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Questions and Answers
What is the primary site of haemopoiesis?
What is the primary site of haemopoiesis?
Bone marrow
Which of the following cells are formed from pluripotent stem cells? (Select all that apply)
Which of the following cells are formed from pluripotent stem cells? (Select all that apply)
Haemopoietic growth factors are not relevant to the process of blood cell formation.
Haemopoietic growth factors are not relevant to the process of blood cell formation.
False
The cells that give rise to red blood cells, granulocytes, monocytes, and platelets are called ______.
The cells that give rise to red blood cells, granulocytes, monocytes, and platelets are called ______.
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Which proteins are frequently mutated in haemopoietic malignancies? (Select all that apply)
Which proteins are frequently mutated in haemopoietic malignancies? (Select all that apply)
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What role does autophagy play in cells?
What role does autophagy play in cells?
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Which of the following proteins are part of the Janus-associated G-CSF kinase family?
Which of the following proteins are part of the Janus-associated G-CSF kinase family?
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What do growth factors induce in progenitor cells?
What do growth factors induce in progenitor cells?
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JAK proteins act on ______ cells.
JAK proteins act on ______ cells.
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Which signal transducer is activated by phosphorylated JAK proteins?
Which signal transducer is activated by phosphorylated JAK proteins?
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The JAK2 gene mutation is the cause of polycythemia vera.
The JAK2 gene mutation is the cause of polycythemia vera.
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What is interphase divided into?
What is interphase divided into?
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Which process describes programmed cell death?
Which process describes programmed cell death?
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What do adhesion molecules mediate?
What do adhesion molecules mediate?
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The gene ______ is overexpressed in follicular lymphoma to prevent apoptosis.
The gene ______ is overexpressed in follicular lymphoma to prevent apoptosis.
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What are the main pathways that activate caspases?
What are the main pathways that activate caspases?
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What is the approximate number of mature blood cells produced by one stem cell after 20 cell divisions?
What is the approximate number of mature blood cells produced by one stem cell after 20 cell divisions?
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Where does haemopoiesis occur in adults?
Where does haemopoiesis occur in adults?
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What occurs to the number of stem cells with aging?
What occurs to the number of stem cells with aging?
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Stem cells can accumulate genetic mutations with age.
Stem cells can accumulate genetic mutations with age.
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What binds to its receptor CXCR4 on HSC?
What binds to its receptor CXCR4 on HSC?
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Which transcription factor is involved in erythropoietic and megakaryocytic differentiation?
Which transcription factor is involved in erythropoietic and megakaryocytic differentiation?
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Haemopoietic growth factors only act locally.
Haemopoietic growth factors only act locally.
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Which of the following is NOT a characteristic of myeloid and lymphoid growth factors?
Which of the following is NOT a characteristic of myeloid and lymphoid growth factors?
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What is required for stem cell survival and differentiation?
What is required for stem cell survival and differentiation?
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Which factors are involved in mediating apoptosis following DNA damage?
Which factors are involved in mediating apoptosis following DNA damage?
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Necrosis is a form of programmed cell death.
Necrosis is a form of programmed cell death.
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What initiates apoptosis?
What initiates apoptosis?
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The intracellular ratio of pro-apoptotic proteins like ______ to anti-apoptotic proteins like BCL-2 affects cytochrome c release.
The intracellular ratio of pro-apoptotic proteins like ______ to anti-apoptotic proteins like BCL-2 affects cytochrome c release.
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What role do growth factors play in apoptosis?
What role do growth factors play in apoptosis?
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What is haemopoiesis?
What is haemopoiesis?
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Match the following components with their roles in apoptosis:
Match the following components with their roles in apoptosis:
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What is autophagy?
What is autophagy?
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Where does haemopoiesis occur in adults?
Where does haemopoiesis occur in adults?
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What are the progeny of centrocytes known as?
What are the progeny of centrocytes known as?
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Which infections are associated with acute lymphocytosis?
Which infections are associated with acute lymphocytosis?
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Which of the following is a chronic infection associated with lymphocytosis?
Which of the following is a chronic infection associated with lymphocytosis?
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What causes Infectious mononucleosis?
What causes Infectious mononucleosis?
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EBV infection rarely causes symptomatic disease.
EBV infection rarely causes symptomatic disease.
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Chronic lymphoid leukaemias can lead to __________.
Chronic lymphoid leukaemias can lead to __________.
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What is the role of intrinsic factor in B12 absorption?
