Benign and Malignant Disorders of Large Granular Lymphocytes: Diagnostic and Therapeutic Pearls


Ling Zhang, M.D. and Lubomir Sokol, M.D., Ph.D. (Editors)
H Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, FL, US

Series: Recent Advances in Hematology Research
BISAC: MED038000

Natural killer (NK) cells are important effector cells of innate immune system implicated in many physiological processes including elimination of cancer cells and virus infected cells. NK cells comprise a majority of large granular lymphocytes circulating in peripheral blood with a minority derived from T cell lineage. Even though NK cells were first described more than 40 years ago, it was not until the 1980s and 1990s when immunophenotyping was incorporated into clinical diagnostic methods and resulted in discovery of distinct disorders of large granular lymphocytes and NK cells. Since then, significant progress was made in our better understanding of immunophenotypic and genotypic characteristics, biology, functions as well as disorders of these cells. Most recently, clinical studies using NK-cell based immunotherapy have shown promising results in treatment of some of malignant diseases.

Disorders of large granular lymphocytes and NK cells are rare comprising only about 1% of all lymphoid malignancies in western countries. The rarity of these conditions was a main reason that the progress in our understanding of pathogenesis and development of novel therapeutic approaches has been delayed compared to development in more common B cell lymphoid malignancies. The low incidence of these diseases and scarcity of prospective clinical trials also limit the availability of evidence based research literature as well as comprehensive reviews about NK cell disorders. Thus, the editors decided to take on the challenging task and summarize our current knowledge about malignant and benign diseases of large granular lymphocytes in this book based on the best available evidence. The editors selected topics most relevant to clinical practice in order to provide a useful guide for practicing physicians. Chapters describing four disorders (T-cell large granular lymphocytic leukemia, chronic lymphoproliferative disorder of NK cells, extranodal NK cell lymphoma and aggressive NK cell leukemia) incorporated into most recent 2016 revision of classification of lymphoid malignancies are separated into experimental, diagnositc and clinical parts for easier understanding and reading. We are aware of challenges and inherited limitations of any larger project like this one due to a rapid progress especially in the field of genomics, which may not be incorporated in this book before it is published. The editors and contributing authors would like to thank the publisher NOVA for their support.

Table of Contents

Table of Contents


Chapter 1. Molecular Biology and Genetics of LGL Leukemia (pp. 1-24)
T. Tiffany Wang, Jeffrey C. Xing, Thomas L. Olson, David J. Feith, and Thomas P. Loughran Jr.

Chapter 2. Abnormal Function of Innate and Adaptive Immune Systems in Large Granular Lymphocytic Leukemia (pp. 25-42)
Lili Yang, Houfang Sun, and Sheng Wei

Chapter 3. NK Cell Biology and the Role of NK Cells in Hematopoietic Stem Cell Transplantation and Cellular Immunotherapy (pp. 43-74)
Rawan Faramand and Asmita Mishra

Chapter 4. Pathogenesis of Myelodysplastic Syndromes Harboring Clonal Large Granular Leukocytes (pp. 75-100)
P.K. Epling-Burnette

Chapter 5. Benign T-LGL and NK Cell Proliferations Evolved After Hematopoietic Cell Transplantation, and Anti-Neoplastic Agents (pp. 101-114)
Taiga Nishihori

Chapter 6. Routine Diagnostic Approaches of T-Cell large Granular Lymphocytic Leukemia (pp. 115-148)
Ling Zhang

Chapter 7. Chronic Lymphoproliferative Disorder of Natural Killer Cells: An Entity Falling to a Benign or Malignant Process (pp. 149-164)
Prerna Rastogi, Rawan Faramand, Lubomir Sokol, and Ling Zhang

Chapter 8. T-Cell Large Granular Lymphocytic Leukemia: Conventional Treatment Approaches and Novel Strategies (pp. 165-182)
Magali Van den Bergh and Lubomir Sokol

Chapter 9. Extranodal NK/T-Cell Lymphoma, Nasal Type-Diagnostic Approaches (pp. 183-224)
Stefanie Grewe, Carolina Dominguez, and Haipeng Shao

Chapter 10. Clinical Intervention and Novel Therapeutic Strategies for Extranodal NK/T Cell Lymphoma, Nasal Type (pp. 225-248)
George Yang, Daniel Grass, Kamran Ahmed, and Sungjune Kim

Chapter 11. Aggressive Natural Killer (NK) Cell Leukemia (pp. 249-264)
Xiaohui Zhang

Chapter 12. Clinical Manifestation and Management of Aggressive Natural Killer Cell Leukemia (pp. 265-278)
Emilie Wang, Ling Zhang, and Lubomir Sokol

Chapter 13. Natural Killer Cell Dysfunction in Primary and Acquired Hemophagocytic Lymphohistiocytosis (pp. 279-300)
Seongseok Yun and Ling Zhang

Editors’ Contact Information (pp. 301-302)

Index (pp. 303)

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