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Hematological disorders

Hematological disorders. By : Dr. Sanjeev. Hematological disorders. Normal hematological levels varies with age and sex Anemia : - Hb level : 6 months to 6 years old : below 11 g /dL Older children : below 12 g /dL Severe anemia : below 5 g /dL Moderate anemia : 5 – 10 g /dL.

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Hematological disorders

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  1. Hematological disorders By : Dr. Sanjeev

  2. Hematological disorders • Normal hematological levels varies with age and sex Anemia : - Hb level : • 6 months to 6 years old : below 11 g /dL • Older children : below 12 g /dL Severe anemia : below 5 g /dL Moderate anemia : 5 – 10 g /dL

  3. Classification • According to the morphology of red cells or the etiological factors : MICROCYTIC HYPOCROMIC ANEMIA : 1. IRON DEFICIENCY ANEMIA A. During infancy 1. Nutritional 2. Post – hemorrhagic B . Older children 1. Post – hemorrhagic 2. Nutritional 2. INEFFECTIVE ERYTHROPOIESIS A. Thalassemia B. Pyridoxine responsive anemia C. Dyserythropoietic anemia D. Lead poisoning

  4. NORMOCYTIC NORMOCHROMIC ANEMIA 1. IMPAIRED CELL PRODUCTION ( reticulocyte count low ) A . Leukocytes and platelets normal 1. Physiological anemia of infancy 2. Infections 3. Pure red cell aplasia B . Leukocytes and platelets normal or decreased 1. Chronic renal and liver disease 2. Hypothyroidism C . Leukocytes and platelets reduced 1. Aplastic anemia – hereditary, idiopathic 2. Myeloproliferative disorders – leukemia 2. HEMOLYSIS ( reticulocyte count is high )

  5. 1. Megaloblastic erythropoiesis A. Nutritional 1. Vitamin B12 deficiency 2. Folate deficiency 3. Kwashiorkor B. Toxic 1. Therapy with antifolate compounds, methotrexate 2. Therapy with anticonvulsant , phenytoin 3. Malabsorption 2. Non – megaloblastic erythropoiesis A. Chronic hemolytic anemia: folate deficiency B. Liver disease C. Hypothyroidism MACROCYTIC ANEMIA

  6. Approach to a child with anemia Anemia (Hb less than normal level) - No lymph nodes - No hepatosplenomegaly - No petechiae or ecchymosis ----- Nutritional iron deficiency or megaloblastic ----- Pure red cell aplasia ----- Thalassemia trait ----- Lead poisoning ----- Renal disease

  7. Cont.. Anemia (Hb less than normal level) - No lymph nodes - No hepatosplenomegaly - With petechiae and ecchymosis ----- Aplastic anemia ----- Bleeding disorder ----- Coagulation disorder ----- ITP ----- DIC

  8. Cont.. Anemia (Hb less than normal level) With hepatosplenomegaly ----- Thalassemia ----- Liver disorders

  9. Cont.. Anemia (Hb less than normal level) With petechiae, lymphadenopathy and hepatosplenomegaly --- Leukamia --- Infections --- DIC

  10. Common causes of anemia during neonatal period : • Hemorrhage : Obstetric accidient, slipped umbilical cord tie, internal hemorrage • Hemolysis : G – 6 – phosphate dehydrogenate deficiency, pyruvate kinase deficiency, alpha thalassemia, malaria, DIC • Infections: Intrauterine (viral) or acquired (bacterial) • Impaired red cell production : Prematurity, small for date

  11. Physiological anemia of early infancy Hb concentration of cord --- 15 to 18 g/dL Causes : • 1. Diminished red cell production because of low erythropoietin levels in early infancy • 2. Increase in the blood volume in a rapidly growing infants and • 3. A shortened survival of the red cells Note : - It does not respond to iron or folic acid therapy. - If Hb level falls below 6 g/dL, a small blood transfusion is essential to correct it.

