Approach to Anemic Child [Autosaved].pptx

it's about hematologic diseases in pediatrics

1. Approach to Anemic Child

2. Anemia is a problem of not having enough healthy red blood cells or hemoglobin to carry oxygen to the body's tissues. Generally caused by not enough making or too much breaking Reduction in Hemoglobin concentration, hematocrit, RBC count Hemoglobin (Hb) level less than -2 SD for age and sex of an individual

3. The national prevalence of anemia in the 2016 Ethiopia Demographic and Health Survey (DHS) was estimated to be  56%  among children under the age of 5 years Approximately 29% of children have mild anemia, with hemoglobin levels between 10-10.9 g/dL. Around 23% of children have moderate anemia, with hemoglobin levels between 7-9.9 g/dL. About 5% of children have severe anemia, with hemoglobin levels below 7 g/dL.

4. PHYSIOLOGIC ANEMIA OF INFANCY Intrauterine hypoxia stimulates erythropoietin → ↑ RBCs (Hb, Hct ) High FiO2 at birth downregulates erythropoietin Progressive drop in Hb over first 2–3 months until tissue oxygen needs are greater than delivery (typically 8–12 weeks in term infants, to Hb of 9–11 g/dL) Exaggerated in preterm infants and earlier; at 3–6 weeks to Hb of 7–9 g/dL In term infants—no problems, no treatment; preterm infants usually need transfusions depending on degree of illness and gestational age

5. Normal Values RBC = 5 million cells/ ul Hgb = 15g/dl Hct = 45% Red Blood Cell Measurements • RBC count • Hemoglobin • Hematocrit Rule of 3 • Hgb = 3 x Red Blood Cell Count • Hct = 3 x Hgb

6. RBC Indices • Mean corpuscular volume (MCV) • Normal range: 80 to 100 femtoliters • Mean corpuscular hemoglobin (MCH) • Amount (mass) of hemoglobin per red cell • Usually reported in picograms (per cell) • Mean corpuscular Hgb concentration (MCHC) • Concentration of Hgb in red cells • Usually reported g/dL

7. Classification could be based on or physiologic morphologic base. Morphologic : RBC size(MCV) Microcytic Normocytic Macrocytic Physiologic Anemia of decreased production Increased destruction or loss The peripheral blood reticulocyte percentage or absolute number will help to make a distinction between the two

8. Reticulocytes • Immature red blood cells • Usually about 1-2% of RBCs in peripheral blood • Increased reticulocyte count: Hallmark of hemolysis • ↑ reticulocytes: normal marrow response to anemia

9. Microcytic anemia • Loss of iron • Loss of globin (thalassemia) • Loss of heme (lead, sideroblastic) Microcytic Anemias • Usually due to ↓ hemoglobin in red cells • Usually associated with ↓ MCH and MCHC • Low hemoglobin → hypochromic RBCs on smear

10. Iron deficiency anemia most common cause of anemia worldwide Infants with decreased dietary iron typically are anemic at 9–24 months of age: caused by consumption of large amounts of cow milk and foods not enriched with iron; also creates abnormalities in mucosa of GI tract → leakage of blood ( GI ulcer, polyps, hemangioma, worm infestation), further decrease in absorption( lead poisioning , celiac disease, tropical sprue). Adolescents also susceptible → high requirements during growth spurt, dietary deficiencies, menstruation

11. Clinical appearances— pallor most common irritability, lethargy pagophagia, pica Tachycardia, systolic murmurs long-term with neurodevelopmental effects

12. Laboratory findings First decrease in bone marrow hemosiderin (iron tissue stores) Then decrease in serum ferritin Decrease in serum iron and transferrin saturation → increased total iron-binding capacity (TIBC) Increased free erythrocyte protoporhyrin (FEP) Microcytosis, hypochromia, poikilocytosis (abnormal shape) , anisocytosis (heterogeneous in size/shape) Decreased MCV, mean corpuscular hemoglobin (MCH), increase RDW, nucleated RBCs, low reticulocytes Bone marrow—no stainable iron

13. Treatment Oral ferrous salts Limit milk, increase dietary iron Within 72–96 hours—peripheral reticulocytosis and increase in Hb over 4–30 days Continue iron for 8 weeks after blood values normalize; repletion of iron in 1–3 months after start of treatmen

14. Parenteral therapy indications poor compliance severe bowel disease intolerance of oral iron chronic hemorrhage acute diarrhea disorder Contraindications Neonates. Iron overload. History of hypersensitivity to parenteral iron preparations. History of severe allergy or anaphylactic reactions. Clinical or biochemical evidence of liver damage. Acute or chronic infection.

