ANEMIA Anemia: decrease in the number of RBCs, Hb content, or Hct content below the lower limit of the normal range for the age and sex of the individual Pediatric Anemia A. Hemolytic Anemia: there is premature destruction of RBCs 1. Hereditary Spherocytosis: most common inherited abnormality of RBC membrane; defect in ankyrin, band 3 or spectrin proteins surface area deficiency leading to spherocytosis 2. Pyruvate Kinase Deficiency: defect in PKLR gene PK cannot convert phosphoenolpyruvate to pyruvate low ATP levels producing membrane injury 3. G6PD Deficiency: defect in G6PD protein G6PD enzyme cannot oxidize glucose-6-phosphate to 6-phosphogluconate (which concomitantly reduces NADP+ to NADPH, which protects RBCs from oxidative stresses) no NADPH makes RBCs susceptible to oxidative stresses 4. Thalassemia: imbalance in alpha and beta globin chain production disruption of RBC maturation leading to ineffective erythropoiesis B. Normal Lab Values (important to determine if there is anemia) *Remember: Normal values vary depending on the source and might cause confusion. So just try to memorize the magic numbers to simplify things.
Nutritional Anemias 1. Folic Acid Deficiency Anemia: macrocytic, megaloblastic anemia 2. Vitamin B12 Deficiency Anemia: macrocytic megaloblastic anemia 3. Iron Deficiency Anemia: microcytic, hypochromic anemia Serum Iron ↓ ↑/N ↑ ↓ Total Iron Binding Capacity ↑ (only IDA has high TIBC) ↓/N ↓/N ↓ % Saturation ↓ ↑/N ↑ ↓
M: 38.8-46.4 F: 35.4-44.4 M: 13.3-16.2 F: 12.0-15.8 4-5 6 3 x 10 /mm 2
MAGIC NUMBER: 35 (Equivalent measurements: Packed Cell Volume, Erythrocyte Volume Fraction) MAGIC NUMBER: 12 MAGIC NUMBER: 4,000,000/mm 3
IDA Thal SA ACD Time 12-24 H 36-48 H 48-72 H 4-30 D 1-3 M C.
RBC count RPI
Reticulocyte Production Index: to determine reticulocyte response to anemia [Retic Count x (Hct/Normal Hct)]/2 (hypoproliferative: 2)
Mean Cell Hemoglobin: Hgb/RBC count (hypochromic: 31)
Iron administration response Replacement of intracellular iron enzymes; CLINICAL IMPROVEMENT Initial bone marrow response; ERYTHROID HYPERPLASIA RETICULOCYTOSIS peaking at 5-7 days HEMOGLOBIN INCREASE REPLETION OF IRON STORES
Mean Cell Hemoglobin Concentration: Hgb/Hct (hypochromic: 36)
Mean Cell Volume: to differentiate types of anemia based on RBC size (microcytic: 100) 3
150-450 3 3 x 10 /mm
MAGIC NUMBER: 150,000/mm
Treatment 1. For Hemolytic Anemias Exchange transfusion for hyperbilirubinemia Transfusions of packed RBCs for severe anemia or for aplastic crises Splenectomy for recurrent and very severe anemia (patient must be 5-6 years of age) Post-splenectomy vaccinations (pneumo, meningo, HiB) PKD: prevention of hemolytic agents is the most important therapeutic measure Thal: plus chelation and bone marrow transplant (last resort) 1/15
By Yves Liong, Jen Magno and Chi Vivo
2. For Nutritional Anemias Supplementation of deficient nutrient Blood transfusion if necessary Correction of underlying condition Hgb 9.5 g/dL (low; NV12) Hct 30% (low; NV: 35) BP 140/90 (hypertension) A 45 y/o male with Chronic Renal Failure secondary to IgA Nephropathy has been undergoing dialysis for 5 years now. Vital signs stable.CBC showed: Hgb 10.0 g/dL (low; NV: 12) Hct 30% (low; NV: 35) A 58 y/o female with Hodgkin’s lymphoma presents with pallor. Hgb 8.5 g/dL (low; NV: 12) Hct 28% (low; NV: 35) Afebrile BP 120/80 HR 135/min (tachycardic) fluid to improve Hgb and Hct. Anemia Secondary to Chronic Disease Erythropoeitin
Adult Anemia A. Hypoproliferative Anemia: marrow production defects 1. Associated with normocytic and normochromic RBCs with low RPI 2. Includes anemia of acute/chronic inflammation/infection, anemia of renal disease, anemia of hypometabolic states, pure red aplasia, aplastic anemia B. Ineffective erythropoiesis: red cell...