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Questions and Answers
What is the main function of mast cells in inflammation?
What is the main function of mast cells in inflammation?
Which type of cells are classified as agranulocytes?
Which type of cells are classified as agranulocytes?
What is the largest secondary lymphoid organ?
What is the largest secondary lymphoid organ?
Which process directly leads to the production of erythrocytes?
Which process directly leads to the production of erythrocytes?
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Bone marrow contains which type of stem cells crucial for blood cell production?
Bone marrow contains which type of stem cells crucial for blood cell production?
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What role do lymph nodes play in the body’s immune response?
What role do lymph nodes play in the body’s immune response?
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What stimulates the production of erythrocytes from erythroblasts?
What stimulates the production of erythrocytes from erythroblasts?
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Which of the following organs is primarily active in blood cell production during adulthood?
Which of the following organs is primarily active in blood cell production during adulthood?
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What is a defining characteristic of Hemophilia A?
What is a defining characteristic of Hemophilia A?
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What percentage of children with acute lymphoblastic leukemia (ALL) can expect a 5-year survival rate?
What percentage of children with acute lymphoblastic leukemia (ALL) can expect a 5-year survival rate?
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Which condition is known as a fatal form of alpha-thalassemia?
Which condition is known as a fatal form of alpha-thalassemia?
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What condition is characterized by a platelet destruction rate that exceeds production?
What condition is characterized by a platelet destruction rate that exceeds production?
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Which type of lymphoma is most commonly seen in childhood?
Which type of lymphoma is most commonly seen in childhood?
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Which factor is NOT associated with the etiology of Non-Hodgkin lymphoma (NHL)?
Which factor is NOT associated with the etiology of Non-Hodgkin lymphoma (NHL)?
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What is the primary function of erythrocytes in the blood?
What is the primary function of erythrocytes in the blood?
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Which component of blood serves as a carrier and helps control plasma oncotic pressure?
Which component of blood serves as a carrier and helps control plasma oncotic pressure?
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What is a hallmark of acute leukemia?
What is a hallmark of acute leukemia?
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Which form of alpha-thalassemia results from having two defective alpha chain forming genes?
Which form of alpha-thalassemia results from having two defective alpha chain forming genes?
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What is the lifespan of a mature erythrocyte?
What is the lifespan of a mature erythrocyte?
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Which type of white blood cell is primarily involved in early inflammation?
Which type of white blood cell is primarily involved in early inflammation?
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Which of the following is NOT a function of the hematologic system?
Which of the following is NOT a function of the hematologic system?
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What percentage of blood volume is made up of plasma?
What percentage of blood volume is made up of plasma?
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Which plasma protein is primarily responsible for blood clotting?
Which plasma protein is primarily responsible for blood clotting?
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What type of blood component is primarily responsible for defending the body against infections?
What type of blood component is primarily responsible for defending the body against infections?
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What is the function of erythropoietin in the regulation of erythropoiesis?
What is the function of erythropoietin in the regulation of erythropoiesis?
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What is the primary site for the destruction of aged erythrocytes?
What is the primary site for the destruction of aged erythrocytes?
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Which of the following vitamins is not essential for hemoglobin synthesis?
Which of the following vitamins is not essential for hemoglobin synthesis?
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What component of hemoglobin is responsible for binding oxygen?
What component of hemoglobin is responsible for binding oxygen?
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What happens to protoporphyrin during the destruction of senescent erythrocytes?
What happens to protoporphyrin during the destruction of senescent erythrocytes?
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Which of the following best describes endomitosis in the development of platelets?
Which of the following best describes endomitosis in the development of platelets?
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What stimulates the production and release of erythropoietin?
What stimulates the production and release of erythropoietin?
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Which type of leukocytes are released into the bloodstream before they fully mature?
Which type of leukocytes are released into the bloodstream before they fully mature?
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What is the primary role of platelets in hemostasis?
What is the primary role of platelets in hemostasis?
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Which factor initiates the intrinsic pathway of the coagulation cascade?
Which factor initiates the intrinsic pathway of the coagulation cascade?
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What is the lifespan of circulating platelets before they lose functional capacity?
What is the lifespan of circulating platelets before they lose functional capacity?
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What is the role of transcript factors in primary hemostasis?
What is the role of transcript factors in primary hemostasis?
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Which component is responsible for the breakdown of fibrin clots?