  12. Microcytic Hypochromic Anemia Iron deficiency anemia Iron helps to get enough oxygen. Body uses iron to make hemoglobin. Hemoglobin is a part of red blood cells. Hemoglobin carries oxygen. less iron ---- less red cell --- less HB --- less oxygen - Most common cause of anemia and usually results from blood loss. • RBCs tend to be microcytic and hypochromic, and iron stores are low as shown by low serum ferritin and low serum iron levels with high serum total iron binding capacity. • Common in rural area and in children from poor socioeconomic status

  13. Iron absorption • Site : - Iron is absorbed in the duodenum and upper jejunum. • It depends on : Extraluminal and intraluminal factors. • Extraluminal factors : iron absorption is controlled by the body stores of iron, rate of erythropoiesis and the iron needs of the body. • Intraluminal factors : iron absorption is regulated by the level of iron in the diet. Ferrous salts are better absorbed than the ferric salts. • Factors that inhibit : phosphates, calcium, milk and eggs, tannic acid (tea and coffee) • Factors that enhance : lactose, ascorbic acid, fruit juices and amino acids (cystine, lysineand histidine) • Hcl of the gastric juice facilitates ---- releasing iron from the ferric complexes (ferrous form)

  14. Mechanism of absorption Absorption occurs in two steps : 1. mucosal uptake and 2. mucosal cells to the plasma 1. mucosal uptake • Apoferritin (mucosal cells protein) • Ferritin (storage form of iron) Iron + apoferritin = ferritin Ferritin : • 1. iron delivered to plasma according to its needs. • 2. rest is deposited as ferritin in the mucosal cells • At the end of the life span of mucosal cells ferritin is sloughed out. When the iron absorption is : • Enhanced : iron entering directly to the plasma. • Depressed : trapped in the form of ferritin. • Small amount of iron may also be absorbed by the process of diffusion.

  15. 2. Mucosal cells to the plasma • Body iron needs are determined by plasma iron level or transferrin saturation. • Transferrin (glycoprotein) • Each molecule of tranferrin binds with 2 atoms of iron. This is called total iron binding capacity (TIBC). Iron + transferrin • Transferrin in the bone marrow provides iron for the developing red cells. • If saturation of transferrin is less than 20 % of the total capacity, iron is made rapidly available for the developing red cells in the bone marrow.

  16. Cellular uptake of iron and transferrin receptors • Present in : erythroid cells, placental and liver cells, etc. Iron + transferrin --------- release iron to the cell • Greatest numbers of transferrin receptors are present in the younger erythroid cells (reticulocytes). Stores of iron: • Reticuloendothelial cells (as ferritin) and bone marrow. • Red cells----- breakdown ----- iron liberated ------ taken up by R.E cells and iron is transferred to ferritin for reutilization • R.E system (principal source)

  17. Sequence of changes in iron deficiency • Iron stores (liver bone marrow) – diminished • Serum ferritin level – falls • Total iron – binding capacity ( below 15 %) – decrease • Free erythrocyte porphyrin (FEP) level – increases • Hemoglobin – decreased • MCV and MCH – decreased • Microcytic hypochromic picture

  18. Causes of iron deficiency Low iron stores : • If the birth weight of the infant is less (preterm, small for date) • In twins • If the cord was clamped early (as much as 80 – 100 mL of blood may remain in placenta) • Hemorrhage from cord, placenta • Malnutrition • Parasitic infestation and rapid growth.

  19. Reduced iron intake : Breast milk is better source of iron. Cow`s milk ------- poor source Excessive losses of iron may occur from the body through apparent or occult bleeding. Common causes : Hookworm infestation, Meckel`s diverticulum, Hiatus hernia, Prolapse rectum, Ulcerative colitis, Dysentery and Cephal hematoma Decreased iron absorption : Celiac disease Calcium salts and rich fibers in the vegeterian diet Increased iron demand : Premature and low birth weight infants Rapid growth during infancy and puberty Defective iron metabolism : Sideroblastic anemia Congenital transferrin deficiency, Iron is not utilized for erythropoiesis but stored in tissues Cont..

