Cumulative From Exam (I-II-III) Flashcards
What is the disorder when an enzyme is missing in heme synthesis?
Multi fill in the blank - Aplastic anemia+ hemolytic anemia (lab findings)
Deficiency disorder hemostasis RBC+WBC
Propitious anemia B12 , Intrinsic factor
APTT - monitors heparin
Alternative anti factor Xa - Assay
PT - Monitors Warfarin/Coumadin
Withholds vitamin K form prothrombin proteins
average blood volume
4-6 L in adult
blood pH
7.35-7.45
blood specific gravity
1.049-1.065
blood composition
55% plasma/serum (fluid)
45% formed elements (cells)
Serum
NO anticoagulant used, some clotting factors consumed
In serum, there are proteins …
55% are albumin
WBC count
4.8-10.8x10^3
Major blood functions
Transport oxygen to cells
Transport carbon dioxide and wastes away
Provide defense
Regulate body pH , body temp, fluid balance
homeostasis
maintaining a constant environment equilibrium
(major blood function)
Platelets (thrombocyte)
Size: 1-4 um
No nucleus
Life span: 10 days
Function: Hemostasis –> blood clots, and arrested bleeding
Neutrophils
Inflammation and phagocytosis - 1st line of defense against infections.
2-5 lobes connected by thin filament, segmentation allows passing through openings between lining cells
life span: 6-10hrs
Eosinophil
Defense in parasitic and fungal infections.
Secondary granules (red/orange)
Basophil
Mediates allergic reactions
smallest WBC in circulation
histamine
vasoconstriction
heparin
blood thinner
lymphocyte functions
Immune response and viral infections
varies in size and shape
Monocytes function
Phagocytosis
Nucleus: horseshoe, lima bean, convoluted
Cytoplasm: Ground glass - cloudy
mature RBC - Erythrocyte
Major function: Oxygen transport from lungs to tissue
6-8um Biconcave disc –> max SA for gaseous exchange
No nucleus - no mitosis
Hgb is 95% dry weight
120 days life span.
Organs within Hematopoietic system
BM
Liver
Spleen
Lymph node
Thymus
Embryo
Mesoblastic phase
Fetal
Hepatic phase
Birth
Meduallry phase
Embry- Mesoblastic phase
2 weeks - 2 months gestation
Yolk sak - mesoderm layer
Hgb Formed
- Hgb Gower 1
- Gower 2
- Hgb Portland
Embryonyc Hgb^
Fetus - hepatic phase
2 months - 7 months
Liver and spleen
Hgb Formed:
- Fetal hemoglobin
- Hemoglobin A1 (small amount)
Birth- medullary phase
7 months - rest of life!
BM = intramedullary hematopoiesis - red marrow
Hgb Formed
- Hemoglobin A1
- Hemoglobin A2
Infancy and childhood
Volume and red marrow in infacts = red marrow in adults (distal long bones and axial skeleton)
at 4 years, yellow marrow, starts to replace red marrow –> limits sites of hematopoisis
active hematopoietic sites in axial skeleton
Sternum
Ribs
Pelvis
Vertebrae
Skull (after 40, no more skull)
Pelvis and sternum are
preferred bone marrow aspiration sites –> last place to be replaced by yellow marrow
Blood cell development theory
Monophyletic –> All blood cells arise from one precursor cell that is multipotential or pluripitenital and called stem cell
pluripotential/multipotential
can develop into any cell line in the body
progenitor cell
committed to cell line
cytokines
soluble messages to tell early cells to differentiate
Thrombopoietin
stimulates CFU-Meg, and causes release of platelets
Erythropoitetin
Stimulates CFU-E, and regulates erythroid progenitor cells to mature.
Glycorpotein prodcued by the peritubular interstitial cell in juxtaglomerular apparatus (kidney tubulus)
T cell
Cytotoxic, suppressing actvites
B cell
antibody production, can futher develop to plasma cell and secrete antibodies
CFU-GEMM
gives rise to a serius of committed cells (MYELOID)
BM
Myeloid: Eryrhoid ratio
2:1 - 5:1
Eythrpid maturation
Large –> small/pyknotic (condensation and shrinkage through degeneration)
Euchromatin –> heterochromatin –> Nucleus is extruded
Cell development: Asynchrony
Megaloblastic anemia
Basophilic Normoblast/prorubricyte
Chromatin: Some granularity
Cytoplasm: Slighlty less basophilic “Cornflower blue”
Polychromatophilic normoblast
Chromatin moderately compacted, condensed, “soccer ball”
Orhtochromatic normoblast (nRBC)
Last nucleated stage
Chromatin: completeley pyknotic
Polychromatiophilic erythrocyte
NO NUCLEUS
polychromatophilia
Wrights stain
polychromatiphilic erythrocyte
new methylene blue (vital stain)
reticulocyte
mature erythrocyte
central pallor
no longer able to synthesize hemoglobin
RBC membrane
selective barrier
Water and anions - passive diffusion
cations and others - active transport
potassium
found inside RBC 25:1
sodium is found
outside RBC 1:12
Outer hydrophilic portion of RBC membrane
Containts glycolipids, glycoproteins, proteins
central hydrophobic layer
contains proteins, cholesterol, phosphilipids
inner hydrophilic layer
protein
cholesterol
Central hydrophobic layer
- Target cells
- Acanthocytes
Choline phosphilipid
phosphatidyl choline and sphingomylein
outer half
amino phospholipids
phosphatidylethanolamine and phosphatidyl serine
inside
if outside, can initaite clotting
integral protein example
glycophorin
peripheral membrane pritein
spectrin, beneath lipid bilayer, forms cytoskeleton
Glycophorin
Principle RBC integral membrane protein
sialic acid
spectrin
most abundant peripheral membrane protein
RBC deformability
RBC survival through microvasculature
Oxygen deliver function
hgb infant
14-22g/dl
hgb male
14-18g/dl
hgb female
12-16g/dl
Hemoglobin =
4 heme rings
4 iron
4 globin chains
protophoryns
precursor of heme
1 heme ring =
1 porphoryin = 4 pyrrole rings
major rate limiting step in heme syntehsis
ALA synthetase
HgbA1
2a + 2 b
Hgb A2
2a+2d
HgbF
2a+2y
methemoglobin
Iron is oxidied to ferric state (Fe3+)
carboxyhemoglobin
Oxygen is replaced by CO - increased binding
sulfhemoglobin
sulfur incorporated into heme structure
irreversible
90% of RBC energy comes from
non-oxidative pathways
RES
Removes RBC from circulation
- spleen
spleen
primary site of phagocytosis
home to littoral cells
Extravascular hemolysis
Heme –> biliverdin –> unconjugated bilirubin –> conjugated bilirubin –> urobilinogen
Urobilinogen eliminated in stool
Hgb released in blood vessel
Circulates - Hemoglobinemia
- Filtered at the kidney = hemoglobinuria
Carried by haptoglobin to liver
if BM cannot keep up
Extramedullary hematopoiesis
renal failure patients
lack erythropoietic response to hypoxia
female RBC
4.2-5.4 x 10^6 ul
Male Rbc
4.7-6.1x10^6 ul
HCT
Proportion of red blood cells in whole blood
MCV
80-100fl
MCH
average Hgb mass/weight
27-31pg
MCHC
32-36 %
Average Hgb Concentration
neutrophil %
50-70%
lymphocyte %
20-44%
monocyte %
2-9%
band neutrophil
2-6%
eosinophil
0-4%
basophil
0-2%