Hematopoiesis Formation and Development

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What is the result of mutations in the genes for the hemoglobin protein?

Hemoglobinopathies

What is the normal range of hematocrit value for males?

37-49%

What is the function of Mean Corpuscular Volume (MCV)?

To measure the average size or volume of the RBC

What is the normal lifespan of RBCs?

<p>100-120 days</p> Signup and view all the answers

What is the characteristic of reticulocytes?

<p>No nucleus, reticulum of RNA, and light blue staining</p> Signup and view all the answers

What is the normal range of MCV?

<p>70-100 fl</p> Signup and view all the answers

What is the formula for calculating MCV?

<p>Hct X 10/RBC Count in microliter</p> Signup and view all the answers

What does MCH indicate?

<p>The weight of Hb in the average red cell</p> Signup and view all the answers

What does MCHC measure?

<p>The concentration of Hb in the average red cell</p> Signup and view all the answers

What is the normal value of MCHC?

<p>32-36%</p> Signup and view all the answers

Where does hematopoiesis occur in the third stage of intrauterine development?

<p>Bone marrow</p> Signup and view all the answers

What is the main component of the hemoglobin molecule?

<p>Polypeptide chains</p> Signup and view all the answers

What is anemia defined as?

<p>A reduction in red blood cell mass or blood hemoglobin concentration</p> Signup and view all the answers

During childhood, what replaces hematopoietic tissues in the long bones?

<p>Fatty tissues</p> Signup and view all the answers

What is the manifestation of anemia attributed to?

<p>Tissue hypoxia</p> Signup and view all the answers

How much oxygen binds to 1 gm of Hb?

<p>1.39 ml O2</p> Signup and view all the answers

What is the predominant type of hemoglobin in the fetus?

<p>Hemoglobin F</p> Signup and view all the answers

What is one of the compensatory mechanisms to increase oxygen delivery?

<p>Increase in blood flow</p> Signup and view all the answers

Where is red marrow concentrated in older children and adults?

<p>Skull, vertebrae, pelvis, ribs, clavicles, sternum, and scapula</p> Signup and view all the answers

What is the percentage of Hemoglobin A at birth?

<p>30%</p> Signup and view all the answers

What happens when the Hb level is less than 7 gm/dL?

<p>Clinical findings of tachycardia and increased capillary pulsation</p> Signup and view all the answers

When does the δ chain synthesis begin?

<p>Late in the third trimester</p> Signup and view all the answers

What stimulates the differentiation and clonal maturation of erythroid precursors into mature erythrocytes?

<p>Erythropoietin</p> Signup and view all the answers

What is the percentage of Hemoglobin F at 6-12 months of age?

<p>Less than 2%</p> Signup and view all the answers

What is the main function of vitamin C in iron absorption?

<p>Converting ferric to ferrous iron</p> Signup and view all the answers

Where is iron mainly absorbed in the body?

<p>Duodenum and proximal jejunum</p> Signup and view all the answers

What increases iron absorption in the body?

<p>Both A and B</p> Signup and view all the answers

What is a common manifestation of iron deficiency anemia?

<p>Pallor</p> Signup and view all the answers

What diet can decrease iron absorption?

<p>Rich in phosphorus</p> Signup and view all the answers

What is a risk factor for iron deficiency anemia in neonates?

<p>All of the above</p> Signup and view all the answers

What is a potential cause of chronic blood loss in infants and children?

<p>All of the above</p> Signup and view all the answers

What is a potential symptom of iron deficiency anemia?

<p>All of the above</p> Signup and view all the answers

What is the daily requirement of iron in mg/day?

<p>8-15</p> Signup and view all the answers

Which type of anemia is characterized by MCV of 100fl and MCHC of 32-34?

<p>Normocytic normochromic</p> Signup and view all the answers

Which of the following is an example of extracorpuscular defect leading to anemia?

<p>Immune mechanism</p> Signup and view all the answers

What is the primary cause of anemia due to inadequate production of RBCs or HB?

