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Basic principles
Anemia is a in RBC number or Hb concentration to a level that is more
than two standard deviations below the mean (for the age and gender group)
Hb is at birth. Declines and reaches nadir in 2 3 mo of age. Reach adult
levels after puberty
HbF gradually and virtually disappears by 6 9 mo of age
20% of all children in the USA and 80% of children in developing nations
have anemia at some time during childhood
Presentation:
Weakness, fatigue, dyspnea
Pale conjunctiva and skin
Headache and lightheadedness
Angina (with preexisting coronary disease)
Normal Hb levels in adults:
: 13.5 17.5 g/dL
: 12.5 16.0 g/dL
Normal mean corpuscular value (MCV) is 80 100 m
MCV < 80 m microcytic anemia
MCV > 100 m macrocytic anemia
CLASSIFICATION OF ANEMIAS
1. MICROCYTIC ANEMIAS
Basic principles
Anemia with MCV < 80 m
Hb Erythroblast performs extra division smaller RBCs (to maintain
Hb concentration)
Hemoglobin = Heme (protoporphyrin + iron) + Globin
Microcytic anemias may be due to:
iron (iron deficiency anemia, anemia of chronic disease, chronic
blood loss)
protoporphyrin (sideroblastic anemia)
globin (alpha and thalassemias)
1.1 IRON DEFICIENCY ANEMIA
Most common type of anemia
iron heme Hb microcytic anemia
Iron is the most common nutritional deficiency worldwide (affects 1/3 of the
world population)
Iron absorption:
Heme transporter and non-heme (DMT1) (lumen to enterocyte)
Ferroportin (enterocyte to blood) Transferrin (blood to liver and bone
marrow macrophages) Ferritin (stored iron within macrophages).
Labs (stages of iron deficiency anemia):
Stored iron is used, so Ferritin and TIBC (total iron binding
capacity; transferrin levels in blood)
Serum iron depleted serum iron and % saturation (< 33%);
FEP
Normocytic anemia (normal, but fewer RBCs)
Microcytic, hypochromic anemia; RDW
Causes of iron deficiency:
Infants: breast-feeding iron in human milk is easily absorbed, but
far from enough after 6 mo
Children: poor diet. Cow milk microhemorrhages.
Adults: peptic ulcer disease in and menorrhagia or pregnancy In
Elderly: colon polyps / carcinoma, hookworm (Ancylostoma
duodenale and Necator americanus)
Other causes malnutrition, malabsorption, and gastrectomy (acid
iron absorption by maintaining it in the ferrous state Fe+)
Clinical features of iron deficiency:
Anemia
Koilonychia (older children and adults; spoon-shaped nails)
Pica (craving for non-nutritive items)
Geophagia: vit B12 from bacteria in the soil; oligoelements
(zinc, calcium, copper, magnesium, iron)
Pagophagia: cold may help with glossitis and stomatitis
associated with iron deficiency
Tx
Ferrous sulfate (300mg/day)
Vitamin C ( intestinal absorption)
1.4 THALASSEMIA
globin (alpha or ) Hb microcytic anemia
Inherited mutation. Carriers are protected against Falciparum malaria
Normal types of Hb:
-Thalassemia
4 genes are present on chromosome 16. Thalassemia occurs due
to deletion of one or more genes.
1 gene deleted asymptomatic
2 genes deleted mild anemia with RBC count
Cis Asians; 2 deletions occur in the same chromosome;
risk of severe thalassemia in offspring
Trans Africans; 1 deletion in each chromosome
3 genes deleted severe anemia, chains form tetramers (HbH)
that damage RBCs
4 genes deleted lethal in utero; chains form tetramers (Hb Barts,
binds strongly to O2 and doesnt let it go); hydrops fetalis:
oxygen delivery to tissues (hypoxia) + extramedullary
hematopoiesis (impairs liver function) tachycardia (in response
to hypoxia) + protein synthesis in the liver renal perfusion
(due to severe tachycardia impairing diastolic filling of the
ventricles and thus fucking up the cardiac output) activation
of RAAS fluid retention + plasmatic proteins hydrops
fetalis
-Thalassemia
-Thalassemia minor:
Usually asymptomatic, with an RBC count
Microcytic, hypochromic RBCs and target cells are seen on
blood smear
Hb electrophoresis shows slightly HbA with HbA2 (5%;
normal 2.5%) and HbF (2%; normal 1%)
-Thalassemia major:
Presents with severe anemia a few months after birth; high HbF
at birth is temporarily protective
tetramers aggregate and damage RBCs ineffective
erythropoiesis and extravascular hemolysis (removal of
circulating RBCs by splenic macrophages)
Massive erythroid hyperplasia:
-
REFERNCIAS
Baker RD, Greer FR, Committee on Nutrition American Academy of Pediatrics.
Diagnosis and prevention of iron deficiency and iron-deficiency anemia in
infants and young children (0-3 years of age). Pediatrics 2010; 126:1040.
Oski FA. Iron deficiency in infancy and childhood. N Engl J Med 1993; 329:190.
Finberg KE. Iron-refractory iron deficiency anemia. Semin Hematol 2009; 46:378.