Blood diseases.

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• Blood diseases.
• Dr. Abed M. Al Hazmi.
• Associated prof. of pediatrics.
• King A/Azziz University Jeddah KSA.

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• Definition of ANEMIA
• Anemia is defined as reduction in red blood cell
  mass, below 2SD of the range of values for that
  age, and sex found in healthy person,
  i.e. (lt-2SD).

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Normal Values ( mean and lower limit of normal
(-2 SD)),for Hemoglobin, and Mean Corpuscular
Volume (MCV) at various Ages.

• Hemoglobin (g/dl) MCV (fl)
  Hematocrit ().
• Age Mean -2SD Mean -2SD Mean
  -2SD
• __________________________________________________
  __________________
• Cord blood 16.5 13.5 108 98 51 42.
• 1 WK 17.5 13.5 107 88 54 42.
• 2 WK 16.5 12.5 107 86 51 39.
• 1 mo 14.0 10.0 104 85 43 31.
• 2 mo 11.5 9.0 96 77 35 28.
• 3-6mo 11.5 9.5 91 74 35 29.
• 0.5-2yr 12.0 10.5 78 70 36 33.
• 2-6yr 12.5 11.5 81 75 37 34.
• 6-12YR 13.5 11.5 86 77 40 35.
• 12-18YR
• Female 14.0 12.0 90 78 41 36.
• Male 14.5 13.0 88 78 43 37.
• 18-49yr
• female 14.0 12.0 90 80 41 36.
• Male 15.5 13.5 90 80 47 41.

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• Classification of anemia
• A useful classification of the anemia is to
  divided anemia into three large groups by the RBC
  CELL Mean Corpuscular Volume (MCV).

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• Microcytic Macrocytic Normocytic
• MCV lt 76 MCV gt 96 MCV 76-96 femto.
• Iron deficiency Folate deficiency
  Hemolytic anemia's
• Thalassemia Vit. B12 Deficiency Anemia
  of chronic dis.
• Lead poisoning cong. Hypoplastic
  Acute blood loss
• Sideroblastic anemia anemia.(D.B.S)
  Aplastic anemia.
• Anemia of chronic Preleukemia. SCA. G6PD.
• Disease Liver disease Fanconi anemia.
• Copper deficiency Hypothyroidism Infiltrative
  process.
• Thiamine deficiency.

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Others Classifications of Anemia

• anemia may be classified into the following 3
  primary categories
• 1- Decreased red cell production
• 2- Increased red cell destruction (hemolysis)
• 3- Anemia due to blood loss.

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Clinical features of anemia

• NB When oxygen delivery by RBC to tissues is
  decrease the body responds by compensatory
  mechanism include
• 1- Increase cardiac output 2- Increase 2-3
  DPG.(diphosphoglycerate)
• 3- Increase EPO production 4- Shift of blood to
  vital organs.
• The clinical effects of anemia are influenced by
  4 major factors
• 1- The speed of onset 2- Severity
  of anemia.
• 3- Age of onset 4- The
  HGB O2 dissociation curve.

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• Symptoms
• 1- Shortness of breath (particularly on
  exercise).
• 2- Weakness.
• 3- Lethargy.
• 4- Anorexia.
• 5- Headache.
• 6- Heart failure.
• 7- Palpitation ( older children Adult ).

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• Signs
• General signs
• 1- Pallor (mucous membrane). Present when Hgb
  lt10g/dl.
• 2- Hyper dynamic circulation ( tachycardia,
  bounding pulses).
• 3- Features of heart failure.
• 4- Failure to thrive.
• Specific signs
• 1- Jaundice ( Hemolytic anemia ).
• 2- Leg ulcer ( SCA ).
• 3- Koilonychias ( spoon nail ).
• 4- Bone deformities ( Thalassemia).
• 5- Smooth red painful tongue ( vit. B12 Def. ).

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Anemia in the Neonate First three months of age

• Some types of anemia occur at birth or in early
  infancy. They are due to
• Physiological,
• Acquired,
• Inherited,

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• A two months old infant seen in ER, because of
  one day history of S.O.B. and cough.
• He is a product of S.V.D, full term pregnancy.
• A CBC was done showed
• WBC 15000/mm3.
• RBC 4.5 x 10 12/mm3.
• Hgb 8.2 g/dl.
• MCV 80 pf.
• Platelet 250000/mm3.
• What is your comment on this CBC ?
• What is your management ?