<p>Decrease in the number of RBC precursors in the bone marrow</p> Signup and view all the answers

Which of the following is an example of a microcytic hypochromic anemia?

<p>Iron deficiency anemia</p> Signup and view all the answers

What is the cause of anemia in chronic renal disease?

<p>Decrease production despite the normal number of RBC precursors</p> Signup and view all the answers

Which of the following is an example of an intrinsic defect leading to anemia?

<p>Membrane defect</p> Signup and view all the answers

What is the cause of anemia in pancytopenia?

<p>Decrease in the number of RBC precursors in the bone marrow</p> Signup and view all the answers

Study Notes

Hematopoiesis

  • Hematopoiesis is the process of production of all types of blood cells, including formation, development, and differentiation of blood cells
  • Prenatally, hematopoiesis occurs in three stages:
    • Yolk sac hematopoiesis (1st 8 weeks)
    • Visceral hematopoiesis (8th week to 6th month)
    • Myeloid hematopoiesis (6th month onwards)

Hemoglobin

  • Hemoglobin molecule is a tetramer made up of 2 pairs of polypeptide chains
  • Each chain has a heme group
  • Polypeptide chains differ from each other in both the number and sequence of amino acids
  • Synthesis is directed by separate genes

Types of Hemoglobin in Humans

  • In the embryo (4-8 weeks gestation):
    • Gower 1 (ζ2ε2)
    • Gower 2 (α2ε2)
    • Hemoglobin Portland (ζ2γ2)
  • In the fetus:
    • Hemoglobin F (α2γ2) predominates during fetal life
    • Represents 70% of total Hb at birth
    • Decreases to less than 2% after 6-12 months of age
  • In adults:
    • Hemoglobin A (α2β2)
    • Hemoglobin A2 (α2δ2)

Genetics

  • Mutations in the genes for the hemoglobin protein result in hemoglobin variants
  • Some mutant forms of hemoglobin cause a group of hereditary diseases termed hemoglobinopathies

Role in Disease

  • Hemoglobin deficiency can be caused by:
    • Decreased amount of hemoglobin molecules
    • Decreased ability of each molecule to bind oxygen at the same partial pressure of oxygen
  • Hemoglobinopathies may cause both

Blood Indices

  • Normal RBCs:
    • No nucleus
    • Biconcave discs
    • Center 1/3 pallor
    • Pink cytoplasm (Hb filled)
    • Cell size 7-8 µ
    • 100-120 days life span
  • Reticulocyte (young RBCs):
    • No definite nucleus
    • Reticulum of RNA
    • Deep blue staining
    • Light blue cytoplasm
    • Cell size about 10 µ

Hematocrit Value (HCT)

  • Portion of 100 blood composed of erythrocytes
  • Normal males: 37-49%
  • Normal females: 36-46%

Mean Corpuscular Volume (MCV)

  • Reflects the average size or volume of the RBC
  • Expressed in femtoliters (fl)
  • Calculated as: MCV = Volume of packed red cells in 1000 ml blood (Hct X 10) / RBC Count (Red cell count) in million in microliter
  • Normal value: 70-100 fl

Mean Cell Hemoglobin (MCH)

  • Indicates the weight of Hb in the average red cell

Mean Cell Hemoglobin Concentration (MCHC)

  • Indicates the concentration of Hb in the average red cell or the ratio of the weight of Hb to the volume in which it is contained
  • Expressed in percent
  • Normal value: 32-36%

Anemia

  • Defined as a reduction in red blood cell mass or blood hemoglobin concentration either singly or in combination
  • Compensatory mechanisms:
    • Increase in blood flow
    • Increase in red cell mass
    • Increase oxygen unloading

Classification of Anemia

  • Etiological classification:
    • A- Inadequate production of RBCs or HB
    • B- Anemia due to excessive destruction of RBCs
    • C- Anemia due to excessive blood loss
  • Morphological classification:
    • A- Normocytic normochromic
    • B- Microcytic hypochromic
    • C- Macrocytic normochromic anemia

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