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Approach to Childhood Anemia. H. Tamary Hematology, Schneider Children’s Medical Center of Israel. Normal Hemoglobin and MCV Values in Term Infant. Hb MCV (g/dL) (fl) Day 1 19.0±2.2 119 ±9.4 12 weeks 11.3 ±0.9 88 ±7.9. Regulation of Erythropoiesis.

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Approach to childhood anemia

Approach to Childhood Anemia

H. Tamary

Hematology, Schneider Children’s Medical Center of Israel


Normal hemoglobin and mcv values in term infant
Normal Hemoglobin and MCV Values in Term Infant

HbMCV

(g/dL) (fl)

Day 1 19.0±2.2 119 ±9.4

12 weeks 11.3 ±0.9 88 ±7.9






Criteria for identifying children with low hemoglobin values
Criteria for Identifying Children with Low Hemoglobin Values

Age Hemoglobin

(g/dL)

6ms –11 years <11

>11 male <13

>11 female <12


Etilogical classification of anemia i
Etilogical Classification of Anemia (I)

A. Blood loss

B. Excessive blood destruction

1. Intrinsic factors

a. Defects of membrane: spherocytosis, elliptocytosis

b. Defects of hemoglobin

  • Structural anomaly: HbS

  • Synthesis anomaly: thalassemia


Etilogical classification of anemia ii
Etilogical Classification of Anemia (II)

c. Enzymatic defect: G6PD deficiency, pyruvate kinase

2. Extrinsic factors

a. Immune mechanisms: Rh, ABO incompatibility, autoimmune hemolytic anemia

b. non-immune mechanisms: infections


Etilogical classification of anemia iii
Etilogical Classification of Anemia (III)

C. Decreased production

1. Deficiency of substance: iron, Vit B12, folic acid

2. Mechanical interference: malignant replacement

3. BM failure

a. Primary: aplastic anemia

b. Secondary: renal, liver disease


Etiological classification of neonatal anemia
Etiological Classification of Neonatal Anemia

  • A.Blood loss-fetal to fetal, feto-maternal, traumatic delivery

  • B.Increased blood destruction-Rh, ABO or minor blood group incompatibility, enzymopathy, hemoglobinopathy a-thalassemia

  • C.Decreased production-pure red cell aplasia


Anemia historical factors
Anemia Historical Factors

  • Age-Neonatal period initial manifestation of hemolytic disease, 6 m-iron deficiency, b-thalassemia

  • Ethnic group-Thalassemia syndromes, G6PD def

  • Diet- documented sources of iron

  • Drugs- oxidant-induced hemolytic anemia, drug induced aplastic anemia

  • Inheritance-family history of anemia, jaundice, gall stones


Anemia physical findings
Anemia Physical Findings

Skin Hyperpigmentation Fanconi Anemia (FA)

Facies Frontal bossing Thalassemia Prominence malar Major

&maxillary bone

Eyes Microphthalmia FA

Hands Abnormal thumb FA

Spleen Enlargement Hemolytic anemia, infection, leukemia




Complete blood count
Complete Blood Count

  • Hemoglobin

  • MCV

  • WBC and differential count

  • PLT

  • RDW- red cell distribution width

  • CHr - hemoglobin concentration in reticulocytes


Microcytic anemias mcv 80fl
Microcytic AnemiasMCV<80fl

  • Iron deficiency anemia

  • Thalassemia syndromes

  • Chronic inflammation

  • Siderblastic anemias

  • Lead poisoning


Normocytic anemias mcv 80 90fl
Normocytic AnemiasMCV 80-90fl

  • Congenital hemolytic anemia

  • Acquired hemolytic anemia

  • Acute blood loss

  • Splenic pooling

  • Chronic disease


Macrocytic anemias mcv 90fl
Macrocytic AnemiasMCV>90fl

With megaloblastic bone marrow

  • Vitamin B12 deficiency

  • Folic acid deficiency

  • Hereditary orotic aciduria

    Without meglaoblastic bone marrow

  • Aplastic anemia

  • Pure red cell aplasia

  • Liver disease

  • Congenital Dyserythropoietic Anemia





Bone marrow biopsy
Bone Marrow Biopsy

Normal

Aplastic anemia





Distribution of iron in man
Distribution of Iron in Man

Cytochromes 3%

Myoglobin 10%

Ferritin & Hemosiderin 22%

Hemoglobin 65%








Prevention of nutritional iron deficiency anemia
Prevention of Nutritional Iron Deficiency Anemia Concentration

  • Encourage breast feeding for the first 6 months

  • Avoid cow’s milk at least for the first year of life

  • Iron fortified formula (12mg/l)

