Anemia 2011 Student Dental f Copy
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And hold fast, all together, by the rope which God (stretches out foryou), and be not divided among yourselves; and remember with
gratitude God's favour on you; for ye were enemies and He joined
your hearts in love, so that by His Grace, ye became brethren; and
ye were on the brink of the pit of Fire, and He saved you from it.
Thus doth God make His Signs clear to you: That ye may be
guided. [003:103]
Todays Quranic verse
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No one can make you feel inferior
without your consent
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Proerythroblast
(Pronormoblast)
Basophilic
Normoblast
Polychromatophilic
Normoblast
Orthochromatophilic
Normoblast
Reticulocyte
Erythrocyte
Early Intermediate Late
Steps in Erythropoisis
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C.B.C
Haemoglobin - 152.5, 14 2.5 - g/dl
PCV - 0.47 0.07, 0.42 0.05 - l/l (%)
Haematocrit, effective RBC volume better
RBC count - 5.5 1, 4.8 1 x1012/l
MCHC - Hb/PCV - 30-36 - g/dl
Hb synthesis within RBC
MCH - Hb/RBC - 29.5 2.5 pg/l
Average Hb in RBC
MCV - PCV/RBC 85 8 fl
RDW (Red Cell Distribution Width )-13 1.5
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Red Blood Cell Disorders
Anemias
AnemiaofBloodLoss: (Acutevs. Chronic) Anemiaof Erythropoiesis
Hemolytic Anemia: (Congenital/Acquired)
Polycythemia
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Anemias
Hb (g/dL)
Male Female
13-17 12-15
(152) (13.5 1.5)
An (without) -emia (blood):
Reduction below normalinhemoglobinorred bloodcellnumber(forage & sex).
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Anemias
REDISTRIBUTION2,3 DPG
C.O. PLASMA
Anemia
HYPOXIA
COMPENSATORY
MECHANISMS
C.O.PATHOPHYSIOLOGY
OF ANEMAIS
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Anemias TIREDNESSLASSITUDE
EASYFATIGUABILITY
MUSCLEWEAKNESS
CLINICAL FEATURES
GISCNS
CRS GUSPALLOR
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Anemias
Etiological Morphological
ClassificationClassification
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AnemiasETIOLOGIC
Classification
BLOOD LOSS(Haemorrhagic)
IMPAIREDRED CELL
FORMATION(Erythropoiesis)(Dyshaemopoietic)
INCREASED
RED CELLDESTRUCTION(Haemolytic)
I
II III
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Anemias
ETIOLOGICClassification
BLOODLOSS
IMPAIREDRED CELL
FORMATION
HEMOLYTICANEMIAS
I
II III
CHRONICACUTE
DEFICIENCYNON
DEFICIENCY
RBCABNORMALITIES
RBC ENVIRONMENTABNORMALITIES
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DEFICIENCY ANEMIAS
ANEMIAS DUE TO DEFICIENCY OF SUBSTANCESESSENTIAL FOR ERYTHROPOIESIS
IRON
DEFICIENCYANEMAIS
MICROCYTIC HYPOCHROMIC
ANEMIAS
FOLIC ACID/VITAMIN B12
DEFICIENCYMEGALOBLASTIC ANEMAIS
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NONDEFICIENCY ANEMIAS
ANEMIAS NOT DUE TO DEFICIENCY OF SUBSTANCESESSENTIAL FOR ERYTHROPOIESIS
APLASTIC
ANEMIA
ACD
ANEMIA OF
CHRONIC
DISORDERS
ANEMIA
OF
BONE MARROW
INFILTRATION
(myelophthisic)
OTHERS
LEUKEMIAS,LYMPHOMAS
MYELOMA,MYELOFIBROSIS
INFECTION COLLAGENDISEASES
RENALFAILURE, LIVERFAILURE
MALIGNANCY
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Anemias
MORPHOLOGICClassification
IIMICROCYTIC
HYPOCHROMIC
III
MACROCYTICANEMIAS
I
NORMOCYTICNORMOCHROMIC
Iron DeficiencyThalassemias
ACD
Megaloblastic
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Iron Deficiency Anemia
A world-wideproblem
3% oftoddlers age 1-2 years
2-5% ofwomenofchild bearingage
Ironmetabolism
Iron stores
Laboratoryfindings ofirondeficiency
Causes ofirondeficiency
Treatment
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MajorIron Compartments
Metabolic
Hemoglobin 1800-2500 mg
Myoglobin 300-500 mg
Storage
Iron storage 0-1000 mg
Transit
Serumiron 3 mg
Total 3000-4000 mg
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Causes ofIron Deficiency
