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CANCER ETIOLOGY, DIAGNOSIS AND TREATMENTS B L URKITT YMPHOMA D , R F IAGNOSIS ISK ACTORS T AND REATMENT No part of this digital document may be reproduced, stored in a retrieval system or transmitted in any form or by any means. The publisher has taken reasonable care in the preparation of this digital document, but makes no expressed or implied warranty of any kind and assumes no responsibility for any errors or omissions. No liability is assumed for incidental or consequential damages in connection with or arising out of information contained herein. This digital document is sold with the clear understanding that the publisher is not engaged in rendering legal, medical or any other professional services. C E , D ANCER TIOLOGY IAGNOSIS T AND REATMENTS Additional books and e-books in this series can be found on Nova’s website under the Series tab. CANCER ETIOLOGY, DIAGNOSIS AND TREATMENTS B L URKITT YMPHOMA D , R F IAGNOSIS ISK ACTORS T AND REATMENT DOUGLAS V. BERTHELOT EDITOR Copyright © 2021 by Nova Science Publishers, Inc. DOI: https://doi.org/10.52305/VODC3429 All rights reserved. No part of this book may be reproduced, stored in a retrieval system or transmitted in any form or by any means: electronic, electrostatic, magnetic, tape, mechanical photocopying, recording or otherwise without the written permission of the Publisher. We have partnered with Copyright Clearance Center to make it easy for you to obtain permissions to reuse content from this publication. Simply navigate to this publication’s page on Nova’s website and locate the “Get Permission” button below the title description. This button is linked directly to the title’s permission page on copyright.com. Alternatively, you can visit copyright.com and search by title, ISBN, or ISSN. For further questions about using the service on copyright.com, please contact: Copyright Clearance Center Phone: +1-(978) 750-8400 Fax: +1-(978) 750-4470 E-mail: [email protected]. 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In addition, no responsibility is assumed by the Publisher for any injury and/or damage to persons or property arising from any methods, products, instructions, ideas or otherwise contained in this publication. This publication is designed to provide accurate and authoritative information with regard to the subject matter covered herein. It is sold with the clear understanding that the Publisher is not engaged in rendering legal or any other professional services. If legal or any other expert assistance is required, the services of a competent person should be sought. FROM A DECLARATION OF PARTICIPANTS JOINTLY ADOPTED BY A COMMITTEE OF THE AMERICAN BAR ASSOCIATION AND A COMMITTEE OF PUBLISHERS. Additional color graphics may be available in the e-book version of this book. Library of Congress Cataloging-in-Publication Data ISBN: (cid:28)(cid:26)(cid:27)(cid:16)(cid:20)(cid:16)(cid:25)(cid:27)(cid:24)(cid:19)(cid:26)(cid:16)(cid:19)(cid:27)(cid:22)(cid:16)(cid:19)(cid:11)(cid:72)(cid:37)(cid:82)(cid:82)(cid:78)(cid:12) Published by Nova Science Publishers, Inc. † New York CONTENTS Preface vii Chapter 1 Burkitt Lymphoma: Diagnosis and Treatment Issues 1 Daniella E.C.C.O.H Kingsley-Godwin, Maria Jana Kingsley Godwin and Joshua Kingsley-Godwin Chapter 2 Burkitt Lymphoma in Patients with Primary Immunodeficiency Disorders 89 Zeinab Afify and Oussama Abla Chapter 3 Post Solid Organ Transplant Burkitt Lymphoma 143 Zeinab Afify and Angela Punnett Chapter 4 Burkitt Lymphoma 179 Luis M. Juárez-Salcedo, Diego Conde-Royo and Samir Dalia Chapter 5 Examining the Therapeutic Advances for Burkitt Lymphoma 203 Daniella E.C.C.O.H Kingsley-Godwin, Maria Jana Kingsley Godwin and Joshua Kingsley-Godwin vi Contents Chapter 6 Burkitt Lymphoma: Evaluation of Characteristics, Clinical Updates and Management Approaches Trends 229 Daniella E.C.C.O.H Kingsley-Godwin, Maria Jana Kingsley Godwin and Joshua Kingsley-Godwin Index 253 PREFACE Burkitt lymphoma (BL) is a form of non-Hodgkin’s lymphoma in which cancer starts in immune cells called B-cells. If left untreated, it is rapidly fatal. Chapter one of this monograph evaluates the etiology, pathological issues, diagnosis, clinical manifestations, epidemiology, research, innovation and treatment issues of BL using the clinical systematic review research method and experts’ opinion analysis approach. Chapter two reviews the spectrum, unique characteristics and special management considerations when BL develops in specific subgroups of patients with primary immune deficiency disorders. Chapter three reviews the incidence, clinicopathological and epidemiologic features, treatment and outcome data available in pediatric and adult patients with post solid organ transplant BL. Chapter four focuses on the pathobiology and the treatment of first-line and relapsed/refractory cases of BL and describes new therapeutic strategies which could improve results in this pathology. Chapter five examines the therapeutic advances in BL and its variants. Finally, Chapter six explores and emphasizes the trends and issues in research and innovation in the characteristics, clinical updates and management approaches of BL. Chapter 1 - Burkitt lymphoma is a rarely encountered aggressive non- Hodgkin B-cell lymphoma. The disease is associated with Epstein Barr virus, human immunodeficiency virus, and chromosomal translocations viii Douglas V. Berthelot that cause the overexpression of oncogene C-MYC. Although the critical role of MYC in Burkitt's lymphoma has been well described, recent biological insights have identified several new mutations that cooperate with MYC in driving lymphomagenesis, paving the way for novel drug testing in this disease. The World Health Organization classifies Burkitt lymphoma into three clinical groups: endemic, sporadic and immunodeficiency-related. The endemic form is linked to malaria and Epstein Barr virus. The immunodeficiency-related variant is associated with human immunodeficiency virus and to a lesser extent, organ transplantation. With intense chemotherapy treatment disease prognosis is excellent in children but poor in adults. In addition, recently, intermediate- intensity approaches have been tested in Burkitt lymphoma. Early multicenter results demonstrate good tolerability while maintaining high cure rates in all patient and age groups. There is a need for more awareness of the trends and issues surrounding the diagnosis and treatment of Burkitt lymphoma. Hence the purpose of this chapter is to evaluate the etiology, pathological issues, diagnosis, clinical manifestations, epidemiology, research and innovation and treatment issues of Burkitt lymphoma using clinical systematic review research method, and experts’ opinion analysis approach. Chapter 2 - Mature B cell Non-Hodgkin lymphoma (B-NHL) including Burkitt lymphoma (BL) and diffuse large B cell lymphoma (DLBCL) are among the most common malignancies associated with primary immune deficiency disorders (PIDs). Unlike sporadic BL, PIDs associated B-NHL are commonly Epstein Barr virus (EBV) driven. There is a wide spectrum of PIDs associated with BL resulting in unique features with each association. For example, patients with X-linked lymphoproliferative disease type 1 (XLP1) are at particularly high risk of developing multiple lymphomas. Other PIDs are also chromosome instability syndromes, such as ataxia telangiectasia (AT) and Bloom Syndrome (BS). In these syndromes, chemotherapy, if not modified, confers a high risk of severe morbidity and mortality. Patients with PIDs are routinely excluded from clinical trials of B-NHL, therefore, limited data are available regarding potential differences in lymphoma biology, risk factors, optimal treatment