Burkitt Lymphoma: Diagnosis, Risk Factors and Treatment

274 Pages·2021·7.813 MB·English

by  Douglas V. Berthelot

CANCER ETIOLOGY, DIAGNOSIS AND TREATMENTS

B L
URKITT YMPHOMA

D , R F
IAGNOSIS ISK ACTORS
T
AND REATMENT

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C E , D
ANCER TIOLOGY IAGNOSIS
T
AND REATMENTS

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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

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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