Anemia -dr.Bertha- by: Huei, Elsya, Yuli, Au, Nagisa Development of the hematopoietic system Mesoblastic : yolk sac, 10 14 th days of gestation Hepatic : liver, 6-8 weeks to 24 weeks of gestation, 85% erythroid Myeloid : bone marrow, 40% erythroid Pluripotent stem cells : capable of self renewal and clonal maturation into all blood cell lineages Eritropoietin (EPO) Glikoprotein 30-39 kd Berikatan dengan reseptor spesifik yang terdapat pada permukaan prekursor eritroid stimulasi diferensiasi, maturasi klonal hingga terbentuk eritrosit matur Produksi EPO sangat dipengaruhi oleh konsentrasi O 2
o Hipoksia, anemia stimulasi produksi EPO stimulasi eritropoiesis Hemoglobin O 2 -carrying protein Complex protein consisting: Heme iron containing tetramer (2 pairs of peptide chains) Globin (protein) HbA 22 (adult) HbF 22 (fetal) Hb embrionik : Gower-1, Gower-2, Portland Anemia Reduction of RBC volume or Hb concentration below the normal values Reduction in the amount of Hb decrease O 2 -carrying capacity Few clinical disturbances occur 7-8 g/dL Normal value Age Hb (g/dL) Ht (%) Cord blood 16.8 (13.7-20.1) 55 (45-65) 2 wk 16.5 (13-20) 50 (42-66) 3 mo 12.0 (9.5-14.5) 36 (31-41) 6 mo 6 yr 12.0 (10.5-14) 37 (33-42) 7-12 yr 13.0 (11-16) 38 (34-40) Adult: Female 14 (12-16) 42 (37-47) Male 16 (14-18) 47 (42-52)
Inadequate production o Defisiensi Fe, folic acid, vit. B12, vit. C, protein, vit. B6 o Bone marrow failure congenital vs acquired including : chronic disease, renal failure (Fe, eritropoeitin, IFN-, IFN-) Blood loss Hemolytic process o Cellular defect (corpuscular) membrane defect enzyme deficiency hemoglobinopathy o Ekstracellular defect (extracorpuscular) autoimmune hypersplenism
Clinical Manifestations
Pallor (skin and mucous membrane) Physiologic adjustments o cardiac output o O 2 extraction ( A-V O 2 difference) o Shunting of blood flow towards vital organs & tissues o 2,3-DPG within the RBC When moderate-severe anemia develops slowly few signs and symptoms dissapear Weakness, tachypnea, tachycardia, cardiac dilatation, CHF Diagnosis History and PE Initial lab examination Advance lab examination Approach to common causes of anemia in children Is anemia associated with other hematologic abnormalities? Yes aplastic anemia, anemia, other bone marrow replacement disorders Is anemia associated with reticulocytosis? Yes bleeding, hemolysis Is there associated hyperbilirubinemia? (or increased serum lactase dehydrogenase?) Yes hemolysis Is anemia associated with reticulocytopenia? Yes assess RBC size Are red blood cells microcytic? Yes hemoglobinopathy, Fe deficiency, lead poison Are red blood cells macrocytic? Anemia dr. Bertha- by: Huei, Elsya, Yuli, Aurora, Nagisa Page 3
Yes o Is there neutrophil hypersegmentation? Yes folate def, vit B12 def, inborn error metabolism No Diamond Blackfan anemia, Pearson Syndrome Are red blood cells normocytic? Yes chronic disease, renal failure, hypothyroidism
Causative Physiologic anemia of infancy Normal newborn higher Hb & Ht, larger RBC Within 1 st week progressive decline in Hb level begins and persists for 6-8 weeks Physiologic anemia Normally reached between 8-12 weeks old (Hb 9-11 g/dL) Preterm infants occur by 3-6 weeks, Hb level of 7-9 g/dL