Which component is responsible for the breakdown of fibrin clots?
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Which statement accurately describes the process of clot retraction?
Which statement accurately describes the process of clot retraction?
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What is the primary action of the fibrinolytic system?
What is the primary action of the fibrinolytic system?
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Which of the following tests is used to evaluate bone marrow function?
Which of the following tests is used to evaluate bone marrow function?
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What is the most common blood disorder in infancy and childhood?
What is the most common blood disorder in infancy and childhood?
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Which of the following is a manifestation of sickle cell disease?
Which of the following is a manifestation of sickle cell disease?
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What causes sickling of red blood cells in sickle cell disease?
What causes sickling of red blood cells in sickle cell disease?
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Which complication is associated with hemolytic disease of the fetus and newborn (HDFN)?
Which complication is associated with hemolytic disease of the fetus and newborn (HDFN)?
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What is the genetic inheritance pattern of thalassemias?
What is the genetic inheritance pattern of thalassemias?
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Which type of anemia results from maternal antibodies acting against fetal antigens?
Which type of anemia results from maternal antibodies acting against fetal antigens?
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In which condition are both alpha and beta globin chains affected?
In which condition are both alpha and beta globin chains affected?
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Which statement describes the condition known as beta-thalassemia major?
Which statement describes the condition known as beta-thalassemia major?
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Study Notes
Structure and Function of the Hematologic System
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Learning Objectives: Students will compare and contrast various hematologic disorders, including etiology, pathogenic mechanisms, altered morphology/physiology, signs/symptoms, genetic/environmental factors, and complications. Students will also examine risk factors. Specific disorders listed include iron deficiency anemia, megaloblastic anemias, hemolytic anemias (ex. sickle cell anemia), Hodgkin/Non-Hodgkin Lymphomas, acute and chronic myelogenous leukemia, multiple myeloma, thrombocytopenia, thrombocytosis, qualitative platelet disorders, and coagulation disorders (clotting factor deficiencies and DIC).
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Functions of the Hematologic System: Delivering substances for cellular metabolism, removing waste products, defending against microorganisms and injury, and maintaining acid-base balance.
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Composition of Blood: Consists of 91% water and 9% solutes (6 quarts or 5.5 liters). Cellular components comprise 45% of the blood volume, and plasma accounts for 50-55%. Plasma contains organic and inorganic elements and plasma proteins (including albumins, globulins, and clotting factors, mainly fibrinogen). Globulins are carriers and immunoglobulins.
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Cellular Components:
- Erythrocytes (Red Blood Cells): Most abundant blood cells, responsible for tissue oxygenation. They are biconcave and lack a nucleus and cytoplasmic organelles, with a lifespan of 120 days.
- Leukocytes (White Blood Cells): Defend against infection and remove debris. Granulocytes (containing granules) have inflammatory and immune functions and can move by ameboid movement (diapedesis). Specific examples of granulocytes include neutrophils (60-70%), eosinophils (2-4%), and basophils (0.5-1%). Mast cells are functionally and structurally similar to basophils.
- Agranulocytes: These include monocytes (immature macrophages, 3-8%), macrophages (monocytes in tissues), lymphocytes (including T cells, B cells, plasma cells, and natural killer [NK] cells, accounting for 20-25%), and platelets (disk-shaped cytoplasmic fragments of megakaryocytes, essential for blood clotting).
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Lymphoid Organs: Include primary organs (thymus and bone marrow), and secondary organs (spleen, lymph nodes).
- Spleen: Largest secondary lymphoid organ, with splenic pulp containing lymphoid tissue, macrophages, and venous sinuses. It plays a role in phagocytosis of old/damaged blood cells and blood storage (300 ml). Involved in immune and hematologic systems.
- Lymph Nodes: Part of the immune and hematologic systems, assisting with maturation of lymphocytes, transportation of lymphatic fluid back to the circulation, and cleansing of lymphatic fluid for microorganisms and foreign particles.
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Hematopoiesis: The process of blood cell production, occurring in two stages: mitosis (proliferation), and maturation and differentiation.
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Bone Marrow: Contains red (active) and yellow (inactive) marrow. Red marrow is the source for blood cell production in adults and is located in pelvic bones, vertebrae, cranium/mandible, sternum/ribs, and humerus/femur.