Increasediron
requirements
Bloodloss
Gastrointestinaltract
Genitourinarytract
Blooddonation Worminfestation
Pregnancyandlactation
Inadequateiron supply Insufficientdietaryiron (malnutrition)
Impairedironabsorption
Gastric surgery
Intestinalmalabsorption
Celiacdisease
Iron deficiency is a symptom, not a disease
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Esophageal webs and strictures
Koilonychia
Systemic Manifestations ofIron
Deficiency
Behavioralandneuropsychiatric
manifestations
Pica (pagophagia)
Angularstomatitis
Glossitis
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Lab Findings
Hb lowMCV low
Serum iron lowTIBC high
Ferritin lowBM iron absent
Peripheral smear: Microcytic hypochromic
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Hypochromic Microcytic RBC
Smear
Microcytic / Hypochromic RBCs,Anisocytosis, Poikilocytosis
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Macrocytic Anaemias
Defition:- Anaemias with MCV >95 fl
Classification:-
A. Megalo-blasticB. Non-megalo-blastic
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Megaloblastic anemia
Vitamin B12/Folicaciddeficiency
Secondmostcommontypeofanemia.
Multi SystemdiseaseAllorgans withincreasedcelldivision.
Macrocyticanemia, pancytopenia.
Pernicious anaemia
autoimmune, Gastricatrophy, VitB12 def.
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Enteric Processing & Absorption
of Cobalamin
Peptic
digestionH+
Cbl + R-binder
R-Cbl
Pancreatic
enzymes
OH-IF + Cb
Cbl-IF
Food-Cbl
IF receptor Cbl + TC
Cbl-TC complex
Stomach
Duodenum
Distal ileum
R-Cbl
Cbl-IF
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Vitamin B12 Deficiency
Mechanisms Malnutrition
Intragastricevents Inadequatedissociationofcobalaminfromfoodprotein
Totalorpartialgastrectomy Absentintrinsicfactorsecretion
Proximal smallintestine Impairedtransferofcobalaminfrom R proteintointrinsicfactor
Usurpationofluminalcobalamin
Bacterialovergrowth Diphylobothrium latum (fishtapeworm)
Distal smallintestine Diseaseoftheterminalileum
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Megaloblastic Anemia-Pathogenesis
Decreased VitB12 / Folate
Decreased DNA Synthesis
Delayed maturation of erythroblasts (Nucleus)
Increased cell size (macrocytes)
Normal Hb content(Normochromia)
Decreased RBC number & Decreased WBC number (pancytopenia)
Anemia & Pancytopenia.
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Pernicious Anemia
Mostcommoncauseofvitamin B12 deficiency
Occurs inallages andethnic backgrounds
Autoimmunedestructionofgastricmucosachronic Atrophicgastritis IF
Associated withotherautoimmunediseases
Screenforthyroiddiseaseevery 1-2 years
Pernicious anemiais a systemicdisease
Gastrointestinaltractinvolvement
Neurologicinvolvement (sub-acutecombineddegenerationofspinalcord)
Anti-intrinsicfactorantibodies (~60% positive) Specific butnot sensitive
Anti-parietalcellantibodies (~90% positive) Sensitive butnot specific
Schillingtest
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Macrocytes & Macropoly & Megaloblast
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Folate Deficiency
Minimumdailyfolaterequirementis 50 g
Usualdietaryfolate 50-500 g
Absorptionin smallintestine
Causes offolatedeficiency
Dietary (90%)
Alcoholabuse
Pregnancy
Malabsorption
Drug-induced
Treatment - oralfolicacid supplementation
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Anemia of Chronic Disease
Associatedconditions Prevalence Infection 20-95%
Viral, bacterial, TB, parasitic, fungal
Autoimmunedisease 8-17% RA, SLE, sarcoidosis, IBD, vasculitis
Cancer 30-77%
Chronic solidorganrejection 8-70%
Characteristics
WBC andplateletcounts arenormal Anemiaofvariable severity (mild-severe)
Low erythropoietinlevel
Low reticulocytecount
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Anemia of Chronic Disease
