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.
Author(s): Douglas V. Berthelot
Series: Cancer Etiology, Diagnosis and Treatments
Publisher: Nova Science Publishers
Year: 2021
Language: English
Pages: 272
City: New York
Contents
Preface
Chapter 1
Burkitt Lymphoma: Diagnosis and Treatment Issues
Abstract
Introduction
Methods
Inclusion Criteria
Data Extraction
Methodological Quality
Data and Statistical Analysis
Results
Classification of Burkitt Lymphoma
The Endemic Type (Which Is Also Known as "African Variant")
The Sporadic Type of BL (Also Known as "Non-African" Variant)
Immunodeficiency-Associated BL
Etiology of BL
Symptoms of BL
Epidemiology of BL
Epidemiologic Features of Endemic, Sporadic and AIDS-Associated BL
Pathophysiology
Genetics
MicroRNA Expression
BL Pathogenesis and EBV Manifestation
The Roles of Holoendemic Malaria and HIV Infection in BL Pathogenesis
Histopathology
Patient History and Physical Examination
Diagnosis of BL
Characteristics/Features of Malignant B Cells
Microscopic Investigations
Immunohistochemistry Analysis
Evaluation and Laboratory Tests for Patients
Staging of BL
St. Jude/Murphy Staging System in Children
Stage I
Stage II
Stage III
Stage IV
The Murphy Staging System in Adults
Stage I
Stage II
Stage HR (Hormone Receptor Stage)
Stage III
Stage III A
Stage III B
Stage IV
Ann Arbor System in Adult
Stage I
Stage II
Stage III
Stage IV
Differential Diagnosis
Risk Factors for BL
Treatment and Management Issues
Toxicity and Side Effect Management
Complications of BL
Prognosis of BL
Multi-Professional Healthcare Team Management Need
Research and Innovations in the Diagnosis and Treatment Advances of Bl
Research in Diagnostic Advances of BL
Gene Targets
Issues in BL Pathogenesis and Cellular Genetic Change
The Effects of C-Myc Overexpression and C-Myc Translocation
Research Efforts and Updates on Myc Gene
Mechanisms Whereby C-Myc Drives BL Tumorigenesis
Recent Studies to Determine the Role of C-Myc in Tumorigenesis
Matters in Histologic Diagnosis of BL
Recent Advances in the Molecular Diagnosis of BL
Research in the Treatment Advances of BL
Revelation of New Approaches to Burkitt Lymphoma Treatment and Management
Less Toxic Alternative to Standard Treatment for Adults with BL
Discussion
Conclusion
References
Biographical Sketches
Chapter 2
Burkitt Lymphoma in Patients with Primary Immunodeficiency Disorders
Abstract
1. Introduction
2. Clinical Cases
2.1. Case-1 [15]
2.2. Case 2 [16]
3. Epidemiology
3.1. Data from Large PIDs Registries
3.2. Age and Gender Distribution
3.3. Burkitt Lymphoma in Patients with PIDs
4. Lymphomagenesis in Immune Deficiency Disorders
4.1. Cancer Immune Surveillance in Immune Deficiency Disorders
4.2. EBV and PIDs-Associated Lymphoma
4.3. Mechanisms of Lymphomagenesis in PIDs
5. Primary Immune Deficiency Disorders Commonly Associated with Lymphoma
5.1. Chromosome Instability Syndromes
5.1.1. Ataxia Telangiectasia (AT)
5.1.2. Nijmegen Breakage Syndrome (NBS)
5.1.3. Bloom Syndrome (BS)
5.2. Wiskott-Aldrich Syndrome (WAS)
5.3. Common Variable Immunodeficiency (CVID)
5.4. X-linked Lymphoproliferative Disease Type 1 (XLP1)
5.5. Other PIDs Associated with Burkitt Lymphoma
6. Treatment Considerations
6.1. Index of Suspicion of PIDs in Newly Diagnosed B-NHL
