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93 Cards in this Set

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  • Back
What percentage of blood is plasma?
60%
What is the major component of plasma? What are the other components?
Water…Electrolytes and proteins
What are the proteins in plasma?
Hemostatic…Complement…Immunoglobulins…Transport
What are the three major types of blood cells?
Erythrocytes…Leukocytes…Thrombocytes
What are the thre categories of white blood cells (leukocytes)?
Granulocytes…Lymphocytes…Monocytes
What are the three types of granulocytes?
Neutrophils…Esoinophils…Basophils
What is the purpose of neutrophils?
First line of defense in the inflammatory process.
What is the purpose of esoinphils?
Attack parasitic worms.
What is the purpose of basophils?
Contain amines such as histamine. Involved in infection and allergies.
What are the two types of lymphocytes? Describe them.
T-lymphocytes: cell-mediated immunity; produced by the thymus…B-lymphocytes: humoral immunity; produce antibodies.
What is the role of monocytes? Where do they do their job?
Matures into macrophages that remove debris, present antigens to T-cells. They circulate and then move into the tissue to work.
How are platelets produced?
The peripheral cytoplasm of megakaryocytes in bone marrow is fragmented.
What are the functions of blood?
Respiratory gas exchange…Transport of nutrients and waste…Thermoregulation…Hemostasis
During embryogenesis, where are blood cells produced? Where does it occur after embryogenesis?
Yolk sac mesoderm, liver, and spleen. Bone marrow.
Where does hematopoiesis occur in infants to 4 year olds?
In all marrow cavities.
Where does hematopoiesis occur after 4 years of age?
In parts of the marrow cavity; the marrow outgrows the need.
From what cells are all blood cells derived?
The "founder cell" is a pluripotent stem cell that divides indefinitely and can differentiate into daughter cells.
What are the two stem cells that divide from the "founder cell"? What are they referred to as?
Lymphoid stem cell…Myeloid stem cell…Multipotent stem cells.
What are the two types of growth factors that influence hematopoiesis? What is the difference between the two?
Non-lineage specific growth factors: act on pluripotent and multipotent stem cells; interleukin-3, granulocyte-monocyte colony stimulating factor (GM-CSF)…Lineage specific growth factors: erythropoietin, interleukin-5.
What is the difference between aspiration and biopsy?
Aspiration: a needle is inserted and a liquid sample is removed…Biopsy: a core of bone marrow is removed and examined.
How long do erythrocytes live?
Approximately 120 days.
What is unique about the color of erythrocytes?
1/3 of the diameter will have a palor.
What organ responds to low blood oxygen levels? How so?
The kidneys detect the condition and release erythropoetin into the blood.
What is the role of erythropoetin?
It travels to the bone marrow and stimulates an increase in the production of RBCs.
What organ destroys damaged or old RBCs?
The spleen.
What are the three required hematopoetic growth factors?
Interleukin-3 (IL-3)…granulocyte-monocyte colony stimulating factor (GM-CSF)…Erythropoetin (EPO).
What are the growth factors that are important to the early stages of stem cell differentation?
IL-3 and GM-CSF
What is hematocrit?
The amount of packed red blood cells in a sample of blood; a percentage.
What is MCV?
Mean Corpuscular Volume…The volume of an average RBC; measured in femtoliters (fL).
What is MCH?
Mean Corpuscular Hemoglobin…The average amount of hemoglobin in an RBC; measured in picograms (pg).
What is MCHC?
Mean Corpuscular Hemoglobin Concentration…The average amount of hemoglobin as a percentage of the volume of the RBC; measured in g/dL…Calculated using MCH/MCV x 100.
What are the three morphological classifications of anemias?
Normocytic…Microcytic…Macrocytic…Measurement based on MCV and MCHC.
What is the iron carying part of the RBC called? What type of molecule is this?
Heme…It is a porphyrin.
What cyclic tetrapole is vital to DNA synthesis?
Vitamin B12 and cobalt.
What cyclic tetrapole is vital to photosynthesis?
Chlorophyll and magnesium.
What cyclic tetrapole is vital to oxygen transport in humans?
Heme and ferrous iron (Fe2+)
What is the composition of heme?
Four pyrroles linked by methylene bridges to a central ferrous iron.
What enzyme adds Fe to produce hemoglobin?
Protoporphyrin IX
What is the role of myoglobin?
It is an oxygen storage molecule.
Who recognized air as a requirement for life? When?
Boyle…1660
Who identified oxygen as the vital component of air? When?
Lavoisier…1777
Who name hemoglobin? When?
Hoppe-Seyler…1864
Who discovered the 3D shape of hemoglobin? When?
Perutz…1936-59
What is the general mutation that causes Hb function to diminish?
An internal hydrophobic amino acid is exchanged for a hydrophilic amino acid, attracting water molecules and preventing oxygen from binding.
What is bound to the proximal histadine?
Fe2+ of the heme.
What is bound to the distal histadine?
Oxygen binds between this histadine and the Fe2+
Where are the genes found for hemoglobin?
They are found on chromosomes 11 and 16.
What are the types of hemoglobin and which polypeptide bonds make them up?
