McCance 31 Book. Hematology in Human Development
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Questions and Answers

During what stage of human development does the production of erythrocytes begin in the yolk sac?

  • Shortly after 2 weeks of gestation (correct)
  • At approximately the eighth week of gestation
  • During childhood
  • Before 2 weeks of gestation

    • Which of the following areas does hematopoietic marrow retreat to during childhood?

  • Ends of the long bones
  • Vertebrae, ribs, sternum, pelvis, scapulae, skull, and proximal ends of the femur and humerus (correct)
  • Bony cavities of the entire axial skeleton
  • Middle of the shaft of the long bones

    • What is the primary function of erythrocytes?

  • To transport oxygen to tissues (correct)
  • To clot blood
  • To produce antibodies
  • To fight infections

    • What happens to hematopoietic marrow in some bones during childhood?

    <p>It is replaced by fatty marrow (B)</p> Signup and view all the answers

    In diseases characterized by hemolysis, what is the maximum increase in erythrocyte production compared to normal levels?

    <p>Eight times (A)</p> Signup and view all the answers

    What is the most common cause of Iron Deficiency Anemia (IDA) during the first few years of life?

    <p>Inadequate intake (B)</p> Signup and view all the answers

    In what age group is the prevalence of IDA highest in the United States?

    <p>Toddlers (C)</p> Signup and view all the answers

    Which of the following is NOT mentioned as a possible cause of chronic IDA from occult (hidden) blood loss?

    <p>Nutritional deficiencies (C)</p> Signup and view all the answers

    At what age is the neutrophil count similar to that of an adult?

    <p>Around 4 years of age (D)</p> Signup and view all the answers

    What is the most common cause of IDA during childhood and adolescence?

    <p>Blood loss (B)</p> Signup and view all the answers

    Which blood cell type is elevated in the first year of life, compared to children, teenagers, or adults?

    <p>Eosinophils (B)</p> Signup and view all the answers

    What is the significance of the lymphocyte count at birth and in the first year of life?

    <p>It declines steadily throughout childhood and adolescence. (C)</p> Signup and view all the answers

    Which of these factors is NOT related to the incidence of IDA?

    <p>Gender (C)</p> Signup and view all the answers

    Which of the following factors influences the mother's capacity to produce anti-Rh antibodies?

    <p>The mother's genetic predisposition to produce antibodies against the Rh antigen D. (A), The amount of fetal-to-maternal bleeding during pregnancy. (B), The occurrence of any bleeding earlier in the pregnancy. (C)</p> Signup and view all the answers

    What is the primary risk associated with sequestration crisis in young children with SCA?

    <p>Infection and septicemia (C)</p> Signup and view all the answers

    What condition may contribute to hyperhemolytic crisis in individuals with SCA?

    <p>G6PD deficiency (B)</p> Signup and view all the answers

    Which treatment strategy is proposed to reduce the risk of infections in children with SCA?

    <p>Prophylactic antibiotics and vaccination (D)</p> Signup and view all the answers

    What symptom is NOT typically associated with hyperhemolytic crisis?

    <p>Fatigue (D)</p> Signup and view all the answers

    Which organisms are most commonly responsible for infections in patients with impaired splenic function?

    <p>Pneumococcus pneumoniae and Haemophilus influenzae (B)</p> Signup and view all the answers

    What is a potential outcome of severe splenic damage in adults with SCA?

    <p>Splenic infarction (C)</p> Signup and view all the answers

    What approach is recommended for managing fever in children with SCA?

    <p>Aggressive management and early diagnosis (A)</p> Signup and view all the answers

    What is the primary purpose of treatment for patients with SCD?

    <p>Preventing complications from anemia and managing crises (C)</p> Signup and view all the answers

    What percentage of fetal hemoglobin is present at birth?

    <p>70% (D)</p> Signup and view all the answers

    Which of the following inhibits hemoglobin-oxygen binding?

    <p>2,3-diphosphoglycerate (D)</p> Signup and view all the answers

    What type of hemoglobin is most prevalent in a 6-month fetus?

    <p>HbF (C)</p> Signup and view all the answers

    Which stage of development sees the emergence of HbA?

    <p>First trimester (C)</p> Signup and view all the answers

    How does fetal hemoglobin facilitate oxygen transport in the uterine environment?

    <p>By increasing its affinity for oxygen. (C)</p> Signup and view all the answers

    At what stage of fetal development can certain hemoglobin disorders be identified?

    <p>First trimester (A)</p> Signup and view all the answers

    What is the primary function of fetal hemoglobin (HbF)?

    <p>To transport oxygen from the mother to the fetus. (A)</p> Signup and view all the answers

    What happens to the percentage of fetal hemoglobin in the blood after birth?

    <p>It decreases. (B)</p> Signup and view all the answers

    What is the primary treatment for Hemolytic Disease of the Newborn (HDFN) caused by Rh incompatibility when Rh immune globulin was not administered?

    <p>Exchange transfusions with Rh-positive blood (B)</p> Signup and view all the answers

    What is the main reason for administering exchange transfusions within the first 24 hours of life for HDFN?

