approach to childhood anemia
Download
Skip this Video
Download Presentation
Approach to Childhood Anemia

Loading in 2 Seconds...

play fullscreen
1 / 105

Approach to Childhood Anemia - PowerPoint PPT Presentation


  • 171 Views
  • Uploaded on

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.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' Approach to Childhood Anemia' - brone


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
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
  • 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
“The 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
  • No iron prophylaxis
  • No introduction of meat products
  • Increased tea consumption
iron depletion
Iron Depletion
  • Hb, MCV, RDW, CHr-Normal
  • SI, TIBC-Normal
  • Serum Ferritin- Low
iron deficiency no anemia
Iron Deficiency – No Anemia
  • Hb, MCV- Normal
  • RDW- High
  • CHr- Low
  • Serum Ferritin- Low
  • Serum Iron – Low
  • TIBC- High
iron deficiency anemia
Iron Deficiency Anemia
  • Hb-Low
  • MCV- Low
  • RDW- High
  • CHr –Low
  • Serum Iron –Low
  • TIBC – High
  • Serum Ferritin - Low
iron deficiency biochemical markers
Iron Deficiency-Biochemical Markers
  • 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
  • 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
  • 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)

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)

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
  • -thalassemia
  • -thalassemia
  • Sickle cell anemia
clinical classification of b thalassemia
Clinical Classification of b-thalassemia
  • b-thalassemia trait
  • Homozygous b-thalassemia Thalassemia Major Thalassemia Intermedia
differential diagnosis of microcytosis
Differential Diagnosis of Microcytosis

Iron deficiency Carriers of Anemia  Thalassemia

Serum Iron Low Normal

Transferrin High Normal

Ferritin Low Normal

Hemoglobin Normal High A2

electrophoresis

thalassemia carrier detection
-thalassemia Carrier Detection
  • Microcytic anemia
  • MVC <78fl, MCH<27pg
  • HbA2>3.5%
peripheral blood smear
Peripheral Blood Smear

Normal

Beta-thalassemia Homozygote

pathogenesis of b thalassemia major
Pathogenesis of b-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 Iron Overload
  • Cardiac: arrhythmias, CHF
  • Endocrine: growth failure, delayed sexual maturation, hypoparathyroidism, hypothyroidism, DM
  • Skin: bronze discoloration
  • Liver: cirrhosis
slide79

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 L1and 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

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
prevention of thalassemia
Prevention of -thalassemia
  • Carrier screening
  • Prenatal diagnosis

CVS and DNA analysis

Pre-implantation diagnosis (PGD)

DNA extracted form fetal erythroblasts in maternal circulation

a thalassemia abnormal hbs
a-thalassemia-Abnormal Hbs

22

22

Hb Bart’s

Hb H

hydrops fetalis syndrome
Hydrops Fetalis Syndrome
  • 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

hemoglobin h disease
Hemoglobin H Disease
  • Genotype --/-a
  • On cord blood: 10-20% Bart’s hemoglobin
  • Moderate microcytic anemia
  • Hb electrophoresis 5-30% Hb H
a thalassemina trait
a-thalassemina 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-thalassemia Silent Carrier
  • -a/aa
  • Hb electrophoresis on cord blood:

traces to 2% Hb Bart’s

  • No anemia or microcytosis on adult blood
a thalassemia genotype spectrum
a-thal Trait

--/aa

-a/-a

aTa/aa

aTa/-a

Hb H Disease

--/-a

aTa/aTa

aTa/--

a-thalassemia Genotype-Spectrum
anemia of chronic infection1
Anemia of Chronic Infection
  • Serum Iron- Low
  • TIBC- Low
  • Serum ferritin- High
  • Reduced release of iron form macrophages and reduced intestinal iron absorption
ad