HgF (fetal): α2γ2…HgA2 (minor): α2δ2…HgA (major): α2β2
How many amino acids are found in the α chain?
141
How many amino acids are found in the non-α chain?
146
What is the relationship between pH and oxygen binding capacity in heme?
Hb binds oxygen efficiently in a high pH environment and releases it in a low pH environment.
How is oxygen affinity modulated?
2,3-diphosphoglycerate (2,3-DPG) decreases Hb affinity for oxygen.
What is the affinity for oxygen for myoglobin? For hemoglobin?
Myoglobin always has a higher affinity for oxygen than hemoglobin.
What does 2,3-DPG do to Hb? How does it do this?
It decreases it's affinity for oxygen. 2,3-DPG is hugely negative at blood pH and binds to the positive interior of the Hb, preventing oxygen from binding.
What occurs in high altitude to adjust for the lower oxygen pressure?
Ventilation rate increases…RBC production increases…2,3-DPG production increases to further lower the hemoglobin's affinity for oxygen.
What are the differences from HbF and HbA?
HbF has a higher affinity for oxygen and does not bind 2,3-DPG as readily
What are the four stages of iron deficiency?
Depletion…Deficiency without Anemia…Deficiency with Anemia…Advanced Iron Deficient Anemia
What is the most common cause of iron deficiency?
Blood loss
What are the clinical signs of iron deficiency?
Brittle nails, mouth sores, glossitis, pica
What is the treatment for iron deficiency?
Oral ferrous sulfate
What are the major characteristics of megaloblastic anemia? What are the other hematologic presentations?
Low reticulocyte count, Retardation of DNA synthesis…Anemia, Elevated MCV, Hypersegmented neutrophils
What is the activity level of bone marrow in megaloblastic anemia?
Hyperactive
How long does it take for folate to be depleated? B12?
4 months…10 years
What does vitamin B12 do?
It removes a methyl group from THF (tetrahydrofolate), activating the THF. The methyl is transferred to homocysteine.
If B12 is missing, what is the effect?
Myelin synthesis is diminished and neurological effects occur.
What is the treatment for megaloblastic anemia due to folic acid deficiency?
5 mg of folate for 4 months.
What is the treatment for megaloblastic anemia due to vitamin B12 deficiency?
6 doses of 100µg over 2-3 weeks.
What occurs as RBCs age?
Increased regidity, decreased concentration of 2,3-DPG
If the lifespan of RBCs is decreased, what is this called?
Hemolytic disorder
How does the body combat hemolytic disorders? How long can it do this?
It produces more RBCs by activating more bone marrow. Can make up for the extra RBC death until the average lifetime drops from 120 days to 15 days.
What are the hereditary causes of hemolytic anemia?
Red cell membrane defect…Red cell metabolism defect…Abnormal hemoglobin
What are the causes of acquired hemolytic anemia?
Inappropriate immune activation…Drug and chemical ingestion…Vasculitis…Infectious disease…Liver of kidney failure
What are the clinical features of hemolytic anemia?
Jaundice, tea colored urine, gallstones, ankle ulcers
What is produced when RBCs are broken down?
Bilirubin
What is the treatment for hereditary spherocytic anemia?
Splenectomy
How is hereditary spherocytic anemia inherited?
Autosomal dominant
What is seen in the blood of a patient with hereditary spherocytic anemia after a splenectomy?
Howell-Jolly bodies: small DNA fragments
In which population is G6PD highly prevalent?
African American males
Where is G6PD located?
X chromosomes
What is thalassemia?
A disorder characterized by a defective or absent globin chain
What does hypochromic mean?
Decreased hemoglobin
What are the types of thalassemia? Where do they tend to occur?
α-thalassemia: far east
β-thalassemia: Mediterranean Sea
What is the molecular basis of α thalassemia?
Deletions in chromosome 11 and 16
What is the molecular basis of β-thalassemia?
Regulation of gene syntheis or single point mutations.
What are the three types of β-thalassemia?
β-thalassemia major: produces no β-globin chains and no HbA1
β-thalassemia intermedia: some β-globin and HbA1 is produced
β-thalassemia minor
What occurs to the marrow in β-thalassemia major?
Marrow becomes overactive and the skull can become deformed.
What is agglutination? When does it occur?
Since there are no β-chains for the α-chains to bind to, the α-chains do not form tetramers and eventually form Heinz Bodies
What are the classifications of α-tahalassemia?
Silent carrier (1 gene deletion)
α-thalassemia minor (2 gene deletion)…HbH disease (3 gene deletion)…hydrops fetalis (four gene deletion)
What are Hb Barts?
Excess γ-chains build up in fetuses because there are no α-chains for it to bind with.
What is the major cause of mortality in thalassemias? How is it treated?
Iron overload…Low iron diet, Desferal
What is the mutation that causes malaria resistence?
Heterozygous for β globin chain mutation.
What are the three gene mutations that cause sickle cell? What does this cause?
HbS: β6 Glu to Val… HbC β6 Glu to Lys… Chapel Hill: α74 Asp to Gly…Causes a hydrophobic patch to appear that causes them to bind to one another.
What drug based therapy is used for sickle cell?
Induction of HbF formation with hydroxyurea.