1. 1 Approach to Anemia in Children Dr.Hekmati Moghaddam

2. 2 Case 1  A 2- years old baby with following CBC refers to you. History and physical examination were normal, Hg: 12.1 Hct: 36.3 RBC: 4.6 MCV: 76 MCH:26

3. 3 Question 1  What is your recommendation?

4. 4 Question 2  If ferritin and hemoglobin electrophoresis were normal, what is next step?

5. 5 Case 2  A Pregnant women with the history of anemia in herself and her husband and one of her children refers to you with the following lab tests:

6. 6 Father:  Hb: 11 RBC: 5.9 MCV: 74 HbA2: 1.2%  Deletion of 2 α gene Mother:  Hb: 10.5 RBC: 5.9 MCV: 63 HbA2: 6% β gene mutation, normal α gene β gene mutation, normal α geneDaughters: Hb: 13 RBC: 4.9 MCV: 83 HbA2: 2.4 Hb: 13 RBC: 4.9 MCV: 83 HbA2: 2.4 Hb: 11 RBC: 6 MCV: 71 HbA2: 1.1 Hb: 11 RBC: 6 MCV: 71 HbA2: 1.1

7. 7 Question  What is your recommendation?

8. 8 Approach to the child with anemia

9. 9 Definition of Anemia

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11. 11  Classification of anemia (Morphologic) Microcytic Microcytic Macrocytic Macrocytic Normocytic Normocytic

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13. 13 History taking  Patients with inherited etiologies often present in childhood. Thus, when evaluating the history of an anemic patient, one must not only review the symptoms of the patient, but also ask pointed questions regarding family history

14. 14  Severity and initiation of symptoms  Questions relating to hemolytic episodes  Prior therapy or anemic episodes  Questions about possible blood loss  Underlying medical conditions  Prior drug or toxin exposure  Questions relating to diet  Birth history  Developmental milestones  Family history, race, and ethnicity

15. 15 Physical examination

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17. 17  Clinical pallor in the conjunctiva, palm, and nail beds was detected in only 20 percent of those with HGB <11.0 g/dL and 61 percent of those with severe anemia (HGB <7.0 g/dL)

18. 18 LABORATORY EXAMINATION

19. 19  Blood smear  Red blood cell indices  Red cell distribution width  Reticulocyte count  White blood count and platelet count

20. 20  In the pediatric population, many blood samples obtained for anemia screening are capillary samples such as finger or heel "sticks." Although these means of sampling are acceptable, one must keep in mind that HGB and HCT values may be slightly elevated in such samples as compared to venous samples when using automated counting methods. This difference may be more pronounced when using microhematocrit measurements from capillary samples. Although the likelihood of masking significant anemia is low, borderline low values may be "normalized" using the capillary collection and processing method.

21. 21  The laboratory examination should begin with a complete blood count including red blood cell indices, a Reticulocyte count, and a review of the peripheral blood smear

22. 22  The red cell distribution width (RDW) is a quantitative measure of the variability of RBC sizes in the sample (anisocytosis).

23. 23  The RDW is a function of MCV and, therefore, normal values vary slightly with age. However, normal values generally fall between 12 and 14 percent

24. 24  The RDW is especially helpful in differentiating Iron deficiency from thalassemia in the pediatric patient with microcytic anemia. Patients with a RDW greater than 20 are more likely to have Iron deficiency, whereas patients with normal RDW values are more likely to have thalassemia or the anemia of chronic disease

25. 25   Table 3.1 Some causes of microcytosis.   Inherited   β thalassaemia heterozygosity (β thalassaemia trait, β thalassaemia minor)   β thalassaemia homozygosity or compound heterozygosity (β thalassaemia major or intermedia)   δβ and γδβ thalassaemia heterozygosity or δβ thalassaemia homozygosity   Haemoglobin Lepore heterozygosity or homozygosity   Hereditary persistence of fetal haemoglobin homozygosity and some instances of heterozygosity   α0 thalassaemia heterozygosity   α+ thalassaemia homozygosity or, to a lesser extent, heterozygosity   Haemoglobin Constant Spring heterozygosity   Haemoglobin H disease   Sickle cell heterozygosity [6,7] (disputed, see p. 303)   Haemoglobin C heterozygosity [6,7] and homozygosity   Sickle cell/haemoglobin C disease [8]   Haemoglobin E heterozygosity [9] and homozygosity [10]   Haemoglobin D-Los Angeles (D-Punjab) heterozygosity   Heterozygosity for other rare abnormal haemoglobins producing thalassaemia-like conditions (e.g. haemoglobin Tak, haemoglobin Indianapolis)   Congenital sideroblastic anaemia   Atransferrinaemia   Ferrochelatase deficiency (erythropoietic protoporphyria) [11]   Hepatoerythropoietic porphyria [12]   Associated with iron overload but with absent bone marrow iron [13]   Associated with elliptocytosis [14]   Inherited iron malabsorption plus defect in incorporation of iron [15]   Acaeruloplasminaemia [16]   Copper deficiency [17]   Haem oxygenase deficiency [18]   Homozygosity for DMT1 mutation [19]   Acquired   Iron deficiency (including bone marrow iron deficiency in pulmonary haemosiderosis)   Anaemia of chronic disease   Myelodysplastic syndromes, particularly but not only [20] associated with acquired haemoglobin H disease   Secondary acquired sideroblastic anaemia (e.g. caused by various drugs; some cases of lead poisoning and some cases of copper deficiency [21] or zinc excess with functional copper deficiency, e.g. ingestion of zinccontaining coins as a feature of mental illness) [22–24]; ? hyperzincaemia with hypercalprotectinaemia (nature of anaemia not specified) [25]   Hyperthyroidism [26]   Ascorbic acid deficiency (rarely) [27]   Cadmium poisoning [28]   Aluminium poisoning   Antibody to erythroblast transferrin receptor [29]

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