15. Transfusions:- Indications INFANTS ≤ 4 MO OLD Hemoglobin < 13.0 g/dL and severe pulmonary disease or severe cardiac disease Hemoglobin < 10.0 g/dL and moderate pulmonary disease or major surgery Hemoglobin < 8.0 g/dL and symptomatic anemia

16. CHILDREN AND ADOLESCENTS Acute loss of > 25% of circulating blood volume Hemoglobin < 13.0 g/dL and severe cardiopulmonary disease Hemoglobin < 8.0 g/dL and symptomatic chronic anemia or marrow failure or in the perioperative period Hemoglobin < 4.0 g/dL in malnourished patient Hemoglobin < 10 g/dL in patients undergoing chemotherapy or radiotherapy

17. Factors other than hemoglobin concentration to be considered in the decision to transfuse RBCs include The patient's symptoms, signs, and compensatory capacities; The presence of cardiorespiratory, vascular, and central nervous system disease; The cause and anticipated course of the anemia Alternative therapies Benefit and risk of transfusion versus effect of anemia on growth and development

18. The anemia of chronic disease (ACD), also referred to as anemia of inflammation, is found in conditions where there is ongoing immune activation. Associated with infections, autoimmune diseases, as well as some hematologic and solid malignancies, Common in rheumatoid arthritis, lymphoma There is decreased span of RBC life, relative failure of bone marrow to respond, increased EPO level and blunted response and relative resistance to EPO Iron availability is usually low due to it’s accumulation in reticuloendothelial system and decreased intestinal absorption

19. Labs Hb typically 6–9 g/dL, most normochromic and normocytic (but may be mildly microcytic and hypochromic) Reticulocytes normal or slightly decreased for degree of anemia Iron low without increase in TIBC Ferritin may be normal or slightly increased. Marrow with normal cells and normal to decreased RBC precursors

20. Treatment and Prognosis Anemia of chronic disease does not respond to iron unless there is concomitant deficiency If the underlying systemic disease can be controlled, the anemia will resolve Recombinant human EPO can increase the hemoglobin level and improve activity and the sense of well-being. In this instance, treatment with iron is usually necessary for an optimal EPO effect

21. Megaloblastic anemias Is a macrocytic anemia characterized by ineffective erythropoiesis Almost all are folate or vitamin B12 deficiency . There is usually an associated thrombocytopenia and leucopoenia The peripheral blood smear is notable for large, often oval, RBCs, with increased mean corpuscular volume (MCV). Neutrophils are characteristically hypersegmented , with many having >5 lobes.

22. VITAMIN B12 (COBALAMIN) DEFICIENCY Only animal sources; produced by microorganisms (humans cannot synthesize) Serves as a cofactor in 2 essential metabolic reactions which are important for the production of DNA Sufficient stores in older children and adults for 3–5 years; but in infants born to mothers with deficiency, will see signs in first 4–5 months

23. Inadequate nutrition (extreme restriction [vegans]), lack of intrinsic factor (congenital pernicious anemia [rare], autosomal recessive; also juvenile pernicious anemia [rare] or gastric surgery), impaired absorption (terminal ileum disease/removal)

24. Clinical—weakness, fatigue, failure to thrive, irritability, pallor, glossitis, diarrhea, vomiting, jaundice, many neurologic symptoms

25. diagnosis In very young children evaluation of the mother for anemia and vitamin B12 absorption of vitamin B12 can be assessed by the Schilling test Lab findings:- normal serum folate and decreased vitamin B12 the serum concentrations of methylmalonic acid and homocysteine usually are elevated. Treatment—intramuscular B12 1-2 times weekly.

