Anemia Anaemia is defined as an Hb level

Anemia ▪ Anaemia is defined as an Hb level below the ▪ ▪ ▪ normal range. The normal range varies with age, so anaemia can be defined as: • Neonate: Hb <14 g/dl • 1– 12 months: Hb <10 g/dl • 1– 12 years: Hb <11 g/dl.

Physiologic Anemia of the Newborn ▪ At one week postnatal all RBC indices begin declining to a minimum value reached at about 2 months of age. � decreased RBC production � plasma dilution associated with increasing blood volume � shorter life span on neonatal RBCs (50 -70 days) � more fragile RBCs � switch from Hb. F to Hb. A ■ Hb. F decreases about 3% per week ■ at 6 mo. Hb. F represents only 2% of total Hb ■ switch to Hb. A provides for greater unloading of oxygen to tissues d/t lower oxygen affinity of Hb. A relative to Hb. F. � seldom produces symptoms � not altered by nutritional supplements

Anemia of Prematurity ▪ Occurs in low birth weight infants w/ poor erythropoietin response � Protein content of breast milk may not be sufficient for hematopoiesis in the premature infant. � Hb level rapidly declines after birth to a low of 7 -10 g/dl at 6 weeks of age. � Signs and Symptoms ■ apnea ■ poor weight gain ■ pallor ■ decreased activity ■ tachycardia

Fe++ deficiency ▪ Tx– � Fe++ replacement gives dramatic response reticulocytosis in 72 hr, Hgb responds at ~1 g/L per wk, iron stores us. replenished by 3 mo

Beta Thalassemia Major ▪ ▪ ▪ No production of Beta chains- Chromosome 11 Autosomal recessive 25 % chance with each pregnancy Pre-natal testing for carriers Chorionic villous sampling for diagnosis Transfusion dependent-allows for normal development Pen Prophylaxis, Anti oxidants Splenectomy after age 5 Iron overload- inherent and transfusion Need chelators BMT is a cure

Management Blood transfusion lifelong monthly transfusions of red blood cells. Keep Hb anove 10 g/dl. iron chelation with subcutaneous desferrioxamine, or with an oral iron chelator drug, such as deferasirox, starting from 2 to 3 years of age. Patients who comply well with transfusion and chelation have a 90% chance of living into their forties Bone marrow transplantation, which is currently the only cure. It is generally reserved for children with an HLAidentical sibling as there is then a 90– 95% chance of success (i. e. transfusion independence and long-term cure) but a 5% chance of