Transcript Iron deficiency anaemia - KSU Faculty Member websites

Appraoch to a child with
anaemia
Dr. Pushpa Raj Sharma
Professor of Child
Health
Institute of Medicine
13.11.2060
Definition of anaemia
• The condition of having too few red blood
cells.
• It is a reduction of the red cell volume or
hemoglobin concentration below -2SD for
age, race and sex.
• Microscopic:
normocytic,microcytic,normochromic
macrocytic or specific abnormalities
(spherocyte,sickle cell,target cell)
Case
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Nine months old “Rai” female, single child
Recurrent fever
Mass in abdomen with pallor
No history of rash, bleeding, persistent
diarrhoea, drug, jaundice, pica,
Had blood transfusion 2 days back
No consanguity of marriage
No family history of gall stones, jaundice.
No loss of weight, exclusive breast feeding
Summary of examination
finding
• 9 Kg.
• Mild pallor
• No purpuric rash, lymphadenopathy,
abnormal facies,
• Gross spleenomegaly
Diagnostic Approach-History
• Age : Iron def rare without blood loss
before 6mo in term infants.
• Family Hist & Genetics:
(1)X-linked: G6PD def
(2)Aut dominant: Spherocytosis
(3)Aut recessive: Sickle cell,Fanconi
anemia
(4)Family member with early age of
cholecystectomy/splenectomy
(5)Ethnicity: Thalassemia; G6PD def
Diagnostic Approach-History
• Diarrhoea:
-Malabsorption of VitB12/E/Fe.
Inflammatory bowel disease and anemia of
chronic disease with or without blood loss.
-Milk protein intolerance induced blood loss
-Intestinal resection: Vit B12 def
• Infection:
- Giardia: iron malabsorption
-Intestinal bacterial overgrowth: VitB12def
-EBV,CMV,Parvovirus: BM suppression
-Mycoplasma,Malaria: hemolysis
-Hepatitis: aplastic anaemia
-Endocarditis, HIV
Diagnostic Approach-History
• Nutrition:
(1)Cows milk diet:iron def.
(2)Strict vegetarian:Vit B12 def.
(3)Goats milk: Folate def.
(4)Pica: Plumbism,Iron def.
(5)Cholestasis,malabsorption:VitE def
• Drugs: (1)G6PD:oxidants(sulfa, primaquine, henna)
(2)Immune mediated hemolysis (penicillin)
(3)Bone marrow suppression (chemotherapy)
• (4)Phenytoin increase folate requirement
Physical exam reveals presence
and potential causes of anaemia
• Fever-acute infection,intravascular disease,collagen
vascular disease
• Jaundice suggests hemolysis
• Petechia&Purpura—bleeding tendency
• Hypertension&oedema-renal disease
• Hepatosplenomegaly and lymphadenopathy—
infiltrative disease
• Growth failure or poor wt. gain—Anemia of chronic
disease or organ failure
• Examine stool for blood; urine for hemoglobinuria
Physical Findings in Anaemia
• Skin:Hyperpigmentation,café au lait spotsFanconi anemia
-Jaundice-hemolysis
-Petechia&purpura- BMinfiltration,
autoimmune hemolysis&thrombocytopenia
-Erythematous rash- Parvovirus,EBvirus
-Butterfly rash-SLE; Vitiligo-VitB12def
• Head:Frontal bossing-Thalassemia major
-Microcephaly-Fanconi anemia
Physical Findings in Anaemia
– Eyes: Microphthalmia-Fanconi anemia
-Retinopathy-Sickle cell disease
-Optic atrophy-Osteopetrosis
-KF ring-Wilson disease
– Ears: Deafness-Osteopetrosis
– Mouth: Glossitis-B12 def,iron def
-Angular stomatitis-Iron def
-Pigmentation-Peutz Jeghers syndrome
-Telangiectasia-Osler Weber Rendu
syndrome
Physical Findings in Anaemia
• Chest: Cardiac murmurEndocarditis,prosthetic valve hemolysis
• Abdomen: Hepatomegaly-hemolysis,
infiltrative tumour,chronic disease,
hemangioma,cholecystitis
-Splenomegaly -hemolysis,sickle cell
disease,thalassemia,malaria,EBvirus, portal
hypertension
-Kidney anomaly-pelvic/absent kidney
Physical Findings in Anaemia
• Extremities:Absent thumb-Fanconi
anemia -Spoon nails-Iron deficiency
-Dystrophic nails-Dyskeratosis
congenita
• CNS-Irritable,apathy-Iron def.
