ANEMIA

APLASTIC ANEMIA

DOSE RELATED
CHEMICAL SUBS
(MEDICATIONS)
IDIOSYNCRITIC
HEREDITARY
WAY
GENETIC
RADIATION
ALTERATION
ACQUIRED
/ENVIRONMENTAL
VIRUS

IDIOPATHIC
CAUSES 65%
 APLASTIC ANEMIA S/Sx

ANEMIA LEUKOPENIA THROMBOPENIA

FATIGUE, DOB,
TACHYPNEIC, FREQUENT & PETECHIAE,
TACHYCARDIC, PROLONGED, ECCHYMOSIS,NOSE
DIZZINESS, HA, PALE RECURRENT & GUM BLEEDING,
SKIN, THIN HAIR INFECTIONS PROLONGED BLEED
ETC
APLASTIC ANEMIA LAB RESULTS

CBC – LOW PLATELET

CBC – LOW RBC CT CBC – LOW WBC CT
CT

BONE MARROW
MCV - NORMAL MCH – NORMAL ASPIRATE –
HYPOPLASTIC/APLASTIC

MYELOID STEM CELL

YELLOW BONE SORROUNDED BY
RETICULOCYTOPENIA
MARROW IMMUNE CELLS
(LYMPHOCYTES)
 APLASTIC ANEMIA TREATMENT

YOUNG ADULT –
ALLOGENIC BONE >50 - ATG + BLOOD
MARROW CYCLOSPORIN TRANSFUSION
TRANSPLANT

TREATMENT OF
NEUTROPENIC REMOVAL OF
UNDERLYING
REGIMEN THYMUS
CAUSE
 Nursing Intervention
Neutropenic regime
– Provide well cooked meat, no fresh fruits
vegetables, no fresh flowers
– Limit visitors
– Wear mask
– Avoid crowded areas
– No bathing of pets
– Drink only pasteurized milk
 Nursing Intervention
Thrombocytopenia
• Do not take any medicine, including over-the-counter drugs
• To avoid aspirin and other drugs that contain aspirin
• Use a soft-bristle tooth brush. Do not use dental floss.
• Do not have dental work without your doctor’s approval.
• Do not do heavy lifting, contact sports, or strenuous exercise.
• Do not walk in bare feet.
• When shaving, do not use a razor blade. Use an electric razor instead.
• Be careful when using household tools, such as knives and scissors.
• Do not blow your nose forcefully.
• Do not wear tight clothing.
• Prevent constipation
• If you are a woman having your period, do not use tampons
 REVIEW OF INHERITANCE
PATTERN
INHERITANCE
PATTERN

CLASSICAL NON
MENDELIAN MENDELLIAN
PATTERN PATTERN

SEX
AUTOSOMAL CH
CHROMOSOME
1- 22
CH 23

DOMINANT X LINKED Y LINKED

RECESSIVE DOMINANT

RECESSIVE
REVIEW OF INHERITANCE
PATTERN
 REVIEW OF INHERITANCE
PATTERN
GENE LOCUS HOMOLOGOUS GENOTYPE

AUTOSOMAL
PHENOTYPE DOMINANT RECESSIVE
DOMINANT

AUTOSOMAL X LINKED X LINKED

RECESSIVE RECESSIVE DOMINANT
 ANEMIA DUE TO INCREASE
DESTRUCTION
HEMOLYTIC
ANEMIA CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

IMMUNE NON IMMUNE

INHERITED ACQUIRED
MEDIATED MEDIATED

SPLENOMEGALY/
MEMBRANE MECHANICAL
PNH ISO INFECTION SPLENIC
DEFECT CAUSES
SEQUESTRATION

CARDIAC
ENZYME DEFECT AUTO MECHANICAL
VALVE

DISSEMINATED
HGB DEFECT INTRAVASCULAR
DISSEMINATION

TTP

MARCH
HEMOGLOBINURIA
INTRAVASCULAR HEMOLYSIS
INCREASED
HYPERCELLULAR
ANEMIA RETICULOCYTE
BONE MARROW
COUNT

SLIGHT
HEMOGLOBINEMIA HEMOGLOBINURIA
MACROCYTOSIS

DECREASED OR
ABSENCE OF
HAPTOGLOBIN
EXTRAVASCULAR HEMOLYSIS
INCREASED
HYPERCELLULAR
ANEMIA RETICULOCYTE
BONE MARROR
COUNT