  • Solid food: cereals, meat

  • Oral iron 2mg/kg 4-12months

  • CBC: 9-12 months and 15-18 months


Iron doses for low birth weight infants starting at 1 month of age
Iron Doses for Low Birth Weight Infants Starting at 1 Month of Age

Iron Birth weight

mg/kg/day (g)

4 1000

3 1000-1500

2 1500-2500


The tragedy of iron deficiency during infancy and early childhood
of AgeThe tragedy of iron deficiency during infancy and early childhood”

  • Brain injury as a result of iron deficiency caused by improper nutrition

  • Iron deficiency affects mental development and motor functioning

  • Reduced activity of iron-containing enzymes in CNS, appear to be irreversible

    Buchanan G, J of Ped 135:413, 1999


Nutritional iron deficiency1
Nutritional Iron Deficiency of Age

  • No iron prophylaxis

  • No introduction of meat products

  • Increased tea consumption



Iron depletion
Iron Depletion of Age

  • Hb, MCV, RDW, CHr-Normal

  • SI, TIBC-Normal

  • Serum Ferritin- Low


Iron deficiency no anemia
Iron Deficiency – No Anemia of Age

  • Hb, MCV- Normal

  • RDW- High

  • CHr- Low

  • Serum Ferritin- Low

  • Serum Iron – Low

  • TIBC- High


Iron deficiency anemia
Iron Deficiency Anemia of Age

  • Hb-Low

  • MCV- Low

  • RDW- High

  • CHr –Low

  • Serum Iron –Low

  • TIBC – High

  • Serum Ferritin - Low


Iron deficiency biochemical markers
Iron Deficiency-Biochemical Markers of Age

  • Serum iron concentration-

    Influencedby iron absorption from meals, infection, inflammation and diurnal variation

  • Total iron-binding capacity (TIBC)-Increases in iron deficiency.

    Decrease in malnutrition, chromic infection and cancer.

  • Ferritin-Correlates with total iron stores.

    Acute phase reactant


Iron deficiency serum transferrin receptor
Iron Deficiency- Serum Transferrin Receptor of Age

  • Serum transferrin receptor- in iron deficiency there is increased number of receptors

    Unlike ferritin, increases in iron deficiency but not in chronic infection


Iron deficiency treatment
Iron Deficiency-Treatment of Age

  • Elemental iron 5-6mg/Kg/d

  • Reticulocytosis in one week

  • After 1 month the Hb should increase by at least 1gr%

  • Iron therapy continued 2-3 months after Hb returned to normal

  • No improvement after a month other cause for iron deficiency


Etiologic factors in iron deficiency 1
Etiologic Factors in Iron Deficiency (1) of Age

Increased physiologic requirements

  • Rapid growth

  • Menstruation

    Decreased iron assimilation

  • Iron-poor diet

  • Iron malabsorption: Celiac disease


Etiologic factors in iron deficiency 2
Etiologic Factors in Iron Deficiency (2) of Age

Blood loss

  • Gastrointestinal bleeding

  • Milk induced enteropathy

  • Peptic disease

  • Inflammatory bowel disease

  • Parasite bowel infection

    Hemoglobinuria due to prosthetic valve

    Idiopathic pulmonary hemosiderosis

    Intense exercise


Thalassemia syndromes hemoglobinopathies
Thalassemia Syndromes & Hemoglobinopathies of Age

  • -thalassemia

  • -thalassemia

  • Sickle cell anemia





B thalassemia location and type of mutations
b Disorders-thalassemia -Location and Type of Mutations


Clinical classification of b thalassemia
Clinical Classification of Disorders b-thalassemia

  • b-thalassemia trait

  • Homozygous b-thalassemia Thalassemia Major Thalassemia Intermedia



Differential diagnosis of microcytosis
Differential Diagnosis of Microcytosis Disorders

Iron deficiency Carriers of Anemia  Thalassemia

Serum Iron Low Normal

Transferrin High Normal

Ferritin Low Normal

Hemoglobin Normal High A2

electrophoresis


Thalassemia minor hplc hb electrophoresis
-thalassemia Minor – DisordersHPLC Hb Electrophoresis

Hb A


Thalassemia carrier detection
Disorders-thalassemia Carrier Detection

  • Microcytic anemia

  • MVC <78fl, MCH<27pg

  • HbA2>3.5%




Peripheral blood smear
Peripheral Blood Smear Disorders

Normal

Beta-thalassemia Homozygote


Homozygous thalassemia hb electrophoresis
Homozygous -thalassemia DisordersHb Electrophoresis