-Peripheral neuropathy-lead poisoning
-Ataxia,post.column signs-Vit B12def
- Stroke-Sickle cell anemia
• Short stature-Fanconi anemia,
Malnutrition
COMPLETE BLOOD COUNT
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Hb Conc
(g/dl)
Hematocrit(PCV)
MCV
(fl)
MCH
(pg)
MCHC (detects red cell dehydration)
RBC Count
(…x10 )
WbC Count
(…x10 )
Platelet Count
(…x10 )
Reticulocyte Count ( % )
Normal values
AGE
Hgb
Mean/ (-2SD)
HCT%
Mean/ (-2SD)
MCV
Mean/ (-2SD)
Newborn
16.5 (13.5)
51 (42)
108 (96)
1 Month
13.9 (10.7)
44 (33)
101 (91)
2 Months
11.2 (9.4)
35 (28)
95 (84)
6 Months
12.6 (11.0)
36 (31)
76 (68)
> 6 Months
12.5 (11.0)
36 (33)
Adult
Male
Female
15.5 (13.5)
14.0 (12.0)
47 (40)
41 (36)
81 (70+ age per yr)
90 (80)
90 (80)
Harriet Lane Handbook, The John Hopkins Hospital,15th edition
Reticulocyte Production Index
• RPI corrects the retics for the degree of
anaemia
• RPI indicates whether bone marrow is
responding appropriately to anaemia
• RPI= Retic x Hb(o) x 0.5 divided by Hb(n)
• RPI > 3 increased production (hemolysis or
blood loss)
• RPI < 2 decreased production or
ineffective production for the degree of
anaemia
• Reticulocytopenia—acute onset of anaemia,
antibody mediated destruction, BMdisease
AETIOLOGY
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(1) Inadequate response RPI < 2
A. Hypochromic microcyctic
B. Normochromic Normocytic
C. Macrocytic
(2)Adequate response RPI > 3 R/O
blood loss---Includes Hemolytic
disorders
Microcytic Anaemia
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TEST
Iron def ThalMin
S.Iron
- low
normal
S.Ferritin
- low
N/H
Marrow iron - low
N/H
Hb A2or F - N
H=Bthal
N=Athal
MCV ÷ RBC - >13
< 13
Sickle/B-thal – Hb S > Hb A
Absence of microcytosis in both parents excludes
B-thal or Sickle/B-thal but not A-thal
Macrocytic anaemia
• Vit.B12 def.- (1) pernicious anaemia
(2) ileal resection (3) abnomal
intestinal transport
• Folate def.- (1) malnutrition
(2) malabsorption (3) chronic
hemolysis (4)drugs - phenytoin,
sulfa
• Hypothyroidism
• Chronic liver disease
• Marrow failure-Fanconi
anaemia,Aplastic A
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Haemolytic anaemia
• Hemoglobinopathy– Hb
SS,SC,S-B thal
• Enzymopathy--G6PD def, PK
def
• Membranopathy—Hereditary
spherocytosis, elliptocytosis
• Extrinsic factors—
DIC,HUS,Abetalipoproteinemia,
Wilsons disease,Vit E def
• Immune hemolytic anaemiaAutoimmune,Isoimmune,Drug
induced
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THALASSEMIA-Lab
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Thal trait:Hb 9-10 g/dl
HbH disease:Hb 6-7 g/dl
Thal intermedia:Hb 7-8 g/dl
Thal major:Hb less than 5 g/dl
Peripheral smear:hypochromic,microcytic,
anisopoikilocytosis,target cells
• Hb electrophoresis: (1)Thal trait-HbF 15%, HbA2 3.5-8%,rest HbA
(2)Thal
major- HbF 20-100%,HbA2 2-7%,HbA 060%
Pure Red Cell Aplasia:
Reticulocytopenia + Absent marrow erythroid precursors
Congenital
(Blackfan-Diamond)
- 1st year of life
- congenital anomalies
- defective erythroid stem cell
- high MCV
- treatment: prednisone
blood transfusion
- life long
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Acquired
(Transient Erythroblastopenia of
Childhood-TEC)
- healthy child
- mean age dx: 25 mo.
- serum inhibitor of erythropoiesis (?
virus)
- normocytic
- treatment: transfusion PRBC’s (no
steroids)
- recovery is rule
Aplastic anaemia
• Severe reduction
in platelets and
granulocyte
series
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Anaemia of chronic disease
Normal morphology
Chronic inflammatory disease—
(1)infection (2)collagen vascular disease
(3)inflammatory bowel disease
Recent blood loss
Malignancy/Marrow infiltration
Chronic renal failure
Transient erythroblastopenia of chidhood
Marrow aplasia/hypoplasia
HIV infection
Hemophagocytic syndrome
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Haemolytic anaemia
• Polychromasia
(Wright-Geimsa).
