ANEMIA

DEFINITION
– THE WORLD HEALTH
ORGANISATION (WHO) DEFINES
ANAEMIA AS A HEMOGLOBIN
LEVEL 13G/DL AND 12G/DL
definiton

– ANEMIA(AN-WITHOUT,EMIA-BLOOD) is a
decrease in the RBC count, hemoglobin values
resulting in a lower ability for the blood to carry
oxygen to body tissues.
 Classification of anaemia

– THE FUNCTIONAL CLASSIFICATION OF ANAEMIA HAS THREE

MAJOR CATEGORIES:
– MARROW PRODUCTION DEFECTS (HYPOPROLIFERATION).
– RED CELL MATURATION DEFECTS.(INEFFECTIVE
ERYTHROPOIESIS)
– DECREASED RED CELL SURVIVAL(BLOOD LOSS/HEMOLYSIS).
 HYPOPROLIFERATIVE ANEMIA

NUTRIENT DEFICIENCY BONE MARROW DYSFUNCTION

• IDA
• APLASTIC ANEMIA
• MEGALOBLASTIC
ANEMIA
• ACD
 MORPHOLOGICAL
CLASSIFICATION
MICROCYTIC ANEMIA NORMOCYTIC MACROCYTIC
MCV <80FL,SIZE <7 80 – 100, SIZE <7-8 >100,SIZE >9
S-SIDEROBLASTIC ANEMIA ANEMIA OF CHRONIC DISEASE RETICULOCYTE PRODUCTION
NORMAL

I-IRON DEFICIENCY ANEMIA LIVER DISEASE RECENT BLOOD LOSS

HEMOLYTIC ANEMIA

T-THALASSEMIA HEMOLYTIC ANEMIA RETICULOCYTE PRODUCTION

DEFECIENT:

A-ANEMIA OF CHRONIC ALCOHOLISM • MEGALOBLASTIC ANEMIA

DISEASE

HYPOTHYROIDISM • APLASTIC ANEMIA

MYELODYSPLASTIC SYNDROME • CKD
• HYPOTHYROIDISM
 CLASSIFICATION BASED ON HB

 MILD – 10 g/dl
 MODERATE – 8-10 g/dl
 SEVERE – 6.5 -7.9 g/dl
 LIFE THREATENING - <6.5 g/dl
ACUTE ANEMIA
• SUDDEN BLOOD LOSS & HEMOLYSIS
• MILD – ENHANCED O2 DELIVERY
( O2-HB DISSOCIATION CURVE)
• 10-15% - SIGNS OF VASCULAR
INSTABILITY.
• >30% - POSTURAL HYPOTENTION &
TACHYCARDIA, UNABLE TO
COMPENSATE
• >40% - SIGNS OF HYPOVOLEMIC
SHOCK (DYSPNEA, DIAPHORESIS,
HYPOTENSION,TACHYCARDIA)
CHRONIC ANEMIA
• 2,3 BISPHOSPHOGLYCERATE – O2-
HB CURVE SHIFT TO RIGHT &
FACILITATES O2 UNLOADING – (2-
3G/DL) DEFICIT IN HB
CONCENTRATION
• O2 DELIVERY TO VITAL ORGANS -
SHUNTING OF BLOOD (BLOOD
AWAY FROM KIDNEY,GUT,SKIN)
• CHRONIC INFLAMMATORY STATE
(RA, CA, INFECTION)
• LYMPHOPROLIFERATIVE DISORDER -
AIH
TYPES OF ANEMIA

1. IRON DEFICIENCY ANEMIA

2. HEMOLYTIC ANEMIA
3. PERNICIOUS ANEMIA
4. G6PD DEFICIENCY ANEMIA
5. APLASTIC ANEMIA
6. ANEMIA OF CHRONIC DISEASE
7. POST HEMORRHAGIC ANEMIA
8. SIDEROBLASTIC ANEMIA
IRON DEFICIENCY ANEMIA

