, David Oxman, Arvind Palanisamy, Carlo Pancaro, Lisbeth Lopez Pappas, Benjamin Parish, Samuel Park, Deborah S. Pederson, Beverly K. Philip, James H. Philip, Silvia Pivi, Stephen D. Pratt, Douglas E. Raines, Stephen L. Ratcliff, James P. Rathmell, J. Taylor Reed, Elizabeth M. Rickerson, Selwyn O. Rogers Jr., Thomas M. Romanelli, William H. Rosenblatt, Carl E. Rosow, Edgar L. Ross, J. Victor Ryckman, Mônica M. Sá Rêgo, Nicholas Sadovnikoff, Warren S. Sandberg, Annette Y. Schure, B. Scott Segal, Navil F. Sethna, Swapneel K. Shah, Shaheen F. Shaikh, Fred E. Shapiro, Torin D. Shear, Prem S. Shekar, Stanton K. Shernan, Naomi Shimizu, Douglas C. Shook, Kamal K. Sikka, Pankaj K. Sikka, David A. Silver, Jeffrey H. Silverstein, Emily A. Singer, Ken Solt, Spiro G. Spanakis, Wolfgang Steudel, Matthias Stopfkuchen-Evans, Michael P. Storey, Gary R. Strichartz, Balachundhar Subramaniam, Wariya Sukhupragarn, John Summers, Shine Sun, Eswar Sundar, Sugantha Sundar, Neelakantan Sunder, Faraz Syed, Usha B. Tedrow, Nelson L. Thaemert, George P. Topulos, Lawrence C. Tsen, Richard D. Urman, Charles A. Vacanti, Francis X. Vacanti, Joshua C. Vacanti, Assia Valovska, Ivan T. Valovski, Mary Ann Vann, Susan Vassallo, Anasuya Vasudevan, Kamen V. Vlassakov, Gian Paolo Volpato, Essi M. Vulli, J. Matthias Walz, Jingping Wang, James F. Watkins, Maxwell Weinmann, Sharon L. Wetherall, Mallory Williams, Sarah H. Wiser, Zhiling Xiong, Warren M. Zapol, Jie Zhou
- Edited by Charles Vacanti, Scott Segal, Pankaj Sikka, Richard Urman
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- Book:
- Essential Clinical Anesthesia
- Published online:
- 05 January 2012
- Print publication:
- 11 July 2011, pp xv-xxviii
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6 - Inherited and acquired bone marrow failure syndromes associated with multiple cytopenias
- from Section 1 - General and non-neoplastic hematopathology
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- By Sa A. Wang, University of Texas M.D. Anderson Cancer Center, Victor Zota, Novartis Institutes for BioMedical Research, Inc.
- Edited by Maria A. Proytcheva, Northwestern University Medical School, Illinois
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- Book:
- Diagnostic Pediatric Hematopathology
- Published online:
- 03 May 2011
- Print publication:
- 03 February 2011, pp 90-103
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Summary
Overview
Bone marrow failure syndrome consists of a group of rare diseases with the defining features of ineffective/defective hematopoiesis by the bone marrow and resultant peripheral cytopenia [1, 2]. Bone marrow failure can be attributed to a variety of mechanisms, such as loss of pluripotent hematopoietic stem cells; bone marrow replacement by metastatic carcinoma, lymphoma, leukemia or fibrosis; nutritional/metabolic disorder leading to maturation arrest or clonal hematopoietic stem neoplasm such as myelodysplastic syndromes. Aplastic anemia is the paradigm of the human bone marrow failure syndrome, characterized by peripheral cytopenia and a hypocellular marrow [1, 3, 4]. Injury to or loss of pluripotent hematopoietic stem cells, in the absence of an infiltrative disease of the bone marrow, is the major pathophysiologic characteristic of the disease [3, 4]. Aplastic anemia can result from either inherited or acquired causes. This chapter will discuss the bone marrow failure syndromes, both inherited and acquired, that are associated with multiple cytopenias.
Inherited bone marrow failure syndromes
The inherited bone marrow failure syndromes in children comprise a group of rare congenital disorders in which the bone marrow is unable to produce blood cells effectively resulting in unilineage or multilineage cytopenia (Table 6.1). These syndromes may present with or without physical anomalies [5]. Patients with inherited bone marrow failure syndromes are at risk for severe cytopenia, development of marrow cytogenetic abnormalities, myelodysplastic syndromes (MDS), and other malignancies [6].
14 - Myelodysplastic syndromes and therapy-related myeloid neoplasms
- from Section 2 - Neoplastic hematopathology
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- By Sa A. Wang, University of Texas M.D. Anderson Cancer Center
- Edited by Maria A. Proytcheva, Northwestern University Medical School, Illinois
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- Book:
- Diagnostic Pediatric Hematopathology
- Published online:
- 03 May 2011
- Print publication:
- 03 February 2011, pp 253-271
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Summary
Myelodysplastic syndromes
Definition
Myelodysplastic syndromes (MDS) in childhood encompasses a diverse group of clonal hematopoietic stem cell disorders, characterized by ineffective hematopoiesis with morphologic dysplasia, peripheral cytopenia, and an increased propensity to evolve into acute leukemia. MDS can arise either de novo in a previously healthy child (primary MDS), or develop in a child with a known predisposition as secondary MDS. In adult patients, most of the secondary MDSs are therapy-related, following cytotoxic therapy for prior neoplastic or non-neoplastic conditions. Secondary MDS in childhood includes many cases associated with constitutional bone marrow failure disorders, MDS evolved from acquired aplastic anemia, and familial MDS (Table 14.1), in addition to therapy-related MDS. It is noteworthy that the distinction of primary MDS and secondary MDS may not be clear cut in pediatric patients, since some of “primary MDS” may have an underlying, yet unknown, genetic defect predisposing them to MDS at a young age.
Epidemiology
Pediatric MDS is a rare hematologic malignancy of childhood. The reported incidence of pediatric MDS comprises 1.1 to 8.7% of hematologic malignancies of childhood, with an annual incidence of 1.8 per million in children of 0–14 years of age [1]. It constitutes 4% of cases, and is the third most common hematologic malignancy in children, following acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). In contrast to adult MDS that shows a male predominance (1.7 : 1), MDS in children affects males and females with an equal frequency.
Electron Microscopy Study of Novel Ru Doped La0.8Sr0.2CrO3 as Anode Materials for Solid Oxide Fuel Cells (SOFCs)
- Y Wang, BD Madsen, W Kobsiriphat, SA Barnett, LD Marks
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- Journal:
- Microscopy and Microanalysis / Volume 13 / Issue S02 / August 2007
- Published online by Cambridge University Press:
- 05 August 2007, pp. 100-101
- Print publication:
- August 2007
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Extended abstract of a paper presented at Microscopy and Microanalysis 2007 in Ft. Lauderdale, Florida, USA, August 5 – August 9, 2007