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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 Globin

Protoporphy
Iron
rin

Glucoronyl Indirect Bilirubin


Transferase (Fat Soluble)

Direct Bilirubin
(Water Soluble)
Indirect Bilirubin

Immature Hyperbilirubine
Glucoronyl mia Jaundice
Transferase (7 mg./100 ml)

Kirnecterus
(20 mg/100 ml)

Permanent
brain damage