  20. Clinical features Symptoms : Fatigue and diminished capability to perform hard labor ------- lack of circulating HB --------- due to depletion of proteins that require iron as a part of their structure. • Due to deficiency or dysfunction of non – Hb proteins : - • Weakness , pica (eating no edible substance like mud, ice etc.) , dysphagia , altered resistance to infection, altered behavior. SIGNS On physical examination : • Pallor Abnormalities of epithelial tissues : • Koilonychia (spoon shaped nails) • Glossitis, angular stomatitis, tongue papillae atrophied • Splenomegaly and cardiac enlargement (systolic and even diastolic murmur): occurs with severe, persistent, untreated iron deficiency anemia. Note: There may be no symptoms if anemia is mild.

  21. Laboratory investigations • Hb levels ------ decreased • Hematocrit (packed cell volume or % of RBCs in whole blood) – decreased • Peripheral blood smear shows : poikilocytosis (variation in shape) and anisocytosis (variation in size), microcytic and hypocromic red cells. • MCV, MCH and MCHC ------ low • Reticulocytes --- decreased • Serum iron level --- less than 30 microgram/dL • Total iron binding capacity (TIBC)--- more than 350 microgram / dL and saturation of transferrin is less than 15 %. • Serum ferritin level --- decreased • Prussian blue staining of the marrow shows absence of hemosiderin.

  22. Cont.. Indicator of iron deficiency state : • Serum ferritin ------- less than 10ng /mL • If iron is less ------- protoporphyrin is not converted into heme --------- free erythrocyte porphyrin level in the blood is increased. • If ratio between free erythrocyte porphyrin and Hb is above 2.8 microgram / g indicates iron deficiency.

  23. Cont… • hematocrit (Ht or HCT) or packed cell volume (PCV) : - is the proportion of blood volume that is occupied by red blood cells • mean corpuscular volume, or "mean cell volume" (MCV) : -calculated by dividing the hematocrit by the red blood cell count (number of red blood cells per litre) • mean corpuscular hemoglobin, or "mean cell hemoglobin" (MCH) : -calculated by dividing the total mass of hemoglobin by the number of red blood cells in a volume of blood • mean corpuscular hemoglobin concentration, orMCHC : - calculated by dividing the hemoglobin by the hematocrit

  24. Treatment • Deworming of patients • Change in dietary habits • Wearing of shoes • Causes of persistent blood loss if any (polyps, ulcerative colitis etc.) need to be treated. Oral iron therapy : • Ferrous sulphate, ferrous fumarate, ferrous succinate, ferrous carbonate, ferrous lactate, ferrous gluconate. Dose : 3 – 6 mg /kg of body weight given orally in three divided doses for 6 – 8 weeks. Iron should be continued for another 6 - 12 months to replenish the body's iron stores in the bone marrow.

  25. Cont… • Vitamin C (improves iron absorption) • Milk (diminished iron absorption) .Iron should not be given just after the milk – feeds or after food. Causes of failure to oral iron therapy : • Inadequate dosage • Occult bleeding and continuous blood loss • Intolerance to iron • Malabsorption of iron • Wrong diagnosis

  26. Cont…. • Patients who cannot tolerate iron by mouth can take it through a vein (intravenous) or by an injection into the muscle. Indications : • Intolerance to oral iron • Chronic diarrhea • Bleeding from g.i.t which is aggravated by oral iron therapy and • Severe bleeding when Hb levels cannot be maintained with oral iron. Dosage of parenteral iron (iron dextran) : iron (mg) = wt (kg) x Hb deficit (g/dL) x 4 Site : - Deep intramuscular in the upper and outer quadrant of the buttocks I . V : 250 – 500 mL of saline infused slowly over 6 – 8 hours.

  27. Cont.. Blood transfusion : Indication : • Hb below 4 g / dL • Congestive heart failure • If associated infection prevents proper iron utilization. • Packed red cells should be used at a slow rate ( to prevent cardiac overload) • 1 or 2 doses of frusemide 1 – 2 mg/kg I.V (prevent circulatory overload)

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