26. Folate deficiency, next to iron deficiency, is one of the commonest micronutrient deficiencies worldwide. Sources of folic acid—green vegetables, fruits, animal organs Is absorbed in the proximal small intestine Important for the synthesis of purines

27. Cause —inadequate intake (pregnancy, goat milk feeding, growth in infancy, chronic hemolysis), decreased absorption congenital defects of folate metabolism Drug-Induced abnormalities in folate metabolism

28. Clinical manifestations peak incidence at 4-7 mo of age irritability, chronic diarrhea , and poor weight gain Haemorrhages from thrombocytopenia can occur in advanced cases

29. Lab Findings Macrocytic anemia Low reticulocyte count Neutropenia and thrombocytopenia

30. Treatment Folic acid may be administered orally or parenterally at 0.5-1.0 mg/day for 3-4wks Transfusions are indicated only when the anemia is severe or the child is very ill.

31. CONGENITAL ANEMIAS

32. CONGENITAL PURE RED-CELL ANEMIA (BLACKFANDIAMOND) uncomplicated. The patient has been doing well according to the mother. Increased RBC programmed cell death → profound anemia by 2–6 months Congenital anomalies Short stature Craniofacial deformities Defects of upper extremities; triphalangeal thumbs

33. Labs Macrocytosis Increased HbF Increased RBC adenosine deaminase (ADA) Very low reticulocyte count Increased serum iron Marrow with significant decrease in RBC precursors

34. Treatment Corticosteroids Transfusions and deferoxamine If hypersplenism, splenectomy; mean survival 40 years without stem cell transplant Definitive—stem cell transplant from related histocompatible donor

35. CONGENITAL PANCYTOPENIA Most common is Fanconi anemia—spontaneous chromosomal breaks Age of onset from infancy to adult Physical abnormalities Hyperpigmentation and café-au-lait spots Absent or hypoplastic thumbs Short stature Many other organ defects

36. Labs Decreased RBCs, WBCs, and platelets Increased HbF Bone-marrow hypoplasia Diagnosis—bone-marrow aspiration and cytogenetic studies for chromosome breaks Complications—increased risk of leukemia (AML) and other cancers, organ complications, and bone-marrow failure consequences (infection, bleeding, severe anemia) Treatment Corticosteroids and androgens Bone marrow transplant definitive

37. ACQUIRED ANEMIAS TRANSIENT ERYTHROBLASTOPENIA OF CHILDHOOD (TEC) Transient hypoplastic anemia between 6 months–3 years Transient immune suppression of erythropoiesis Often after nonspecific viral infection (not parvovirus B19) Labs—decreased reticulocytes and bone-marrow precursors, normal MCV and HbF Recovery generally within 1–2 months Medication not helpful; may need one transfusion if symptomatic

38. Hemolysis Hemolysis is defined as the premature destruction of red blood cells (RBCs). Anemia results when the rate of destruction exceeds the capacity of the marrow to produce additional RBCs. – Normochromic, normocytic anemia – Shortened RBC survival – Reticulocytosis – due to ↑ RBC destruction

39. INTRINSIC HEMOLYSIS

40. EXTRINSIC HEMOLYSIS Nonimmune

41. EXTRINSIC HEMOLYSIS . Immune hemolytic anemias 1. Autoimmune hemolytic anemia - caused by warm-reactive antibodies - caused by cold-reactive antibodies 2. Transfusion of incompatible blood

42. Clinical and Laboratory Features Suggestive of Hemolytic Anemia Pallor Icterus Splenomegaly Gallstones History of neonatal icterus Positive family history of anemia, splenectomy, cholecystectomy ↑ Reticulocyte count

43. ↑ RDW (caused by ↑ reticulocyte count) Abnormal RBC morphology ↑ Indirect bilirubin (normal direct bilirubin) ↓ Serum haptoglobin level ↑ Urinary urobilinogen level Hemoglobinuria (+ dipstick test result for blood; no RBCs in urine) ↑ LDH level