PathogenesisCytokines
(IL-1, TNF, IL-4)
Disturbedironmetabolism
Diversionofironfromcirculationtothe RE system
Decreased RBC
precursors
InadequateEpo
response
Normch/normocy, Hypoch/microcy, serum iron, /N TIBC, /N S.Ferritin, Storage iron
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Aplastic Anemia
Suppression of multipotentmyeloid stem cells Anemia, thrombocytopenia, neutropenia Etiology: idiopathic (65%), irradiation, myelotoxic drugs, chemicals,
viruses Idiosyncratic reaction: chloramphenicol, sulfonamides
BM: hypocellular with fat replacement
No splenomegaly, if + diagnosis, notAA
No reticulocytosis Treatment: stop drug, BMT(< 40 y/o) for idiopathic,
immunosuppression for older patients, w/o donors
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Aplasticanemia
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HEMOLYTICANEMIA
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Hemolytic Anemia with Extravascular
HemolysisHereditary
Hemoglobinopathies (sicklecellanemia, thalassemia)
Enzymopathies (G6PD deficiency)
Membranedefects (hereditary spherocytosis)
Acquired Immunemediated
Autoimmunehemolyticanemia Nonimmunemediated
Spurcellhemolyticanemia
Extravascular (reticuloendothelial system)
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Sickle Cell Anemia
Mostprevalenthemoglobinopathy caused by mutations ofthe -globin chain sickle Hb (HbS)
8% of American blacks (1:600)
30% of African blacks (protective effectof HbS vs. malaria)
Autosomal recessive
Single AA substitution in the globin chain (val for glu)
Normal adult: 96% HbA, 3% HbA2, 1% HbF
On deoxygenation: HbS polymerize (gelation/crystallization)
Change in physical state cause RBC distortion sickle/crescents
Reversible sickling irreversible sickling despite adequate O2
RBC hemolysis (anemia), capillary stasis (ischemia/thrombosis)
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TargetcellSicklecells
Anisocytosis/poikilocytosis
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Hemolytic Anemia: Sickle Cell Anemia
Clinical: anemia + complications: acutechest syndrome, strokes, acutepaincrisis, priapism, legulcers (vaso-
occlusion),
Susceptibletoinfection: S. pneumoniae, Staphylococcal, Salmonella(osteomyelitis)
Diagnosis:
-Peripheral smear, Lab iron studies & Electrophoresis
Treatment:
Hydroxyurea Increases HbF = retards sickling byinhibitingpolymerformation
RBC transfusions
RBC exchanges duringacutecrises
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Hereditary Spherocytosis
Abnormal RBC skeletal/membrane proteins (ie. Ankyrin)
RBCs with reduced membrane stability lose membranefragments, assume a sphere
Clinical: anemia, splenomegaly, jaundice
Lab: osmotic fragility
Upon exposure to hypotonic saltsolution, spheroidal shapelimits volume of expansion.
RBCs shortened lifespan
Treatment: splenectomy to alleviate anemia, continue to havespherocytes
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Hereditary Spherocytosis
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Glucose-6-Phosphate Dehydrogenase
Deficien
cy
G-6-PD essentialformaintainingglutathioneinactiveform Detoxifies freeradicals andperoxides
Sex-linkeddisorder Effects > 200 millionpeople
Hemolyticanemiaoccurs inthepresenceofstress (infectionordrugs- antimalarial, sulphonamide, nitrofurantoinetc)
Africanform - mildhemolysis
Mediterraneanform - more severe
Peripheral smearshows Heinz bodies & bitecells
Unique sensitivitytofava beans
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Immunohemolytic Anemias
Caused by antibodies reacting againstnormal or altered RBCmembranes- Autoimmune, drug-induced- Antigen thatstimulates antibody or complement-mediateddestruction ofRBCs
Classification:1. Warm antibody: idiopathic, B-cell Neoplasms (CLL/SLL), SLE,
drugs (aldomet, PCN, Quinidine)2. Cold antibody: M. pneumoniae. Infectious mono.
