Class 1 Red Blood Cells (RBCs)
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Dr. Laxmikanta Say
ErythrocytesRed Blood Corpuscle
{RBC}
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Definition - erythrocyte Greek “erythros” means “red” “kytos” for “hollow” “cyte” translated as "cell" in modern usage.
Circular, biconcave, non-nucleated disc.
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Non-nucleated formed elements of blood which plays a vital role in transport of respiratory gases.
Colour – red, presence of pigment hemoglobin.
Normal count – 4.5-5.5m/cumm of blood
Size-7.2micron,Thickness-2.2 & 1micron.Shape-Biconcave disc (Dumbbell).Life span – 120days.Site of destruction-Reticulo Endothilial System.
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Advantage of biconcave disci. Flexibility to RBC - easy folding of RBCs - squeeze through narrow
capillaries easily
ii. Allows alteration of cell volume - can withstand change in osmotic pressure - resist hemolysis
iii. Greater surface area - exchange of O2 , CO2 - diffusion of other substances
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Actin, Spectrin - a contractile protein - arranged in fibrillar manner - maintains shape & flexibility of RBC membrane - specific blood group substance - “antigen”
Lack of spectrin - Hereditary Spherocytosis. - Fragile in hypotonic solutions.
How the shape is maintained ?
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Composition62.5% - Water35% - Haemoglobin (29.5 ± 2.5 pg/RBC)2.5% a. Sugar – Glucose b. Lipids – Cephalin, Cholesterol & Lecithin c. Protein – Glutathion - a insoluble protein - acts as reducing agent - prevents damage of haemoglobin d. Enzymes of Glycolytic system; Carbonic anhydrase & Catalase e. Ions – Na+, K+, Ca2+ , PO43-, SO42-
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Dimensions o RBC
Diameter –6.5 – 8.8 µm (7.4 µm)
Surface area- 140 µm2
Volume - 78 – 94 µm3 or fL(86± 8 µm3)
Normal Count:
At Birth; 6-7 million/µLMale – 5 -6 million/ µLFemale – 4.5 -5.5 million/ µLClinically 5 million/ µL taken as 100% RBC
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Functions of RBCsTransport of oxygen from lungs to tissue. 97%-Oxyhemoglobin.
Transport of carbon dioxide from tissue to lungs.(33%).
Carbonic anhydrase forms bicarbonate from carbon dioxide and water. (67%).
Buffering action. Regulates acid base.
Blood group determination. ABO and Rh antigens.
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Variations
Physiological cause of increase count.AgeSexHigh altitudeExercise (spleen)Emotion TemperatureMeal.Decrease-High atmospheric pressure in deep sea, pregnancy & sleep.
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PathologicalINCREASE- POLYCYTHAEMIA. Primary, secondary, relative.
DECREASE- ANAEMIA. 1.Blood loss. 2.Deficiency. 3.Haemolytic. 4.Aplastic
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PolycythaemiaPHYSIOLOGICAL -Age, Sex, High altitude, Exercise, Anxiety, Environmental temperature.
PATHOLOGICALi. Primary Polycythaemia - Polycythaemia vera - Erythremia - Malignancy of red bone marrow.ii. Secondary Polycythaemia - Chronic hypoxia - Emphysema, - Congenital heart diseaseiii. Relative Polycythaemia – Polycythaemia due to hemoconcentration - Burn, Dehydration.
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Variations in Shape, Size & StructureSize - Microcyte, Macrocyte, Anisocyte.
Shape - Crenation, Spherocytosis, Sickle cell, Elliptocytosis, Poikilocytosis.
Structural change -Punctate basophilia – lead poisoning
Ring in R.B.C., Howell-Jolly bodies.
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Haemoglobin
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DefinitionoHaem & Globin
o It is red, oxygen carrying pigment found in the RBCs of vertebrates.
Molecular weight 64,450 dalton
Hemoprotein , accounting 90% of dry weight of mature cell.
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o70 kg man contains 750gms of Hb.
oApprox. 6.25 gm(90mg/kg) of Hb are produced & destroyed in the body each day.
Globin makes it ‘species-specific’.
Globin of adult Hb contains high content of “Histidine” & “Lysin” & a small amount of “Isoleucine” .
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Synthesis of haem occurs in Mitochondia.
Iron-protoporphyrin-IX
Other oxygen supplying Haemoproteins -Neuroglobin found inside brain-Myoglobin found in heart & skeletal
muscle, containing single polypeptide chain with heme moiety.
-Cytochrome-C seen in respiratory chain
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HEMOGLOBIN STRUCTURE
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Structure
Methine(=CH--) bridges , , , Side chain 1,3,5,8 Methyl(-CH) 2,4-Vinyl(-CH=CH2) 6,7-Propionic acid (-CH.CH2.COOH)
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Formation of Hemoglobin
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GlobinSynthesized from free ribosome.
Normal adult hemoglobin contains two identical α-chain & two identical β-chain.
Arranged in a configuration of “tetrahedron”.
Each polypeptide chain contains a heme, called “heme-pocket”. Thus one Hb. molecule contains four heme units.
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-GLOBIN GENE FAMILY-141 amino acids-Chromosome no – 16- & (zeta)- embryonic globin-1st 8wks.
-GLOBIN GENE FAMILY-146 amino acids-Chromosome no -11 ( differs by 10 amino acids) (differs by 37 amino acids) (substitution in amino acid glycine or
alanine at codon position)
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Hemoglobin Type
Name Component -
Like subunit
Adult A 2 2
A2 2 2 Fetal F 2 2
Embryonic Portland 2 2
(1ST 8 wks of I.U.life)
Gower 1 2 2
Gower 2 2 2
Abnormal H 4
Bart’s 4
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SOME IMP. DEFINITIONS1.OXYHAEMOGLOBIN-Reacts with oxygen to form oxyhaemoglobin
and represented as HbO2.
