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Anemia Introduction. Dr. Sachin Kale, MD. Asso . Prof, Dept. of pathology In charge, Central Laboratory, MGM. Outline. Introduction to hematology and hematopoiesis Introduction to anemias Iron deficiency anemias Megaloblastic anemia. Sickle cell anemia. Anemias.
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Anemia Introduction Dr. Sachin Kale, MD. Asso. Prof, Dept. of pathology In charge, Central Laboratory, MGM.
Outline • Introduction to hematology and hematopoiesis • Introduction to anemias • Iron deficiency anemias • Megaloblastic anemia. • Sickle cell anemia
Anemias • Signifies a decrease in Hb or Hct and represents underlying disease than a specific diagnosis • Accepted definitions - • Male: < 13.5 g/dl • Female: < 12.5 g/dl • Pregnancy & Children - ( 6 m – 8 yrs): < 11 g/dl • Preterm infants: < 14 ; Full term infant: < 13.5
Anemias • SaO2 ( % of heme groups occupied by O2) and PaO2 ( amount of O2 dissolved in plasma) are normal; since O2 exchange in lungs are normal. • However oxygen content (total amt of O2 available) is decreased owing to reduction in Hbconcentraion.
Mature RBC • Anucleate cells • Devoid of mitochrondria – lack citric acid cycle, beta oxidation of fatty acid, oxidative phosphorylation • Metabolize glucose by anerobicglycosylation – lactate is the end product. • Generate glutathione via pentose phosphate shunt.
Mature RBC • Reduce heme iron from ferric (+3) to ferrous (+2) state using methemoglobinreductase system Synthesizes 2,3 bisphosphoglycerate via Rappapor-Luebering shunt. ( used for right shifts in O-D curve) • ABO & Rh antigens on membranes.
Mature RBC • Senescent RBCs are removed mainly by extravascularhemolysis – endproduct is lipid soluble unconjugatedbilirubin. • Lesser extent – intravascular hemolysis.
Basic pathophysiological categories of anaemia • Blood loss • Impaired red cell production • Inadequate supply of nutrients essential for eythropoiesis, such as: . • iron deficiency • vitamin B 12 deficiency • folic acid deficiency • protein-calorie malnutrition • other less common deficiencies
Impaired red cell production • Depression of erythropoietic activity • Anaemia associated with chronic disorders. such as: • infection • connective tissue disorders • inflammatory disorders • disseminated malignancy • Anaemia associated with renal failure • Aplasticanaemia • Anaemia due to inherited disorders, such as thalassaemia
Impaired red cell production • Anaemia due to replacement of normal bone marrow by: • Leukaemia • Lymphoma • myeloproliferative disorders • Myeloma • myelodysplastic disorders
Excessive red cell destruction • Due to intrinsic defects in red cells • Due to extrinsic effects on red cells
General evidence of hemolysis • Evidence of increased HB breakdown: • Jaundice and Hyperbilirubinemia • Evidence of compensatory erythroid hyperplasia: • Reticulocytosis • Evidence of damage to red cells: • Spherocytosis • Fragmentation RBCs • Heinz bodies
Classification of anemias • Microcyticanemias: ( MCV < 80 fl) • Iron deficiency (most common) • Thalassemia • Anemia of chronic disease • Sideroblastic anemia
Classification of anemias • Macrocytic anemia (MCV > 100 fl) • B12 deficiency • Folate deficiency • Alcoholic liver disease • Hypothyroidism
Normocytic anemia ( MCV 80 – 100 fl) • Reti count: (< 2%) • Acute blood loss • Early iron deficiency • Aplastic anemia • Anemia of chronic disease • Renal disease
Normocytic anemia ( MCV 80 – 100 fl) • Reti count: (> 3%)( Intrinsic RBC defect) • Membrane defects • Congenital spherocytosis/elliptocytosis • Paroxysmal Nocturnal Hemoglobinuria (PNH) • Abnormal hemoglobins: • Sickle cell disease variants • Enzyme deficiencies • G6PD & Pyruvatekinase deficiency.
Normocytic anemia ( MCV 80 – 100 fl) • Reti count: (> 3%) ( Extrinsic RBC defect) • Autoimmune hemolytic anemias ( warm and cold) • Paroxysmal cold hemoglobinuria • Microangiopathic hemolytic anemia
Understanding CBC: the complete blood count • Haematocrit is 3 times the HB value: Rule of 3. • RBC count usually parallels HB and Hct, • In thallasemias RBC count is normal to increased even though Hb is low. • RDW: Red cell distribution width • WBC count: Total and differential • Blood film:
RBC indices • MCV: volume of average red cell (fl or um3) MCV = Hctx1000/RBC count ( in millions per ul) • MCH: content (wt) of Hb of average red cell MCH = Hb (g/l)/RBC ( in millions per ul) • MCHC: average concentration of Hb in given volume of packed cells. MCHC: Hb(g/dl)/Hct
All of the following cause microcytic anemia except Iron deficiency anemia Thalasemia Alcoholic liver disease Anemia of chronic disease
All of the following cause microcytic anemia except Iron deficiency anemia Thalasemia Alcoholic liver disease Anemia of chronic disease
All of the following cause normocytic anemia with reti count < 2%, except Aplastic anemia Hereditary spherocytosis Acute blood loss Anemia of renal disease
All of the following cause normocytic anemia with reti count < 2%, except Aplastic anemia Hereditary spherocytosis Acute blood loss Anemia of renal disease