Lab:- + DAT detects anti-RBC antibodies- Microspherocytes on peripheral smear
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Anti-Globulin (Coombs) Testing
Directantiglobulintesting
Indirectantiglobulintesting
Patients RBCs
Patients serum
Anti-C3d
Anti-IgG
+
RBCs
+
Anti-IgG
+
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Hemolytic Anemia with Intravascular
Hemolysis
Mechanicaldamage (Microangiopathichemolyticanemia)
Chemicaldamage (Burns)
Infection (MalariaorBabesiosis)
Transfusionreaction (ABO incompatibility)
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Thalassemia
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4 -globin genes
on chromosomes No 16
( 2 oneach chromosome)
2 fromthe father
& 2 fromthemother
2 - globin genes
on chromosomes No 11
(1 oneach chromosome)
1 fromthe father
& 1 fromthemother
NORMAL INDIVIDUAL
Normal / globinchainratiois 1/1
Net result :-
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Normal Hemoglobin(s)
2 2 A
2
F
Hb%
FA2
AAdult 1 1.6 - 3.2 97
Infant 7020 Negligible 3020
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Thalassemia
Decreasedproductionofnormalglobinchains
E thalassemia - deficiencyofE gene(s)
F thalassemia - deficiencyofF gene(s)
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Thalassemia
Imbalance of globin chain production
E- vs. F-thalassemia
anemia due to both decreased production and increased hemolysisin spleen
F- thalassemia has decreased/absentHgb A, increased Hgb F, andincreased Hgb A2
Treatment- hypertransfusion with chelation, splenectomy, bonemarrow transplantation
E thalassemiaEurope 4-12%MiddleEastand westernAsia - 12-55%
SoutheastAsia 6-75%
Africa 11-50%
SouthAmericaandthe Caribbean - 7%
HeterozygoteF thalassemiaItalian, Sicilian,and Greek10%
SoutheastAsian populations 5% in
inAfricanandAmerican blackpopulations1.5%
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Father Mother
/
Normal
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-/
--/
--/---/--
Silent carrier
- thal minor(-thal trait)
HbH disease
Hb. Barts
Hydrops fetalis
-Thalassemia(s)
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Alpha Thalassemia: Clinical
Features Absenceof1-2 alphachains
Common
Asymptomatic
Does notrequiretherapy
Absenceof3 alphachains
(Hgb H disease)
Microcyticanemia (Hgb 7-10)
Splenomegaly
Absenceof4 alphachains
Hydrops fetalis (non-viable)
EE
EE
EE
EE
EE
EE
EE
EE
XXX
X X
X XX
EE
EEX XX X
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-
thalassemia
minor
-thalassemia
major
- Thalassemia(s)
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Beta Thalassemia: Clinical
Manifestations
Skeletal Osteoporosis dueto bonemarrow expansion (crew cut)
Pneumatizationofthe sinuses is delayed byexpandedhematopoiesis
Dilatedcardiomyopathy secondaryto severeanemia
Growthanddevelopmentdelayed
Hepatomegalyduetoextramedullaryhematopoiesis
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Skeletal
Osteoporosis
Delayed pneumatization ofthe sinuses
Dilated cardiomyopathy
Growth and developmentdelayed
Hepatomegaly
Beta Thalassemia
Crew cut appearance
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Thalassemia
Smear
Microcytic / Hypochromic RBCs,Anisocytosis, Poikilocytosis, targetcells
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Hemolytic Anemia
RBC destruction
erythropoiesis:reticulocytosis
Iron accumulation:hemosiderosis
Etiology:
Intravascular: heat,
clostridia toxin, traumacomplement fixation
Extravascular (RES)
Intravascular:
hemoglobinemia,hemoglobinuria,
hemosiderinuria haptoglobin
Extravascular
No hemoglobinemia orhemoglobinuria
Normal haptoglobin
Microspherocytes
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Causes of High Hct/polycythemia
Relative or spurious erythrocytosis Hemoconcentration secondary to dehydration
(diarrhea, diaphoresis, diuretics, deprivation of water, emesis,
ethanol, etc.)
Absolute erythrocytosis (True ): Tissuehypoxia Smoking (Co), Highaltitude, Pulmonarydisease,
Cardiac shunts, Highoxygen-affinity Hb.
HighEPO - Tumors eg. HCC. Androgentherapy
Primary - Polycythemiavera
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