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Physiological anemia Anemia of prematurity

• Physiological anemia developed in full term baby
  by age of 6 12 wks with Hb ( 8.0 11 g/dl ).
• Anemia of prematurity developed preterm baby by
  age of 4 -6 wks with Hb level ( 4 10 g/dl ).
• Mechanisms of these anemia
• 1- Cessation of erythropoietin (EPO) production.
• 2- Shortened survival of fetal RBC.
• 3- Expansion of blood volume due to rapid wt.
  gain.
• 4- After birth the ratio of HbF/HbA decreased
  (2-3 DPG) increased, this result in increased O2
  delivery to tissue and decreased hypoxia which
  stimulate EPO production.

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• A two months old infant seen in ER, because of
  one day history of S.O.B. and cough.
• He is a product of S.V.D, full term pregnancy.
• A CBC was done showed
• WBC 15000/mm3.
• RBC 4.5 x 10 12/mm3.
• Hgb 8.2 g/dl.
• MCV 80 fl.
• Platelet 250000/mm3.
• What is your comment on this CBC ?
• What is your management ?

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• Clinical manifestations
• Pure physiological anemia of infancy almost
  always fully compensated with no symptoms or
  signs, unless there is/are other process
  associated e.g. neonatal infection, blood loss,
  hemolysis.
• Therapy
• Usually required no therapy other than ensuring
  that diet contains the essential nutrients for
  normal hematopoiesis as folic ac., iron.
• Transfusion when the baby is not growing
  properly, apneic spell, bradycardia, infections.
• Recombinant human EPO, plus iron folic ac. When
  blood transfusion is not possible.

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• Acquired anemia in the neonate
• Blood loss
• A- Obstetric as a cause of blood loss
• 1- placenta previa.
• 2- Abrupt placenta.
• 3- Hematoma of the umbilica cord.
• 4- Rupture of the umbilical cord.

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• B- Occult blood loss
• 1- Fetomaternal bleeding
• A- Placenta malformation.
• B- Obstetric procedure ( Traumatic amniocentesis,
  external or internal cephalic version, breech
  delivery ).
• C- Spontaneous.
• C- Fetoplacenta bleeding
• 1- Chorioangioma, Choriocarcinoma.
• 2- Cesarean section ( infant hold above
  placenta).
• 3- Tight nucal cord or occult cord prolapsegt

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This is the picture of twin deliveryWhat is
your description ?What are the complications in
the first second twin?
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• D- Twin Twin Transfusion.
• E- Hemolysis
• 1- Rh, ABO, minor blood gr. Incompatibility.
• 2- Infection, DIC, Vit. E deficiency.

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• Hereditary RC Disorders
• A- Metabolic defect as G6PD, Pyruvate kinase def.
• B- RBC membrane defect as spherocytosis,
  elleptocytosis, somatocytosis.
• Hemoglobinopathies
• A- Alfa and gamma thalassemia syndrome.
• B- Alfa and gamma chain structural abnormal.

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• Diminished RBC production
• A- Diamond Blackfan Syndrome.
• B- Congenital leukemia.
• C- Infection ( specially Rubella, Parvovirus).
• D- Osteopetrosis.

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Nutritional Anemia( due to deficiency of
substances essential for erythropoiesis)

• A- Iron deficiency anemia (very common).
• (Uncommon problem in childhood).
• B- Megaloblastic anemia due to deficiency of
  Vit B12 or folic acid.
• C- Anemia due to protein malnutrition
• D- Vit C deficiency anemia (associated with
  scurvy or pyridoxine deficiency).

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• Test name Result Refer. Range Unit.
• __________________________________________________
  _________
• WBC ( White blood Cell Count). 6.7 4.5 -
  11.5 K/ul
• RBC ( Red blood Cell Count ). 3.15 4.00
  6.00 M/ul
• .
• Hemoglobin ( Hb ). 8.5 12 16.
  g/dl
• Hematocrit ( HCT ). 30.00 36 54
  .
• Mean Corpuscular Volume) MCV. 60.00 70 80 fl.
• Mean Corpuscular Hb MCH 23.00 27
  32 pg.
• Mean Cor. Hb Conc. MCHC 28.00 32- 36 .
• RDW ( RBC distribution width ) 17.1 11.5
  14.5 .
• ( MCV/RBC ).