What is the role of intrinsic factor in B12 absorption?
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What is the most important site of haemopoiesis during fetal life?
What is the most important site of haemopoiesis during fetal life?
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What is megaloblastic anaemia primarily characterized by?
What is megaloblastic anaemia primarily characterized by?
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The abnormal appearance of bone marrow erythroblasts in megaloblastic anaemia is typically caused by __________.
The abnormal appearance of bone marrow erythroblasts in megaloblastic anaemia is typically caused by __________.
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Alcohol consumption can lead to macrocytic anaemia.
Alcohol consumption can lead to macrocytic anaemia.
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Which factor contributes to the need for injections of hydroxocobalamin?
Which factor contributes to the need for injections of hydroxocobalamin?
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What are two important examples relevant to gene expression that do not affect DNA sequence?
What are two important examples relevant to gene expression that do not affect DNA sequence?
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Haemopoiesis in adults is confined to the peripheral skeleton.
Haemopoiesis in adults is confined to the peripheral skeleton.
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What role do transcription factors play in gene expression?
What role do transcription factors play in gene expression?
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Apoptosis is a physiological process of cell death resulting from activation of __________.
Apoptosis is a physiological process of cell death resulting from activation of __________.
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Which of these proteins determines the cell's susceptibility to apoptosis?
Which of these proteins determines the cell's susceptibility to apoptosis?
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Where do stem cells reside in the body?
Where do stem cells reside in the body?
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B cells can recognize antigens through their surface immunoglobulin without any assistance.
B cells can recognize antigens through their surface immunoglobulin without any assistance.
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What occurs in the germinal centers as a result of continuing response to antigenic stimulation?
What occurs in the germinal centers as a result of continuing response to antigenic stimulation?
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The antigen recognition site of the TCR is joined to several other __________ in the CD3 complex.
The antigen recognition site of the TCR is joined to several other __________ in the CD3 complex.
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What class of T helper cells produces IL-2, TNF-b, and g-interferon (IFN-g)?
What class of T helper cells produces IL-2, TNF-b, and g-interferon (IFN-g)?
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Study Notes
Authors and Edition
- A.Victor Hoffbrand: Emeritus Professor of Haematology at University College London.
- Paul A.H. Moss: Professor of Haematology at the University of Birmingham.
- Seventh Edition published in 2016, continuing from previous editions dating back to 1980.
Publication Details
- Published by John Wiley & Sons, Ltd.
- Copyright details ensure protection of content as per the UK Copyright, Designs and Patents Act 1988.
Important Content Topics
- Advances in understanding blood and lymphatic diseases due to next-generation DNA sequencing.
- Discovery of genetic mutations linked to conditions such as myeloproliferative diseases and Waldenström’s macroglobulinemia.
Key Developments in Treatment
- Development of therapies that improve patient life expectancy, such as B cell receptor signaling inhibition and JAK2 inhibitors for chronic conditions.
- Introduction of new anticoagulants that simplify treatment for arterial and venous thrombosis, minimizing monitoring needs.
Structure of the Textbook
- Each chapter includes Key Topics and concludes with a Summary for study purposes.
- Covers various aspects of haematology, including haemopoiesis, types of anaemias, hematological malignancies, and blood disorders.
Chapter Highlights
- Haemopoiesis: Blood cell formation from pluripotent stem cells in the bone marrow.
- Erythropoiesis and Anaemia: Discussion of red blood cell formation and associated conditions.
- Malignant Conditions: Insights on acute and chronic leukaemias, lymphomas, and myeloma.
- Coagulation Disorders: Overview of bleeding disorders, pathogenesis, and treatment strategies.
Educational Features
- Inclusion of diagrams and tables for visual understanding.
- Companion website offers additional resources like multiple-choice questions.
Research and Clinical Implications
- Recognition of the impact of genetic mutations on disease progression and treatment responses.
- Need for ongoing education and adaptation of practices in haematology due to evolving research and therapies.
Acknowledgments
- Thanks to contributors who aided in the preparation of the book, including suggestions from experts in the field and providers of illustrative materials.### Stem Cells and Niches
- Stem cells are located in the bone marrow, within microenvironments known as niches.
- They are regulated by stromal cells that facilitate growth and differentiation.
Apoptosis Mechanism
- Apoptosis is a deliberate process of programmed cell death activated by caspases.
- The balance between pro-apoptotic (e.g., BAX) and anti-apoptotic proteins (e.g., BCL-2) influences a cell's likelihood of undergoing apoptosis.