    <p>To prevent the development of kernicterus (B)</p> Signup and view all the answers

    How does phototherapy help reduce the toxic effects of unconjugated bilirubin in HDFN?

    <p>It converts unconjugated bilirubin into a conjugated form that can be excreted (A)</p> Signup and view all the answers

    What is the wavelength range of high-intensity light used in phototherapy to treat HDFN?

    <p>460-490 nm (A)</p> Signup and view all the answers

    What is the primary reason for the occurrence of hemolytic anemia in individuals with G6PD deficiency?

    <p>Premature destruction of red blood cells (B)</p> Signup and view all the answers

    Which genetic inheritance pattern is associated with G6PD deficiency?

    <p>X-linked recessive inheritance (A)</p> Signup and view all the answers

    The prevalence of G6PD deficiency is highest in which population?

    <p>Middle Eastern populations (D)</p> Signup and view all the answers

    What is the primary mechanism by which phototherapy contributes to the breakdown of unconjugated bilirubin?

    <p>Photoisomerization resulting in bilirubin conjugation (C)</p> Signup and view all the answers

    Flashcards

    Antibody-mediated hemorrhagic diseases

    Diseases characterized by bleeding due to immune-mediated platelet destruction, including thrombocytopenia and purpuras.

    Hematopoietic marrow

    Bone marrow responsible for producing blood cells, which gradually transitions to fatty marrow with age.

    Erythropoiesis

    The production of red blood cells; starts early in the yolk sac of embryos and later moves to the marrow.

    Neonatal thrombocytopenias

    Low platelet counts in newborns, potentially caused by autoimmune processes.

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    Hemolysis

    The destruction of red blood cells leading to increased erythrocyte production due to elevated erythropoietin.

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    Fetal Hemoglobin (HbF)

    A type of hemoglobin in fetuses that has a higher affinity for oxygen than adult hemoglobin.

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    Adult Hemoglobin (HbA)

    The main type of hemoglobin in adults that carries oxygen through the bloodstream.

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    2,3-Diphosphoglycerate (2,3-DPG)

    An enzyme that reduces hemoglobin's affinity for oxygen, affecting oxygen binding.

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    Oxygen transport in pregnancy

    Fetal hemoglobin enables efficient oxygen transport from mother to fetus despite low uterine oxygen levels.

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    Embryonic hemoglobin

    Type of hemoglobin found in embryos, mainly before fetal hemoglobin develops.

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    Identification of hemoglobin disorders

    Disorders like sickle cell anemia and thalassemia can be detected early in gestation via hemoglobin type analysis.

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    Composition of neonatal hemoglobin

    At birth, neonatal hemoglobin is approximately 70% HbF, 29% HbA, and 1% HbA2.

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    Decline of HbF after birth

    After birth, the percentage of fetal hemoglobin decreases as adult hemoglobin increases.

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    Rh-negative mother

    A woman without Rh antigen on her red blood cells.

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    Rh-positive fetus

    A fetus with Rh antigen on its erythrocytes.

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    Sensitization

    The process where the Rh-negative mother produces antibodies after first exposure.

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    Hemolytic Disease of the Fetus and Newborn (HDFN)

    A condition where maternal antibodies attack fetal red blood cells.

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    First pregnancy

    Typically uneventful for an Rh-negative mother with an Rh-positive fetus.

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    Placental tear

    A disruption in the placenta that lets fetal blood enter maternal circulation.

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    Antibody production

    The immune response of the mother synthesizing antibodies against Rh antigen.

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    Hydrops fetalis

    A severe fetal condition characterized by gross edema due to anemia.

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    Iron Deficiency Anemia (IDA)

    A common nutritional disorder causing inadequate hemoglobin synthesis, especially in children.

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    Lymphocyte Changes

    Alterations in lymphocyte counts due to infections or immunizations in children.

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    Neutrophil Count

    White blood cell count that peaks shortly after birth and declines over the first few days.

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    Eosinophil Count

    Count of a type of white blood cell that is elevated in the first year of life.

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    Monocyte Count

    Type of white blood cell count that remains elevated through early childhood.

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    Causes of IDA

    Iron deficiency anemia can arise from dietary insufficiencies, absorption problems, blood loss, and increased iron needs.

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    Prevalence of IDA

    Iron deficiency anemia is most common between 6 months and 2 years of age globally.

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    Socioeconomic Factors in IDA

    Factors affecting nutrition that lead to higher rates of iron deficiency anemia in certain populations.

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    HDFN

    Hemolytic Disease of the Newborn; occurs due to Rh incompatibility.

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    Exchange transfusion

    Medical procedure replacing infant's blood to treat HDFN.

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    Kernicterus

    Brain damage caused by high levels of bilirubin in newborns.

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    Phototherapy

    Treatment using light to reduce bilirubin in infants.

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    Unconjugated bilirubin

    Toxic form of bilirubin that can accumulate in the skin.

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    Conjugated bilirubin

    Processed form of bilirubin that is excreted safely.

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    G6PD deficiency

    Genetic enzyme deficiency that can cause hemolytic anemia.