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Iron Deficiency Anemia( Is the most common
anemia in infancy childhood )

• Etiology
• 1- Inadequate intake of Iron
• A- Prolonged breast feeding. Rare before age of 6
  mon.
• B. Preterm baby ( low birth weight ) Unusual
  perinatal blood loss
• C- Poverty.
• 2- Blood loss
• A- Occult bleeding from GIT as Peptic ulcer,
  Meckel diverticulum's, Polyps, heamangioma,
  Inflammatory bowel disease, hookworm infest.
• B- In some areas occult blood loss from GIT due
  to consumption of whole cow's milk
  ( exposure to a heat-labile protein ).
• 3- Defective absorption of Iron
• A- Malapsorption syndrome.
• B- Excess phosphate phytate in diet e.g.
  cereals.
• C- Achlorhydria ( ).
• 4- Increase demand for iron
• A- Rapid growing premature and growing children.
• B- Menstruating females.
• C- Convalescence from disease.

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• Clinical picture
• 1- Pallor is the most important sign of anemia.
• 2- Compensatory mechanisms ( in mild moderate
  IDA Hb 6-10 g/dl).
• A- Increased level of 2,3 DPG ( diphospho
  glycerate ).
• B- Shift of the oxygen dissociation curve.
• 3- Irritability, pica( pagophagia), and anorexia
  in severe anemia Hb lt5 g/dl.
• 4- Iron deficiency with or without anemia have
  effects on neurologicl and intellectual function
  as
• attention span, alertness, and learning in both
  children adolescent.
• 5- Enlargement of the spleen in 10.
• 6- Angular stomatitis, red glazed tongue.
• 7- Nails are brittle, striated and loss their
  luster, in severe anemia there is spooning of
  nails.
• 8- Hemic mur. In severe anemia.

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• Investigations ( follow a sequence)
• Hb RBC are reduced. And disappearing of B.M.
  hemosiderin.
• S. Ferritin decreased.
• Iron-binding capacity of the serum (s.
  transferrin) Increased.
• WBC are normal
• Platelet Increased (in severe IDA Decreased).
• B.M. Hypercrllular with erythrioid hyperplasia.
• AS the deficiency of iron progress the RBC
• 1- Become small (Microcytic), Decrease their Hgb
  content (Hypochromic).
• 2- RBCs become deformed (Poikilocytosis).
• 3- Increase red cell distribution width (RDW).

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• Differential diagnosis
• IDA Should be differentiated from
• 1- Thalassemia Trait.
• 2- Lead poisoning.
• 3- Sideroblastic anemia.
• 4- Chronic diseases.
• 5- Copper deficiency.
• 6- Hemoglobin E.
• 7- Pyridoxine deficiency.

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• Differential diagnosis
• 1- Alfa Beta Thalassemia trait.
• Hb electrophoresis ( Level of Hb A2
  Hb F are increased ).
• 2- Lead poisoning ( level of lead
  increased ).
• 3- Thalassemia major Hemolytic
  manifestations.
• 4- Pyridoxin deficiency ( V.B6 level
  decrease ).
• 5- Chronic disease ( S.iron
  Iron-binding capacity are reduced but
• S. ferritin increased.

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• Treatment
• Oral administration of Ferrous salts ( Sulfate,
  Gluconate, Fumarate )
• 4-6 mg/kg of elemental iron in three
  divided doses.
• Iron dextran IM
• Vit C Increases absorption of Iron from food.
• Stop bleeding and treat parasitic infestation.
• Blood transfusion is indicated when
• 1- Severe anemia ( Hb lt 4g/dl).
• 2- Anemia associated with infection that may
  interfere with iron absorption.
• Family education and diet to prevent IDA in
  infancy.

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• Response to iron therapy
• 12 24 hr. replacement of intracellular iron
  enzymes ( dec. irritability, increase appetite ).
• 36 48 hr. Initial bone marrow response (
  erythroid hyperplasia ).
• 48 72. hr. Reticulocytosis.
• 4 - 30 days Increase in Hb ( 0.5g/dl/24 hr.
• 1 3 months Repletion of iron stores.