• Ferum Inadequacy /Limited Ability To Absorb Iron

Aetiology:
 Blood Loss
 High Demand – Pregnancy, Growth
 Low Absorption – Chrons disease
 Poor iron intake
 Drug induced – antiprostaglandins, prednisolone
 METABOLISM
Duodenum
Diet (Fe 3+) • Absorption Left over
• Storage Transferrin
After
Plasma
120 days

Macrophage in BM
RBC

Erythropoiesis
Clinical features

– Brittle nails
– Koilonychia
– Atrophy of tongue papillae
– Angular stomatitis
– Brittle hair
– Dysphagia/glossitis
– Sudden increase of iron in high amount – bronze skin
INVESTIGATION
– PBS & CBC
• Hb – low
• RBS mass – low
• WBC – normal/low
• PLT – normal/low
• Microcytic hypochromic
• MCH – low
• MCV – low
• MCHC – low
• RDW - high
– BM examination: low iron stores
– Iron profile
MANAGEMENT

– Diet (meat, liver, veg)

– Ferrous sulphate 200mg tds
– Ferrous gluconate 300 bd
– Iron sorbital IM
– Iron sucrose IV 200mg in 100 ml NS
– Hbg - <6.5 g/dl - hemotransfusion
HEMOLYTIC ANEMIA

Intravascular Extravascular
– Hemoglobinemia – +/-
– Hemoglobinuria – +/-
– Hemosiderosis – +/-
– Hemosideriuria – +/-
– Low haptoglobin – Normal
– Jaundice – +
– Gall stones – Gall stones
Hemolysis

Intrinsic defect Extrinsic

– RBC membrane defect – Immune haemolytic anemia
• Hereditary spherocytosis
• Paroxymal nocturnal hemoglobinuria
– Hbg defects
• Thalassemia
– Enzyme defects
• G6PD
 HS
PATHOGENESIS:
Mutation in RBC proteins
RBC looses its shape &
flexibility
Will not able to pass through
the spleen
Eaten up by macrophage
Hemolysis (extravascular)
DIAGNOSITICS:
• PBS & CBC – Low RBC, low
HB, WBC-n, PLT –n,
spherocyte
• MCV –low, MCH – n, MCHC
– high, retic count – high
• OFT (osmotic fragility test) –
screening test- high d/t
more fragile
• Electrophoresis –
confirmatory test – Rx.
Elective splenectomy
 PNH
RBC membrane 2 GPI anchors d/t PIG-A gene

DAF (decay MIRL (M/I)

accelerating factor) (membrane inhibitor of reactive lysis)
CD55 CD 59
Mutation in PIGA No protective
gene covering
Low DAF & low MIRL High lysis of RBC via
complement system ( MAC)

Haemolysis (intravascular)
 Immune Haemolytic
Anaemia

Autoimmune Alloimmune
Warm Cold AIHA
AIHA Starts from
Core periphery Mom Rh (-)/ fetus Rh (+)
central Ab 1st pregnancy – IgM Ab = normal
attach 2nd pregnancy – IgG Ab =
37degree c with RBC 0-4 degree c erythroblastosis fetalis or hydrops
not fetalis
IgG destroy IgM
INVESTIGATION

Autoimmune Allloimmune
• Direct Coombs test + = HIS – Indirect coombs test positive
• - = HS
INVESTIGATIONS

• Low RBC – Screening test – hams test or

• Low WBC acidified serum test
– Sleep – hypoxia – low PH (activates
• Low platelets pancytopenia with
hepatosplenomegaly complement system) – haemolysis
– Confirmatory test – flaer flow
• High mutations: AML, aplastic
anemia, myeloid dysplastic cytometry
syndrome
Pernicious anemia

– Deficiency of vit B12 TRAID

– Colour index >1 1. Macrocytes (size>9 / MCV->100fl)
– Aetiology: s.b12 <100pg/ml 2. Neutrophils(>6segments)
– Chronic gastritis type A/chronic 3. Howell jolly body (nuclear
pancreatitis remnant inside RBC)
– Malabsorption Other findings:
– Drugs – biguanides, methotrexate, • Cabot ring (figure of 8)
alcohol, phenytoin
• Basophillic stippling
– Hyperthyroidism -
 Pathogenesis
methylcobalamine
Homocyteine Methionine

