Dr Heersh HMH Raof Saeed. Component : Plasma. Blood cells Hb. level varies from one age to...
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Transcript of Dr Heersh HMH Raof Saeed. Component : Plasma. Blood cells Hb. level varies from one age to...
HEMATOLOGY (LECTURE 1)
Dr Heersh HMH Raof Saeed
Component :Plasma.Blood cells
Hb. level varies from one age to another. 55% at birth 30% by 2-3 months 35% at 1 year 40% at 3 years 45% at 15 years
Introduction:Blood is essential for maintenance of life
Introduction: cont.
R.B.C. 5.5 millions/ c.mm at birth 4.5 millions/ c.mm at 1 year 5 millions/ c.mm at 15 years
•W.B.C. 22000 cells / c.mm at birth 12000 cells / c.mm at 1 week – 1
year 8000 cells / c.mm at 4 years
Life span: R.B.C: 120 days W.B.C: 5-10 days Platelets: 10 days
Blood volume: 85ml/ kg at birth 75-80ml/kg from 2nd month
Anemias:
Definition: abnormally low R.B.C., Hb, or P.C.V.Aetiology: I_ Decreased or impaired production: A. Substrate deficiency
B. Depression of bone marrow: 1. Hypoplastic anemia: a. congenital; pure red cell anemia , Fanconi anemia b. Acquired 2. Bone marrow replacement
II_ Blood loss: III_ Excessive R.C. destruction ( hemolysis )
Anemias:
Physiologic adjustments to anemia include:cardiac output, oxygen ex traction shunting of blood the concentration of 2,3-
diphosphoglycerate (2,3-DPG) increases within the RBC.
“ shift to the right” erythropoietin (EPO)
Anemias:
History and Physical Examination
HISTORY: Important historical facts should include:• age, sex , race and
ethnicity , diet,medications, chronic diseases, infections,• travel, • A family history of anemia
and/orassociated difficulties such as splenomegaly , jaundice, or early-age onset of gallstones is
EXAMINATION: • pallor ,• sleepiness,• irritability , and decreased exercise
tolerance. • flow murmur is often present. weakness, tachypnea, shortness of breath onexertion, tachycardia, cardiac dilatation, and high-output heart failure
Anemias:
Laboratory StudiesInitial laboratory testing should include
hemoglobin, hematocrit, red cell indices white blood cell count and differential, platelet count, reticulocyte count, and
examination of the peripheral blood smear .additional laboratory studies is dictated
by the history , the physical, and the results of this initial testing
Iron deficiency anemia:
Iron metabolism: newborn contains _________0.5 gm of iron adult contains __________5 gm of iron (full term infant requires 0.8mg daily .)
In the first half of infancy, iron reserves, After 6 months of age, diet. As only 10% of dietary iron is absorbed Iron is transported in the plasma (ferric state) combined with
transferrin’’ , by which way iron can be delivered to the bone marrow , spleen & liver for storage or erythropoiesis
Iron is absorbed in the duodenum ( ferrous state) oxidized iron + apoferritin feriitin
(anemia reduces ferric state increase apofritin)
Iron deficiency anemia:
Aetiology : (most commonly between 6 & 24 months)
1. Inadequate supply of iron; Inadequate
stores of iron at birth:B- Inadequate intake of
iron.
2. Impaired iron absorption:
3. Increased demand for iron: A- Blood loss: B- increased demands for growth:
Iron deficiency anemia:
Clinical feature :
Symptoms:Pallor : g/dl)Lethargy irritablitty anorexia fatigue after
physical activity Pica (plumbism)
Signs:PallorMild splnomegaly in
20-30%Fragile
nails .koilonychias Tachypnea ,function
al murmur &rarely C.H.F in very sever cases
Atrophic glossitis & dysphagia .
Iron deficiency anemia:
Laboratory investigations: Hypochromic ,microcytic anemia with poikilocytosis & target cell in
the blood smear. Decreased PCV. Decreased Hb concentration. Slight reduction of R.B.C count. Decreased MCV. Decreased MCHC. Decreased MCH. Normal W.B.C, platelet & reticulocytes. Decreased S. ferritin to less than 10 microgm/lit. Decreased S. iron. Increased total iron binding capacity ( transferrin ) , with iron
saturation less than 16% . Bone marrow examination is rarely required, when performed; it
shows erythroid hyperplasia with predominance of normoblasts. Hemosiderosin is totally absent.
Differential diagnosis:1. Thalassaemia minor 2. Anemia of chronic infection.3. Lead poisoning ; basophilic stippling of
the R.C. together with elevated blood & urine lead levels are characteristic of lead poisoning
4. Pyridoxine deficiency.5. Sideroblastic anemia.
Iron deficiency anemia:
Iron deficiency anemia:
Treatment: (The regular response of iron-deficiency anemia to adequate amounts of iron is a critical diagnostic and therapeutic feature )
o Iron is available in the form of drops, syrup, tablets & injections.
o is 6 mg/kg/day (Vit. C ) …….. oral therapy ( 4-6 weeks after Hb values is normal )
o Parenteral iron :1. Poor iron absorption 2. When its difficult to compensate continuous iron loss as in hereditary telengeictasia.3. Intolerance of oral iron.4. Non-compliance of giving or taking iron
Iron deficiency anemia:
Blood transfusion is rarely indicated, in extremely severe cases- heart failure is about to occur, - correction of severe anemia prior to a necessary operation.Partial correction, in these situations,, is preferable.
Iron deficiency anemia:
Prevention:Breastfeeding should be encouraged,
with the addition of iron-fortified cereals after 4-6 m o of age
1mg/kg/day should be supplied from 3 months of age
I nfantswho are not breast-fed should only receive iron-fortified formula (12 mg of iron per liter) for the first year
Iron deficiency anemia:
Causes of failure to respond to oral iron:1- Wrong diagnosis.2- Non- compliance.3- Persistent hemorrhage.4- Impaired bone-marrow response as in presence of chronic infections, renal failure, or when B12 & folic acid deficiency co-exists.5- Impaired absorption.6- Unknown cause.
routine screening helps prevent the developm ent of severe anemia. Routine screening using hemoglobin or hematocrit is done at 12 m o of age, or earlier if at 4 m of age the child is assessed to be at high risk for iron deficiency
Sideroblastic anemia
Definition: Sideroblastic anemias result from acquired and hereditary disorders of hem e synthesis.▪ dimorphic population of erythrocytes▪ complete blood cell count indicates an
extremely high RBC distribution width (RDW)▪ impaired heme synthesis leads to retention of
iron within the▪ mitochondria. (aggregates of iron in
mitochondria) that have a perinuclear distribution. (sideroblasts)
▪ Hepato-splenomegaly may be present.
Sideroblastic anemia
Treatment: blood transfusions, iron chelating agents splenectomy may help
Thank you