Dr Heersh HMH Raof Saeed Hematology (lecture 1) Dr Heersh HMH Raof Saeed

Introduction: Blood is essential for maintenance of life 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: 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/or associated 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 on exertion, tachycardia, cardiac dilatation, and high-output heart failure

Anemias: Laboratory Studies Initial 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: Pallor Mild splnomegaly in 20-30% Fragile nails .koilonychias Tachypnea ,functional 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 ) Iron is available in the form of drops, syrup, tablets & injections. is 6 mg/kg/day (Vit. C ) …….. oral therapy ( 4-6 weeks after Hb values is normal ) 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

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