Immune System Responses
- Antigen recognition is critical for immune responses, with CD4+ T cells (helper T cells) classified into Th1 and Th2 subtypes based on cytokine profiles.
- Th1 cells release IL-2, TNF-β, and γ-interferon, enhancing cell-mediated immunity.
- Th2 cells produce IL-4 and IL-10, primarily aiding in antibody production.
B Cell Activation
- B cells recognize antigens via surface immunoglobulins, while T cells assist in producing antibodies for certain antigens.
- Germinal centers within the secondary lymphoid organs form as a result of ongoing immune responses.
Germinal Center Formation
- Activated B cells migrate to germinal centers, where they undergo division and somatic hypermutation to enhance antibody affinity.
- Centroblasts in the dark zone mutate their immunoglobulin genes, while centrocytes in the light zone are selected based on antigen affinity.
Lymphocytosis
- Lymphocytosis refers to an increased number of lymphocytes, often seen in children due to infections.
- Common causes include viral infections (e.g., infectious mononucleosis, HIV) and chronic conditions (e.g., tuberculosis, non-Hodgkin lymphoma).
- Conditions resulting in lymphocytosis can be categorized into acute and chronic infections, with specific examples noted for clarity.### Glandular Fever and Infectious Mononucleosis
- Glandular fever refers to a disease characterized by fever, sore throat, lymphadenopathy, and atypical lymphocytes in the blood.
- Infectious mononucleosis, often caused by Epstein-Barr virus (EBV), can occur following primary EBV infection.
- Most EBV infections are subclinical; significant disease occurs in a minority of cases.
- Atypical lymphocytosis is a hallmark of infectious mononucleosis and occurs due to T cell expansions reacting to infected B lymphocytes.
Macrocytic Anaemias
- Macrocytic anaemias are identified by larger-than-normal red blood cells (MCV >98 fL).
- Common causes include vitamin B12 and folate deficiencies, along with alcohol use, liver disease, and certain drugs.
- Clinical features to consider: hypersegmented neutrophils, leukopenia, and thrombocytopenia.
- Important diagnostic steps include assessing dietary history, conducting serum B12 and folate tests, and evaluating for autoimmune disorders or previous gastrointestinal issues.
- Symptoms like jaundice, glossitis, and neuropathy may indicate megaloblastic anaemia.
Vitamin B12 and Folate Deficiency
- Vitamin B12 malabsorption often occurs with pernicious anaemia, where intrinsic factor production is compromised.
- Folate deficiency can arise from poor diet or malabsorption conditions like gluten-induced enteropathy.
- Both deficiencies lead to megaloblastic anaemia, affecting the morphological appearance of bone marrow erythroblasts.
- Treatment involves hydroxocobalamin injections for B12 deficiency and oral folate supplementation for folate deficiency.
Haemopoiesis Overview
- Early haemopoiesis occurs in the yolk sac and later shifts to the AGM (aorta-gonads-mesonephros), liver, and spleen during development.
- Bone marrow becomes the primary source of new blood cells from 6-7 months of fetal life onward through adulthood.
- In adults, haemopoiesis is largely limited to the vertebrae, ribs, sternum, skull, sacrum, pelvis, and proximal humerus and femur.
- Approximately 50% of bone marrow comprises fatty tissue, with potential for reactivation in certain diseases.
Hematopoietic Stem and Progenitor Cells
- Haematopoiesis initiates with pluripotential hematopoietic stem cells (HSCs), which can self-renew and differentiate into various blood cell lineages.
- HSCs are rare, constituting about 1 in every 20 million nucleated cells.
- The differentiation process involves transitioning through committed progenitor cells, which have restricted developmental potential.
- Many hematopoietic growth factors influence the production of different blood cells depending on physiological demands.
Bone Marrow Microenvironment
- Bone marrow stroma provides a supportive environment for stem cell maintenance and differentiation.
- Interactions between stem cells and stromal cells facilitate survival, self-renewal, and production of differentiated cells.
- Key factors include the stem cell factor (SCF) and jagged proteins that interact with their respective receptors on HSCs, ensuring proper hematopoietic function.
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Description
This quiz is designed to assess your understanding of Hoffbrand's Essential Haematology. It covers key concepts, terminology, and essential knowledge in the field of haematology as presented by A. Victor Hoffbrand and Paul A.H. Moss. Enhance your grasp of important topics and prepare for further studies in haematology.
