Hematologic Disorders

• Iron Deficiency Anemia• Most common cause is due to excessive

bleeding and/or poor diet. Possible H. pylori infection

• High risk: pregnant women, adolescents, children, elderly, people with chronic blood loss

• Prevents proper hemoglobin formation• RBC’s small in size (hypocytic); pale

(hypochromic)• Infant will have difficulty forming RBC’s

• Incidence:• 9 months- 3 years old; adolescent girls when

menstruating• Daily intake of 6-15 gms of Iron per day

• Prevention:• Give iron fortified formula-1st year of life• Iron fortified cereals when breast fed• Give Iron supplements beginning at 2 months

of age

• Predisposing Factors:• Iron deficient mothers• Infants born with structural defects- (GER,

Pyloric stenosis)• Chronic diarrhea (poor absorption)

• Children older than 2 years old• Chronic blood loss due to GI tract lesions

(polyps, Ulcerative colitis, Crohn’s disease, parasitic infestations; frequent epistaxis)

• Pica (eating of inedible substances, ex. Dirt & paper)

• Iron Deficiency AnemiaSymptoms•Fatigue, weakness, and shortness of breath•Pale earlobes, palms, and conjunctivae•Spoon-shaped nails, sore tongue, dryness of

the epithelium in the corners of the mouth• Poor muscle tone; irritability

• Decreased hemoglobin (less than 11 gms/100 ml of blood)

• Hematocrit below 33%• Iron 30 microgram/100 ml. (Normal 70

microgram/100 ml.)

Pallor

Treatment:

• Treatment of underlying cause; rule out GI bleeding

• Diet rich in Iron, extra Vitamin C to enhance Iron absorption

• Ferrous sulfate – 4-6 weeks to replace Iron stores

Aplastic Anemia• Bone marrow hypoplasia or aplasia• Causes: acquired due to drugs

(chemotherapy), viruses, genetics, and neoplasia

• Symptoms: petechiae, ecchymosis, bleeding, infection, pancytopenia

• Due to depression of hematopoietic activity in the bone marrow

• Fanconi’s Syndrome (Congenital Aplastic anemia)

• Autosomal recessive trait• Skeletal/renal abnormalities• Hypogenitalism• Short stature

• Pancytopenia – reduction of all blood cell components

• Risk Factors:• Excessive radiation exposure• Drugs (chloramphenicol, sulfonamides,

arsenics, hydantoin, quinine, benzenes)• Chemotherapeutic drugs• Meningococcal pneumonia

• Assessment:• Pale, fatigues easily, anorexia• Excessive nosebleeds, GI bleeding• Cardiac decompensation (tachypnea,

tachycardia, (SOB)• Bone marrow samples – decreased

hematopoietic forms; blood forming spaces infiltrated by fatty tissues)

• Treatment:• Bone marrow transplantation• Antithymocyte globulin (ATG) & Cyclosporine• Testosterone – stimulate RBC growth• Transfusion of blood elements• Oral corticosteroid (Prednisone)• Stem cell transplant

• Hemophilia• Inherited interference with blood coagulation• Hemophilia A (Factor VIII deficiency)• Classic form of hemophilia• Transmitted as a sex-linked recessive trait

• Assessment:• Recognized in infants who bleeds excessively

after circumcision• Heavily bruising of lower extremities when

bumped• Soft tissue bleeding; hemorrhage in joints -

pain

• Important for the child to be identified of having hemophilia before surgery – fatal bleeding may result

• Lab tests:• Platelets/Prothrombin time normal• Clotting time either prolonged or normal• PTT – test that reveals low levels of Factor VIII

• Therapeutic Management:• Administration of Factor VIII• Supplied as fresh whole blood, fresh frozen

plasma• Best if supplied as a concentrate of Factor VIII• Sometimes children with inhibitors to Factor

VIII can be given Factor IX concentrate (Proplex)

• Von Willebrand’s Disease (Angiohemophilia)• Autosomal dominant disorder affecting both

sexes• Factor VIII deficiency & inability of platelets to

aggregate are the problems• Blood vessels can not constrict• Prolonged bleeding time

• Mucous membrane hemorrhage• Epistaxis a major problem – nose picking;

rubbing• Treatment:• Factor VIII replenishment• Administration of arginine desmopressin

(DDAVP) – vasoconstricting agent

• Christmas Disease (Hemophilia B, Factor IX deficiency)

• Sex linked recessive inheritance

• Treatment:• Factor IX concentrate (available for home

administration)

• Hemophilia C (Factor XI deficiency)• Hemophilia C or Plasma thromboplastin

antecedent deficiency)• Autosomal recessive trait affecting both sexes• Mild symptoms compared to patients with

Factor VIII or Factor IX deficiencies• Treatment:• Administration of arginine desmopressin

(DDAVP) – vasoconstricting agent, blood transfusion or plasma

• Hyperbilirubinemia• Occurs on the 2nd or 3rd day of life in about

50% of cases as a result of breakdown of fetal RBC.

• 7 mg/100 ml. –hyperbilirubinemia• If intestinal obstruction is present; intestinal

flora may break down bile into its basic components leading to the release of indirect bilirubin in the bloodstream

• Early feeding helps prevent build up of indirect bilirubin

• Serum bilirubin is obtained by heel puncture• If level rises above 10-12 mg/100 ml

treatment is considered• 20 mg/100 ml could interfere with the

chemical synthesis of brain cells, resulting to permanent brain damage called Kirnecterus

• Treatment for physiologic jaundice is rarely necessary except for early feeding (to speed passage of feces through the intestine & prevent reabsorption of bilirubin from the bowel)

• Phototherapy – exposure of infant to light to initiate maturation of liver enzymes

• Breastfed babies have a little difficulty in converting indirect bilirubin to direct bilirubin due to pregnanediol (metabolite of progesteron) – depresses the action of glucoronyl transferase

Breakdown of Fetal RBC

Heme Globin

Iron Protoporphyrin

Indirect Bilirubin(Fat Soluble)

Glucoronyl Transferase

Direct Bilirubin(Water Soluble)

Indirect Bilirubin

Immature Glucoronyl Transferase

Hyperbilirubinemia(7 mg./100 ml)

Kirnecterus(20 mg/100 ml)

Jaundice

Permanent brain damage