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Megaloblastic Anemia

• Vitamin B-12 Deficiency
• Requirements 1-2 mic.g/d.
• Sources Animal products ( liver, Kidney).
• Absorption Combined with IF absorbed in the
  terminal ileum.
• Functions Essential for hemopoiesis NS
  integrity.
• Storage 2 mg. are stored in the liver
  (sufficient for 2-4 yr.).

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• Causes of Vit.B-12 Deficiency
• A- Poor dietary intake. Rare in children (
  poverty Vegan).
• B- Gastric causes. Rare in children.
• 1- Pernicious anemia ( meanly in adult).
• 2-Congenital lack or abnormal intrinsic factor
  (IF).
• 3- Total or partial gastrectomy.

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• C- Intestinal causes. Is the usually causes in
  children.
• 1- Intestinal stagnant synd. Jejunal
  devirticulosis,
• blind- loop stricture, ect (B12 utilization
  by bacteria).
• 2- Chronic tropical sprue.
• 3- Ileal resection Cohn's disease.
• D- Congenital deficiency of Vt.B12 carrier
  proteins.
• Transcobalamin Def. ( Tarnscobalamin II ).
• E- Others (rare without clinical importance).
• Celiac dis. Sever pancreatitis, HIV infection.

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• Clinical manifestations The onset usually
  insidious.
• 1- Mild jaundice (due to ineffective
  erythropoiesis).
• 2- Glossitis ( a beefy-red, sore tongue).
• 3- Angular stomatitis.
• 4- Signs of malabsorption with wt. loss.
• 5- Purpura due to thrombocytopenia.
• 6-Widespread melanin pigmentation.
• 7- Vit. B12 Neuropathy (subacute combined
  degeneration of the cord).
• Affecting the sensory nerves and posterior
  lateral columns. It is symmetrical affecting the
  lower limbs more than the upper.
• Ataxia, Paresthesias, Hyporeflexia, Babinki
  Clonus .

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• Laboratory Findings
• 1- CBC Macrocytic red cell indices, evaluates
  for cytopenias.
• WBC and platelet may be moderately reduced.
• Reticulocyte count is low in relation to the
  degree of the anemia.
• 2- Bone marrow Should be consider for any child
  with more than one abnormal cell line on the CBC.
• 3- Serum B12 level.

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• Treatment
• Initial dose IM.
• 10-50 mcg/d for 5-10 d, followed by 100-250
  mcg/dose q2-4 wk.
• OR. 1mg/dose IM .
• If there is evidence of neurological involvement
• 1mg/d IM for 2 wks.
• Maintenance 1 mg IM monthly for life.

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• Folic Ac. Deficiency
• Requirement 100 200 mic.g/d.
• Sources Greens vegetables, fruits,
  liver.
• Absorptions Small intestine.
• Functions DNA synthesis, and a.a.
  interconversions.

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• Causes of Folate Deficiency
• 1- Inadequate dietary intake
• 2- Inadequate absorption
• 3- Antifolate medications (e.g Methiotraxate)
• 4- Medications that impair absorption (eg. Ant
  convulsions).
• 5- Increased use ( eg, Chronic hemolysis such as
  SCA).

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• Clinical manifestations
• Onset 4-7 months of age the infant usually of low
  birth wt. 0r significantly underweight (
  marasmus, or kwashiorkor ).
• They present with
• 1- Megaloblastic Anemia.
• 2- Irritability.
• 3- Failure to gain wt.
• 4-Chronic diarrhea.
• 5- Hemorrhage due to thrombocytopenia.

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• Laboratory Findings
• 1- CBC Macrocytic red cell indices, evaluates
  for cytopenias.
• WBC and platelet may be moderately reduced.
• Reticulocyte count is low in relation to the
  degree of the anemia.
• 2- Bone marrow Should be consider for any child
  with more than one abnormal cell line on the CBC.
• Red blood cell folate level (The best test for
  metabolically active folate).
• Serum Folate measures the circulating pool.

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• Treatment
• Folic ac. 1-5 mg/d for 3-4 wks. Orally.

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