Adenosyl cobalamine
Methylmalonyl CoA Succinyl CoA

M.Severe complication of MA – SACD

(subacute combined degeneration)
Clinical features
– Splenohepatomegaly
– Anorexia,constipation/diarrhea,Abdominal pain.
– Glossitis
– Weight loss
– Neurological symptoms;loss of peripheral
sensation,weakness,dec.reflex,spasticity,paraesthesia,personal
ity changes,altered skin pigmenatation.
– Delusions.
diagnostics

– CBC AND MICROSCOPIC FINDINGS

– Macrocytosis,Anisocytosis,Poikilocytosis.
– HOWELL JOLLY BODIES
– Reticulopenia
– Hypersegmentation of granulocytes.
– Bone Marrow :Erythroid hyperplasia and megaloblastic changes.
 LABORATORY CONFIRMATION:
1. Decreased S.vit B12(150 pg/ml)
2. Serum indirect Bilirubin (Hemolysis).
3. Inc.LDH
4. INC. S.Ferritin Autoantibodies to gastric parietal cells.
5. Autoantibodies to Instrinsic Factor (IF)
6. Achlorhydria (PH>6.5)
7. Absent secretion of instrinsic Factor.
SCHILLING TEST:TO DETERMINE WHERE B12 DEFICIENCY LIES.
 TREATMENT

– VITAMIN B12-1000mg IM 2-4 Times A week until the hematologic count is

corrected ,then once a month for maintenance.

– CAUTION:IF ONLY FA IG GIVEN INSTEAD ,RESULTS IN NEUROLOICAL DEFECIT.

-Oral cobalamine may be used be used instead of parenteral therapy in a dose of 1000mcg/d
nd must be continued indefinitely.
 G6pd deficiency
– Function of G6PD is to neutralize free radical damage (oxidative stress )inside RBC.
– Both EXTRAVASCULAR>INTRAVASCULAR hemolysis.
– TRIGGERS;FAVA BEANS CNSUMPTION OR AM DRUGS like primaquine leads to intravascular hemolysis
hemoglobinemia hemoglobinuria/black urine ATN DEATH.
– DRUGS C/I; ASA,CHLOROQUINE,PRIMAQUINE,MEFLOQUINE.
– USED;ARTESUNATE,ARTEMISININ.
– S.HAPTOGLOBULIN IS ABSENT IN INTRAVASCULAR HEMOLYSIS.(40-200 MG/DL)
INVESTIGATIONS

– IOC;G6PD ASSAY
– P.SMEAR:HB DECRESED.
– BITE CELLS
– SUPRAVITAL STAIN(CRYSTAL VIOLET):HEINZ BODIES.
– CABOT RINGS AND HOWELL JOLLY BODIES(SPLENECTOMY PTS).
 APLASTIC ANEMIA
– Complete failure of bone marrow
• Idiopathic (M/C)
• Fanconi anemia – AR, defect in DNA repair gene
• Drugs – chloramphenicol, NSAIDS, carbimazole, phenytoin
• EBV, CMV, parvovirus B19
 Diagnosis:
 Pancytopenia
 No hepatosplenomegaly
 Low retic count
 BMA BMB = dry drop, low cellularity
 Rx. BMT /if not possible immunosuppressive agents
 ANEMIA OF CHRONIC DISEASE
• d/t chronic injury (drugs,
tumours, inflammation)
• IFN-gama, IL-1, IL-6
Activate liver
Low erythropoiesis in BM
Secrete hepcidin, low transferrin
Anaemia
(normocytic normochromic Blocks ferroprotein
anaemia)
Low transport of iron to BM

Anaemia (microcytic hypochromic

anaemia)
SIDEROBLASTIC ANEMIA

Enzyme
• Succinyl CoA PROTOPORPHYRIN + Fe
B6
ferrochelatase
(cofactor)

HEME
• Cause: congenital – low enzyme
Acquired – low B6
• Diagnosis: Ringed sideroblasts
 IRON PROFILE
IDA ACD Sideroblastic
anaemia

Iron Low Low High

Ferritin Low High High

TIBC High Low Low

T% Low Low High