Hematologic Disorders

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 Anemia Symptoms •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

Iron

Glucoronyl Transferase

Protoporphy rin

Indirect Bilirubin (Fat Soluble)

Direct Bilirubin (Water Soluble)

Globin

Indirect Bilirubin

Immature Glucoronyl Transferase

Hyperbilirubine mia (7 mg./100 ml)

Kirnecterus (20 mg/100 ml)

Permanent brain damage

Jaundice