hematologic disorders
TRANSCRIPT
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