Hb F



Pathogenesis of b thalassemia major
Pathogenesis of Disordersb-thalassemia Major

Free excess of a-globin chains

Hemolysis

Ineffective erythropoiesis

Severe anemia

Skeletal deformities

Increased iron absorption



Clinical manifestations of iron overload
Clinical Manifestations of ErythropoiesisIron Overload

  • Cardiac: arrhythmias, CHF

  • Endocrine: growth failure, delayed sexual maturation, hypoparathyroidism, hypothyroidism, DM

  • Skin: bronze discoloration

  • Liver: cirrhosis


Important studies of Deferoxamine Therapy in ThalassemiaYear Finding1974 IM therapy stabilize hepatic iron 1978 12h portable infusion for iron balance 1981 Therapy reduces hepatic iron 1985 Reduction of cardiac disease in compliant patients 1989 Extended survival in young patients



Combination of l 1 and dfo
Combination of L Therapy in Thalassemia1and DFO

  • L1 not as powerful as DFO

  • Two chelators given on the same day have additive affect on urine iron loss


Bmt in thalassemia
BMT in Thalassemia Therapy in Thalassemia

Prognostic Criteria

  • Hepatomegaly

  • Liver fibrosis

  • Quality of iron chelation

    Prognostic Categories

  • Class I-none of the above

  • Class II One of the above

  • Class III two or three of the above


Btm class i
BTM Class I Therapy in Thalassemia


Prevention of thalassemia
Prevention of Therapy in Thalassemia-thalassemia

  • Carrier screening

  • Prenatal diagnosis

    CVS and DNA analysis

    Pre-implantation diagnosis (PGD)

    DNA extracted form fetal erythroblasts in maternal circulation


A thalassemia

a- Therapy in Thalassemiathalassemia


A globin cluster
a- Therapy in Thalassemiaglobin Cluster


A thalassemia abnormal hbs
Therapy in Thalassemiaa-thalassemia-Abnormal Hbs

22

22

Hb Bart’s

Hb H


Gene deletion in a thalassemia
Gene Deletion in Therapy in Thalassemiaa-thalassemia


Hydrops fetalis syndrome
Hydrops Fetalis Syndrome Therapy in Thalassemia

  • Most Hb- Hb Barts, unable to deliver O2 to tissues

  • Tissue hypoxia & anemia

    Massively enlarged palcenta

    Heart failure, edema anasarca

    Interferes with organogenesis, -congenital malformations

    Extramedullay erythropoiesis


Hydrops fetalis syndrome1
Hydrops Fetalis Syndrome Therapy in Thalassemia


Hemoglobin h disease
Hemoglobin H Disease Therapy in Thalassemia

  • Genotype --/-a

  • On cord blood: 10-20% Bart’s hemoglobin

  • Moderate microcytic anemia

  • Hb electrophoresis 5-30% Hb H


A thalassemina trait
a- Therapy in Thalassemiathalassemina Trait

  • Genotype: - -/aa, -a/-a

  • Hb electrophoresis on cord blood:

    2-10% Hb Bart’s

  • On adult blood: microcytic, with or without anemia

  • Diagnosis by exclusion of b-thalassemia minor & iron deficiency


A thalassemia silent carrier
a Therapy in Thalassemia-thalassemia Silent Carrier

  • -a/aa

  • Hb electrophoresis on cord blood:

    traces to 2% Hb Bart’s

  • No anemia or microcytosis on adult blood


Deletions in the a globin gene cluster
Deletions in the Therapy in Thalassemiaa-globin Gene Cluster


Categories of a thalassemia mutations
Categories of Therapy in Thalassemiaa-thalassemia Mutations


Non deletion a thalassemia mutations
Non-deletion Therapy in Thalassemiaa-thalassemia Mutations

a2

aNco

aHph

aTSaudi


A thalassemia genotype spectrum

a- Therapy in Thalassemiathal Trait

--/aa

-a/-a

aTa/aa

aTa/-a

Hb H Disease

--/-a

aTa/aTa

aTa/--

a-thalassemia Genotype-Spectrum


Strategy for a thalassemia multiplex pcr analysis
Strategy for Therapy in Thalassemiaa-thalassemia Multiplex PCR Analysis


Anemia of chronic infection

Anemia of Chronic Infection Therapy in Thalassemia


Anemia of chronic infection1
Anemia of Chronic Infection Therapy in Thalassemia

  • Serum Iron- Low

  • TIBC- Low

  • Serum ferritin- High

  • Reduced release of iron form macrophages and reduced intestinal iron absorption


Anemia of chronic disease
Anemia of Chronic Disease Therapy in Thalassemia


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