INCREASED MILD TO MODERATE

SLIGHT
UNCONJUGATED JAUNDICE – LEMON
MACROCYTOSIS
BILIRUBIN YELLOW COLOR SKIN

INCREASED
DARK COLORED
INCREASED LDH UROBILINOGEN IN
STOOL
URINE
 HEMOLYSIS – INTRACORPUSCULAR –
HEREDITARY -MEMBRANE DEFECT

HEREDITARY SPHEROCYTOSIS
RBC STRUCTURE
SPHEROCYTOSIS
 HEREDITARY SPHEROCYTOSIS
• Autosomal Dominant disease of the
Erythrocytes
• Mutations on the genes related to membrane
protein formation
• 200 – 300 per million in Northern Europe
• 1 in 5000 in United States
• 1° Extravascular and Intravascular Hemolysis
 H. SPHEROCTOSIS S/Sx

SPLENOMEGAL
ANEMIA
Y

PIGMENT
MILD JAUNDICE
STONES
H. SPHEROCTOSIS LAB RESULTS

LOW RBC LOW HB LOW HCT

LOW MCV NORMAL MCH HIGH MCHC

PRESENCE OF
HIGH
INCREASED RDW SPHEROCYTES OR
RETICULOCYTES
GLOBULAR RBC
H. SPHEROCTOSIS LAB RESULTS
H. SPHEROCYTOSIS TREATMENT

EXCHANGE
SPLENECTOMY
TRANSFUSION
H. SPHEROCYTOSIS COMPLICATIONS
1. APLASTIC BLOOD
PARVO VIRUS
CRISIS TRANSFUSION

EXCHANGE
2. HEMOLYTIC
ANY DSE - SPLEEN BLOOD
CRISIS
TRANSFUSION

3.
BILI STONE CHOLECYSTECTOMY
CHOLELITHIASIS
HEMOLYSIS – INTRACORPUSCULAR –
HEREDITARY - ENZYME DEFECT

G6PD DEFICIENCY
 GLUCOSE 6 PHOSPHATE
DEHYDROGENASE DEFICIENCY
• Sex linked recessive disorder
• Most common enzyme deficiency in humans
• Affects 400M people worldwide
• Africa – affects 20% of the population
• Mediterranian – 4%
• Southeast – 30%
• Intravascular Hemolysis ---- extravascular
PATHOPHYSIOLOGY
 GLUCOSE 6 PHOSPHATE
DEHYDROGENASE DEFICIENCY
 GLUCOSE 6 PHOSPHATE
DEHYDROGENASE DEFICIENCY S/Sx

S/Sx OF ANEMIA JAUDICE DARK COLORED URINE

KERNICTERUS DEATH
GLUCOSE 6 PHOSPHATE
DEHYDROGENASE - NBS
 GLUCOSE 6 PHOSPHATE
DEHYDROGENASE DEFICIENCY LAB
RESULTS
PRESENCE OF HEINZ
HIGH
HIGH LDH BODIES AND BITE
RETICULOCYTES
CELLS

HIGH BILIRUBIN LOW HAPTOGLOBIN HEMOGLOBINEMIA

ACUTE TUBULAR
HEMOGLOBINURIA
NECROSIS
 GLUCOSE 6 PHOSPHATE
DEHYDROGENASE DEFICIENCY
TREATMENT

PREVENT
OXIDATIVE INFECTIONS MEDICATIONS
STRESS/DAMAGE

MOTHBALLS OR
FOODS
NAPHTALINE
SOME MEDICATIONS THAT SHOULD
BE AVOIDED

CHLORAMPHENICOL FLUTAMIDE NALIDIXIC ACID

NITROFURANTOIN PRIMAQUINE SULFA DRUGS

CIPROFLOXACIN ASPIRIN PARACETAMOL

SOME FOODS THAT SHOULD BE
AVOIDED
FAVA BEANS ARTIFICIAL
AND OTHER MENTHOL BLUE FOOD
LEGUMES COLORING

GLUTEN FREE CHINESE

AMPALAYA
PRODUCTS HERBS

BLACK/GREEN
SOYA
TEA
 LIST OF ANTIOXIDANTS

SUNFLOWE
BERRIES WALNUT GINGER
R SEEDS

GRAPES ORANGE PINEAPPLE PRUNES

SPINACH TOMATOES GARLIS

 Nursing Actions
1. Encourage Newborn Screening
2. Educate the parents and significant others
about the condition
3. Avoid exposure to oxidative stress
4. Let the child understand the condition at
earliest possible age
5. Any signs and symptoms of acute hemolysis,
please consult doctor immediately.