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Blood Cell Maturation: The process can be visualized by diagrams illustrating the different types and stages of blood cells during development. Information about specific and distinct cells include Myeloblast, Promyelocyte, Myelocyte, Metamyelocyte, Band, Basophil, Eosinophil, Neutrophil, Monocyte, Megakaryocytes, Platelets, Erythrocytes, and Lymphocytes.
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Erythropoiesis: The specific process of red blood cell production. Key player is erythropoietin (EPO), produced by peritubular cells of the kidney, which is stimulated by hypoxia. Erythropoiesis involves different stages that mature from a stem cell to a committed proerythroblast, then normoblasts or reticulocytes to an erythrocyte.
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Hemoglobin: Oxygen-carrying protein. A single erythrocyte contains up to 300 hemoglobin molecules. Four colorful iron-protoporphyrin complexes in each hemoglobin molecule bind to one molecule of oxygen. Iron is necessary and is found in hemoglobin.
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Hemoglobin Synthesis: Requires specific building blocks (proteins and specific amino acids) and vitamins (B12, B6, B2, E, C, folic acid, and pantothenic acid, and niacin), and minerals (iron and copper).
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Normal Destruction of Senescent Erythrocytes: Aged red blood cells are broken down by macrophages (MPS) in the spleen (primary). The liver takes over if the spleen isn't functional. Globin chains are broken down into amino acids and porphyrin is reduced to bilirubin which is transported to the liver for secretion in the bile.
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Development of Leukocytes and Platelets: Stem cells produce different types of these cells in the bone marrow. Leukocytes (and their maturation) are encouraged by growth factors and colony-stimulating factors. Production also increases when there is infection, release of steroids, increased heart rate, pain, or nausea/vomiting. Platelet production and maintenance are dependent on thrombopoietin and IL-11. Platelets circulate for 10 days before losing functional capacity, and then are destroyed in the spleen.
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Hemostasis: The process of stopping bleeding. Requirements include platelets (adhesion, activation, and aggregation), clotting factors, and endothelial cells.
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Coagulation Cascade: Intrinsic and extrinsic pathways. Involved in secondary hemostasis.
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Control of Hemostatic Mechanisms: Clot retraction (fibrin strands become shorter and denser, facilitated by platelets); Lysis of blood clots (fibrinolytic system involving plasminogen, plasmin, fibrin degradation products, and D-dimers).
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Evaluation of the Hematologic System: Tests used to evaluate bone marrow function include bone marrow aspiration, bone marrow biopsy, and bone marrow iron stores measurement. Blood tests, such as a complete blood count, iron profile, serum vitamin B12, and serum folate measurements, help in the assessment.
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Alterations of Hematologic Function in Children: Includes descriptions of specific acquired and inherited disorders in erythrocytes and coagulation/platelets (ex. iron deficiency anemia, hemolytic disease of the fetus and newborn, sickle cell disease, thalassemias, and hemophilias). Detailed information about the etiology, manifestations, and further complications for each disease.
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Acquired Disorders of Erythrocytes: Includes iron deficiency anemia (most common), congenital hemolytic anemia (HDFN/erythroblastosis fetalis--alloimmune diseases). Etiology, clinical manifestations, and additional complications are noted.
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Inherited Disorders of Erythrocytes: Detailed information about hemoglobin abnormalities (e.g. Sickle cell disease, Sickle cell trait, other forms, manifestations and clinical consequences) . Alpha-thalassemia, and Beta-thalassemia (alpha and beta thalassemias, major vs minor, clinical characteristics).
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Inherited Coagulation and Platelet Disorders: Inherited disorders involving blood clotting abnormalities are highlighted (hemophilias [A, B, C]). Etiologies and symptoms are described .
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Antibody-Mediated Hemorrhagic Disease: Idiopathic thrombocytopenic purpura (ITP) characterized by platelet destruction. Etiology, manifestations (bruising and petechial rash, and complications) and prognosis are detailed.
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Leukemia: Most common childhood malignancy, including acute lymphoblastic leukemia (ALL) and acute myelogenous leukemia (AML). Etiology is highlighted along with clinical manifestations(pallor, fatigue, purpura, fever), blast cell involvement, and prognosis/long-term survival rate.
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Lymphoma: Non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma. Inherited and acquired forms, specific cells (B-cells) involved, possible etiology, common sites of involvement (mediastinal mass), diagnostic considerations and treatments, typical prognosis, and other significant clinical features are included, are all highlighted.
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