Anemia in Children: When to Transfuse Adekunle Adekile, MD, PhD Professor Department of Pediatrics Kuwait University

Outline Normal Hb levels at different ages Classification History and Physical Examination Indications for transfusion Case studies Practical Precautions

Normal Values at Different Ages Hb (g/dl) RBC WBC Birth 14.9-23.7 3.7-6.5 10.0-26.0 2 weeks 13.4-19.8 3.9-5.9 6.0-21.0 2 months 9.4-13.0 3.1-4.3 6.0-18.0 6 months 11.1-14.1 3.9-5.5 6.0-17.5 1 year 11.3-14.1 4.1-5.3 2-6 years 11.5-13.5 3.9-5.3 5.0-17.0 6-12 years 11.5-15.5 4.0-5.2 4.5-14.5 12-18 years (F) 12.0-16.0 4.5-13.0 12-18 years (M) 13.0-16.0 4.5-5.3

Morphologic Classification Microcytic Anemias Iron deficiency Chronic lead poisoning Thalassemias Chronic inflammation

Classification Contd. Macrocytic anemias With megaloblastic marrow vitamin B12 deficiency folic acid deficiency Without megaloblastic marrow aplastic anemia Diamond-Blackfan anemia

Classification Contd. Normocytic Anemias Congenital hemolytic anemias hemoglobin mutants RBC enzyme defects RBC membrane defects Acquired hemolytic anemias antibody mediated microangiopathic hemolytic anemias Secondary to acute infections Acute blood loss Splenic pooling Chronic renal disease

History Taking Age – Fe deficiency common in infants Sex – X-linked traits e.g. G6PD deficiency Ethnicity- hemoglobinopathies common in Mediterranean and Middle East Neonatal History - ?early hemolysis Diet - any evidence of malnutrition; exclusive breastfeeding in older infant Drugs - bone marrow suppression, hemolysis Infection - bone marrow suppression, hemolysis Inheritance - X-linked, autosomal recessive or dominant Diarrhea - ?malabsorption

Physical Examination Skin - pallor Facies – skull bossing, maxillary hyperplasia Eyes - jaundice Mouth – glossitis, cheilosis etc Heart – tachycardia, functional murmurs Hands - koilonychia Spleen/Liver - enlargement

RBC Transfusion: Indications Basic Rule: age, how rapid and how severe? Acute Blood Loss 10 – 15% Pre- or peri-surgical operation Hb <10 g/dl Symptomatic Anemia, usually with fall of 2 g/dl from baseline Hb <7 – 8gdl Suboptimal O2 Capacity (hypoxia, cyanotic heart disease) Hb < 12 – 13% Exchange (in neonates to prevent kernicterus or in HbSS for primary or secondary prevention of stroke) Special considerations in premature babies and critically ill

Case I Previously well 1-year-old male 2 day’s history of pallor and mild jaundice Recent febrile illness No other pertinent findings except for mild splenomegaly HR 100/min; ESM, RR 20/min Hb 6 g/dl, normocytic, normochromic; retics 5%, LDH 500 iu/l, total bilirubin 35 mmol/l DCT +ve What’s the diagnosis? Do you transfuse?

Case II 1-year-old boy 1-day history of pallor, jaundice and dark urine No previous episode History of fava bean ingestion Hemodynamically stable Hb 8 g/dl Do you transfuse?

Case III 1-year-old Poor feeder, still breastfed Presents with pallor of 2 months duration No other pertinent findings Hemodynamically stable Active child Hb 4 g/dl, MCV 48 fl, MCH 18 pg, RDW 20% Ferritin 4 ng/ml ?Transfuse

Case IV 1-year-old boy Known thalassemia major patient since 4 months Not regularly seen in the clinic Presents in the ER with Hb 9 g/dl Mother requests admission for transfusion What do you do?

Case V 1-year-old known HbSS patient Presents with cough, fever and chest pain CXR show bilateral infiltrates O2 sat 92% Hb 9 g/dl (her usual Hb is 11g/dl) Do you transfuse?

Acute Transfusion Reactions Hemolytic Reactions (AHTR) Febrile Reactions (FNHTR) Allergic Reactions TRALI Coagulopathy with massive transfusions Bacteremia

Products for Transfusion Leukoreduced Prevention of CMV, febrile reaction and WBC alloimunization CMV-negative Infants <6 months, transplant candidates, immunodeficiency Irradiated RBCs Infants < 6 months, malignancies, chemo or radiation, transplant, immunodeficiency Washed RBCs Hyperkalemia with poor renal function, anaphylaxis, PNH Extended phenotyping Hemoglobinopathy on chronic transfusion therapy Dose 10 – 15 ml/kg; rate depends on the clinical condition and age

Precautions Prescribing doctor Administering doctor State indication for Tx Volume and rate to be transfused Any anticipated problems and precautions Fever Allo or autoantibodies Administering doctor Check the name and identity of receiving patient Cross-check the blood group and X-matching ?any antibodies (DCT, auto/allo) Liaise with the nurse and give clear written instructions as indicated

Precautions Contd Nurses Two nurses Reconcile the unit and the patient to make sure there’s no mix-up Look at all orders from the prescribing doctor In case of any doubt, check with the doctor again Bring blood to room temperature Monitor patient’s subjective feelings Itching, rash Lightheadedness, chest tightness Nausea, vomiting or diarrhea Headache Monitor vital signs and promptly alert doctor if there’s any problem Jaundice, pallor, dark urine Clear record of the transfusion events

Thank you