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    Bilirubin phototransformation

    Process where light converts toxic bilirubin into a safer form.

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    Sequestration crisis

    A condition where sickle red cells pool in the spleen, leading to splenic enlargement and hypovolemia.

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    Hyperhemolytic crisis

    An accelerated destruction of red blood cells leading to anemia, jaundice, and reticulocytosis.

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    Infection risk with SCA

    Children with Sickle Cell Anemia are at high risk for infections, especially without proper splenic function.

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    Pneumococcus pneumoniae

    A bacterium that causes severe infections in those with impaired splenic function.

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    Splenic function in children

    Impaired splenic function leads to a higher risk of infections and complications in children with SCA.

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    Acute chest syndrome

    A serious complication characterized by hypoxia and anemia, requiring prompt diagnosis and treatment.

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    Management of fever in SCD

    Aggressive management of fever helps reduce morbidity and mortality in individuals with SCD.

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    Prophylactic antibiotics

    Preventive use of antibiotics like penicillin to protect against infections in patients with SCD.

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    Study Notes

    Fetal and Neonatal Hematopoiesis

    • Erythrocyte production begins in the yolk sac, transitioning to the liver sinusoids shortly after 2 weeks of gestation.
    • Leukocyte and platelet production also starts in the liver and spleen around this time, peaking at roughly 4 months.
    • Bone marrow hematopoiesis commences around the fifth month, increasing until birth when it's the primary site.

    Postnatal Changes in the Blood

    • Blood cell counts initially rise above adult levels immediately after birth, due to accelerated fetal hematopoiesis, birth trauma, and cord clamping.
    • These elevated values are accompanied by immature erythrocytes and leukocytes (particularly granulocytes).
    • Immature blood cells gradually decrease over the first 2-3 months of life.
    • Average blood volume in term neonates is about 85 mL/kg, while premature infants have slightly higher volumes (90-100 mL/kg).
    • Blood volume relative to body weight decreases during the first few months, stabilizing around 75-77 mL/kg by three years of age.

    Erythrocytes

    • Fetal erythropoiesis is triggered by the hypoxic uterine environment, leading to polycythemia in newborns.
    • Postnatal oxygenation of the lungs causes a decline in erythropoietin levels and blood cell formation.
    • Reticulocytes (immature erythrocytes) are prominent in full-term newborns, declining rapidly within the first few days of life.
    • Normal erythrocyte lifespan is 60-80 days in full-term infants, potentially shorter (20-30 days) in premature infants, and 120 days in children and adults.

    Leukocytes and Platelets

    • Neonatal lymphocytes have more cytoplasm and less compact nuclear chromatin.
    • Lymphocyte counts are generally high at birth, rising in healthy infants during the first year, only to gradually decrease into later childhood and adolescence.
    • Neutrophil counts peak in the first 6-12 hours after birth, subsequently decreasing to typical adult ranges by approximately 4 years of age.
    • Eosinophil counts are elevated in the first year of life.
    • Monocyte counts are elevated through preschool age, with values generally declining to adult levels by later childhood.

    Disorders of Erythrocytes

    • Anemia is the most common blood disorder in children, caused by ineffective erythropoiesis or premature erythrocyte destruction.
    • Iron deficiency is a frequent cause of insufficient erythropoiesis.
    • Hemolytic diseases can be acquired (e.g., infections, toxins) or inherited (e.g., G6PD deficiency, hereditary spherocytosis, sickle cell disease, thalassemias).
    • Hemolytic disease of the fetus and newborn (HDFN) is an alloimmune disorder caused by maternal-fetal blood incompatibility.

    Disorders of Coagulation and Platelets

    • Hemophilia A and B are inherited X-linked recessive disorders, involving factor VIII and IX deficiencies, respectively.
    • von Willebrand disease is an autosomal dominant disorder affecting von Willebrand factor, causing insufficient factor VIII levels and prolonged bleeding.
    • Inherited thrombophilias (protein C, S deficiencies, and antithrombin III deficiency) lead to an increased risk of thrombosis.
    • Antibody-mediated hemorrhagic diseases (e.g., immune thrombocytopenia, autoimmune neonatal thrombocytopenias) involve antibody-mediated platelet destruction.

    Neoplastic Disorders

    • Leukemia is the most common childhood malignancy, often affecting blood-forming tissues and producing abnormal white blood cells.
    • Acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) are the most common types.
    • Risk factors for leukemia can include genetic predisposition, environmental factors (e.g., radiation exposures, exposure to chemicals) and prior treatment with chemotherapy.
    • Lymphomas, including Hodgkin lymphoma and non-Hodgkin lymphoma (NHL), affect lymphoid tissue, and are relatively rare in infants.

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    McCance 31. PDF Alterations of Hematologic Function in Children

    Description

    This quiz explores key concepts related to hematopoiesis and erythrocyte production in human development. It delves into the stages of blood cell formation, common blood disorders like Iron Deficiency Anemia (IDA), and variations in blood cell counts throughout different age groups. Test your knowledge on these vital aspects of hematology.

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