Hb4 + O2 Hb4O2
Hb4O2 + O2 Hb4O4
Hb4O6 + O2 Hb4O8
-Affinity depends on pH, temperature and concentration of 2,3, diphospho-glycerate
(2,3 DPG) .HbO2 + 2,3-DPG Hb-2,3-DPG + O2
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2.CARBAMINO-HAEMOGLOBIN-forms carbamino-haemoglobin CO2 + HbNH2 HbNH COOH
3.DEOXYGENATED HAEMOGLOBIN(REDUCED)
4.CARBOXY HAEMOGLOBIN or CARBON MONOXY HAEMOGLOBIN
-carbon monoxide react with haemoglobin.-it has >210 times affinity than oxygen.
5.METHAEMOGLOBIN-When reduced or oxygenated Hb exposed to various
drugs or oxidising agents, the ferrous (Fe++) is oxidised to ferric (Fe+++) and the compound is called methaemoglobin (HbOH).
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Functions of Haemoglobin1. Respiratory:- Facilitate transport of oxygen from lungs to tissues.- Facilitates transport of CO2 from the tissues to lungs.2.Nutritive:-Conveys absorbed food materials, glucose, amino
acids, fatty acids, vitamins, electrolyte and trace metals.
3.Excretory: Transports metabolic wastes.4.Homeostasis for water, pH, and electrolyte
concentration: Excellent Buffering actions5.Regulation of body temperature6.Chemical for communication and protection7. Blood group determination. ABO and Rh
antigens.
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CATABOLISM / FATE OF HEMOGLOBIN
RBCs are destroyed in “tissue-macrophage system” also known as “reticulo-endothelial system”.
-Kupffer cell of liver-Alveolar macrophages in Lungs-Osteoclasts in bone-Microglia in brain-Lymph nodes-Spleen-Littoral cells lining the blood sinuses in bone
marrow.
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Types of Haemoglobin
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Adult Haemoglobin
Age Amount of HbA
At 20 wks of intra uterine life
6% (rest Hb-F)
At Birth 20%
At 2 months(post natal)
50%
At 4 months(post natal)
90%
More than 1 year >99% (<1% Hb-F)
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Glycosylated HaemoglobinHb-A1C
Normal conc. 3-6 %
Addition of sugar moiety to valine.
1-amino-1-deoxy fructose to -NH2 terminal of valine of β-chain of Hb-A1.
e.g, Diabetes mellitus (7.5--15%)
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Foetal HaemoglobinDesignated as Hb - 22. β- Chain replaced by - chain (differs by 37 amino –acids)
Greater affinity for oxygen.Life span is less - 80 days.Hb-F predominates after birth to 1 year.(80%)
Persistence of Hb-F beyond 4-6 months, suspicious of disorder synthesis of Hb-A .(Thalassaemia)
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APPLIED / HAEMOGLOBINOPATHIES
Hemoglobins with altered amino acid sequences that result in deranged function or altered physical or chemical properties.
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CLASSIFICATION OF
HEMOGLOBINOPATHIES
1) STRUCTURAL GENE MUTATIONReplacement of a single amino acid
residue of HbA1e.g. Hb-S, Hb-M, Hb-C, Hb-D(Panjab)
2) REGULATOR GENE MUTATIONRate of synthesis of peptide chains , the
amino acid sequence remaining unaffected
e.g. - thalassemia, ß –thalassemia, ß, ß etc.
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HAEMOGLOBIN-S Substitution of valine or lysine
residue for glutamic acid at position 6 in the ß-subunit of hemoglobin.
A point mutation.Polymerization of deoxy-Hb-S, forming
long fibrous precipitates and distorted to sickle shaped.
Sickle cells are more fragile .Vascular stasis and anoxic damage to
tissues.Protection from malaria.Increased incidence to Salmonella
infections.
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PATHOPHYSIOLOGY OF SICKLE CELL ANAEMIA
SCAN
Pathogenesis of sickle cell disease
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THALASSAEMIA
Heterogenous group of inherited disorders.
Genetic lesions leads to decreased synthesis of either alpha or beta - globin chain of HbA.
Types- -thalassaemia & -thalassaemia
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-THALASSAEMIA
Synthesis of -chain repressed and synthesis of and -chain increased.
Hb-H (4)Hb-Barts (4)- Hydropes foetalis
Hb-Portland (2 2) - Hydropes foetalis
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Hemoglobin H Disease
3 alpha-globin gene deletion(4) - most common among Asians - amount 10-20% - extremely high affinity for oxygen, - tissue hypoxia, - prone to oxidation, - precipitates of oxidised HbH form - moderately degree of haemolytic
anaemia
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Hydrops Fetalis
all 4 alpha-globin gene deletionIn fetus excess gamma globin chains
form tetramers (Hb Barts - 4)very high affinity for oxygenNo oxygen delivery to tissuesFetal distress by 3rd trimester,IUD
resultsFetus – pallor, generalized edema,
massive hepatosplenomegalyIntra-uterine transfusion can save such
infants
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-THALASSAEMIASynthesis of -chain repressed. Excess of -chains with -chains
produce Hb-A2 or with -chains produce Hb-F.
Severe anaemia of infancy or early childhood called as Cooley’s anaemia.
Mongoloid features & stunted growth.Icterus, SpenomegalyHypochromasia, polychromasia,
basophilic stipling,target cells++, nucleated cells.
X-ray – Hair-on-end appearance.
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MISCELLANEOUS HbHb SHb CHb EHb D-PanjabHb G-PhiladelphiaHb HasharonHb IHb JHb Korel -BuHb LeporeHb MHb O-ArabHb Kansas
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Thank You For Your Attention!