6.2. Chemotherapy
6.3. Special Treatment Considerations in PIDs Associated BL
6.3.1. Chromosome Instability Syndromes
6.3.2. Ataxia Telangiectasia and Nijmegen Breakage Syndrome
6.3.3. Nijmegen Breakage Syndrome
6.3.4. Bloom Syndrome
6.3.5. Wiscott Aldrich Syndrome
6.3.6. X-linked Lymphoproliferative Disease Type 1
6.4. Treatment Modalities Other Than Chemotherapy
6.4.1. Targeted Immunotherapy
6.4.2. EBV Specific Cytotoxic T cells
6.4.3. Allogeneic Hematopoietic Stem Cell Transplantation (HSCT)
7. Unique Long Term Risks
7.1. Malignancies
7.2. Chronic Lung Disease
Conclusion and Future Perspectives
Acknowledgments
References
Biographical Sketch
Chapter 3
Post Solid Organ Transplant Burkitt Lymphoma
Abstract
1. Introduction
2. Epidemiology
2.2. Transplant Risk Factors
3. Biopathology
3.1. Pathologic and Genetic Features
3.2. The Role of EBV in PTLD and PSOT-BL
3.3. Epstein-Barr Virus DNAemia Load
4. Clinical Features and Involved Sites
5. Treatment
5.1. Are Treatment Strategies for M-PTLD Effective in BL?
5.2. Response Based Treatment Strategies
5.3. Chemotherapy Intensity in PSOT-BL
5.4. Cytotoxic T Cell in PTLD and BL
5.5. CAR-T in PTLD and PSOT-BL
6. Prognostic Factors
7. Outcome
7.1. Potential Long-Term Effects and Need for Therapy Modifications
8. Organ Specific Treatment Considerations
8.1. Cardiac
8.2. Renal and Hepatic Toxicity
Conclusion and Perspectives
Acknowledgements
References
Chapter 4
Burkitt Lymphoma
Abstract
Introduction
Epidemiology
Pathobiology
Clinical Presentation
Diagnosis
Prognosis
Treatment
First-Line Therapy for BL
Treatment Regimen in the Context of CNS Involvement
Treatment of Relapsed and Refractory BL
Targeted Therapies
Chimeric Antigen Receptor (CAR) T-Cell Therapy
Bispecific T-Cell Engagers (BiTEs)
Conclusion
References
Chapter 5
Examining the Therapeutic Advances for Burkitt Lymphoma
Abstract
Introduction
Methods
Results
Etiology/Causes of BL
Types of BL
Endemic BL
Sporadic BL
Immunodeficiency-Related BL
Epidemiology of BL
Clinical Presentations of BL
Clinical Evaluations of BL
EBV and BL
Diagnosis of BL
Determination of the Staging of BL
Histology/Morphology and Cytogenetics
Immunophenotype and Molecular Signature
Treatment Choices for BL
Chemotherapy
Targeted Therapy
Central Nervous System Prophylaxis
Progress on the Role of HSCT in the Treatment of BL
Progress on Exploring the Use of Immunotherapy and Experimental Agents in the Treatment of BL
The Effect of Ofatumumab and Obinutuzumab GA101
Chimeric Antigen Receptor (CAR T-Cell)
Immune-Checkpoint (PD-1 – PD-L1)
Future Directions in MYC Inhibition
Progress on New Finding Therapies for BL
Prognosis of Therapies
Conclusion
References
Chapter 6
Burkitt Lymphoma: Evaluation of Characteristics, Clinical Updates and Management Approaches Trends
Abstract
Introduction
Methods
Results
Etiology of BL
Clinical Characteristics and Manifestations
Epidemiological Updates
Diagnosis Updates
Differential Diagnosis
Trends in the Management Approaches of BL
Progress on DA-EPOCH-R
Situation of When a Patient Is Too Ill to Receive Chemotherapy
When There Is a Lack of Referral to a Specialist/Secondary/Tertiary Care Unit
Updates on Prognosis of BL
Conclusion
References
Index
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