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Leukaemias
• Blast cells
13.11.2060
Laboratory Evaluation
• Hematology:Complete Blood Count, Retic
count,Peripheral smear,ESR,G6PD
Sickling(+/-inf),Hb electrophoresis,Group,DCT,
Osmotic fragility test,BMaspiration
• Biochemistry:LFT,UE,RFT,S.Ferrtin,
S.Haptoglobin,Iron,VitB12,Folate,Ceruloplasmin
• Serology:Heterophil antibody,ANA,Viral t
• Urinalysis,microscopy,culture/sensitivity
• Stool exam.for ova,parasites,occult blood
• Endoscopy: upper and lower bowel
• Imaging:US Abdomen,Skeletal radiographs, Tc
pertechnetate scan for Meckels diverticulum
• Tissue biopsy:skin,lymph node,liver
•Nine months old female, single
child
•Recurrent fever
•Mass in abdomen with pallor
•No rash, bleeding, persistent
diarrhoea, drug
•Had blood transfusion 2 days
back
•No consanguity of marriage
•No loss of weight, exclusive
breast feeding
•9 Kg.
•Mild pallor Hb:9.4 g%
•No purpuric rash, lymphadenopathy, abnormal facies,
•Gross spleenomegaly
Blood report:
TLC: 9,600/ cmm: P: 44%: L: 56%;
Platelets: 30.000/cmm Anisocytosis, hypochromic,
reticulocyte 0.8%, occasional NRC.
The diagnosis
• Reticulocyte production index
= Retic x Hb(o) x 0.5 divided by Hb(n) = 0.3
RPI < 2 decreased production or ineffective
production for the degree of anaemia
– Metabolic
– Malignancy
– Further work up:
– Bone marrow
Bone marrow report
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Normal values
Cell Type
Range
%Myeloblasts
0-2
Promyelocytes
2-5
Myelocytes (neutrophilic)9-16
Metamyelocytes
7-23
Band forms
8-15
Neutrophils
4-10
Myelocytes (eosinophilic)0-2
Band
0-2
Mature
0-3
Monocytes/macrophages0-3
Basophils
0-1
Mast cell
0-2
Plasma cells
3-6
This patient
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Cell Type
Blast
Promyelocyte
Myelocyte
Metamyelocyte
Neutrophils
%
10
1.5
12
18
31
Final diagnosis
• Malignancy
– Juvenile Chronic Myelogenous Leukemia
The commonest cause
• Iron def. is common in children 9mo-3yr
• Iron def. anemia in a child over 3yr should
prompt consideration of occult blood loss.
• Infants less than 6months generally do
not develop iron def.; the exception to
this rule is premature infants,who are at
risk of iron def.at 4mo, if iron
supplementation is not given.
Iron deficiency Anemia
– Dietary iron def is the usual cause
– Manifestations of anemia
– CNS
abnormalities:apathy,irritability,poor
conc,cognitive deficits
– Poor muscle endurance
– GIT dysfunction
– Impaired WBC and T-cell function
Iron deficiency: hypochromic
anaemia
• Iron def.-(1)chronic
blood loss (2)poor
diet (3)cows milk
protein intolerance
• Chronic inflammatory
disease
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Differential Features of Iron Deficiency vs
Acute and Chronic Inflammation
Iron Deficiency
Anemia
Red Cell Indices
Blood Smear
Serum Iron
TIBC
Transferrin Saturation
S. Ferritin
B.M. Iron Stores
MCV,  MCH
Microcytic,
hypochromic
Decreased
Increased
Decrease (<7%)
Decreased (<12
mg/ml)
Acute and Chronic
Inflammation
MCV: N or , MCHC:
N or 
Normocytic/microcytic
Decreased
Normal or decreased
Decreased (<16%)
Normal or increased
Normal or increased
Iron deficiency Anemia
• Treatment:Response to oral iron includes
24-48hr-subjective improvement inCNS
48-72hr-reticulocytosis
4-30days-increase in Hb
1-3 mo-repletion of iron stores
• Therapeutic dose:3-6 mg/Kg/day of
elemental iron.---Induces an increase in Hb
of 0.25-0.4 g/dl per day or 1%/day rise in
hematocrit.
• Failure of response after 2 weeks of oral
iron requires reevaluation for ongoing
blood losses,infection,poor compliance or
other causes of microcytic anaemia.