ANEMIA DUE TO

INCREASED RED
CELL DESTRUCTION
__________
EDNA U. ROBLES, RN MAN
St. Luke’s College of Nursing
DIFFERENT TYPES OF ANEMIA

Decreased Red
cell production

Increased Red
ANEMIA cell
destruction

Blood loss
 REVIEW OF INHERITANCE
PATTERN
INHERITANCE
PATTERN

CLASSICAL NON
MENDELIAN MENDELLIAN
PATTERN PATTERN

SEX
AUTOSOMAL CH
CHROMOSOME
CH 1- 22
CH 23

DOMINANT X LINKED
X
Y LINKED

RECESSIVE DOMINANT

RECESSIVE
REVIEW OF INHERITANCE
PATTERN
 REVIEW OF INHERITANCE
PATTERN
GENOTYPE PHENOTYPE DOMINANT

AUTOSOMAL AUTOSOMAL
RECESSIVE DOMINANT RECESSIVE

X LINKED X LINKED
RECESSIVE DOMINANT
GENOTYPE AND
PHENOTYPE FOR
BLUE EYES

GENOTYPE FOR BLUE EYES

BUT NOT PHENOTYPE FOR
BLUE EYES
DOMINANT BEHAVIOR

BROTHERLY BEHAVIOR
• PARENTS LEFT
• 1st son – Clean the 2nd floor of the house
• 2nd son – clean the 1st floor
• 1st son mutated, will not do the task, will
interfere with the other brother
• 2nd son will perform his duty

PARENTS CAME BACK – SAW THE

PROBLEM
RECESSIVE BEHAVIOR

SISTERLY BEHAVIOR
• PARENTS LEFT
• 1st daughter – Clean the 2nd floor of the house
• 2nd daughter – clean the 1st floor
• 1st daughter mutated, will not do the task, will
not interfere with the other sister
• 2nd daughter will perform his duty and
compensate for his sister’s inactivity

PARENTS CAME BACK – DID NOT SEE THE

PROBLEM

***S/SX USUALLY APPEARS WHEN BOTH ARE

MUTATED****
 AUTOSOMAL DOMINANT
88 88*

88 88 88 88
88 *
*

88* 88 88 88 88
* *

88 88
* 88
* X
*88*
 AUTOSOMAL RECESSIVE
88 88*

88 88 88 88
88 *
*

88* 88 88 88 88
* *

88 88
88 * * *88*

* CARRIER/TRAIT
 X LINKED DOMINANT
XY XX
*

XX
XY XX
*
XX XY
*XY

XY
XY XX XX
*
XY
* XX
*
XX *XY

XX XY
XX *
* * *XY
 X LINKED RECESSIVE
XY XX
*

XX
XY XX
*
XX XY
*XY

XY
XY XX XX
*
XY
* XX
*
XX *XY

XX XY
XX *
* * *XY

* CARRIER/TRAIT
DIFFERENT TYPES OF ANEMIA
Decreased Red cell
production

Increased Red cell

ANEMIA destruction
(HEMOLYSIS)

Blood loss
 ANEMIA DUE TO INCREASE RED CELL
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
NORMAL RBC LIFE CYCLE
 1. MEMBRANE – A.A
2. HEMOGLOBIN
Globin Chain ---A.A
Heme – Iron (recycled)
Heme – Porpyrin --Bilirubin

Conjugated Bilirubin

UNCONJUGATED BILIRUBIN
Bilirubin + Protein Binding B.

Urobilinogen

Stercobilinogen

Urobilinogen
SITE OF HEMOLYSIS

EXTRAVASCULAR
 SPLENOMEGALY

• DRAWING
BILIRUBIN STONES

YELLOWISH PALE COLOR

DARK STOOL

SLIGHTLY DARKER URINE

 *EXTRAVASCULAR HEMOLYSIS*

INCREASED
SPLENOMEGALY HYPERCELLULAR
ANEMIA RETICULOCYTE
HEPATOMEGALY BONE MARROR
COUNT

MILD TO
INCREASED MODERATE
SLIGHT
UNCONJUGATED JAUNDICE – INCREASED LDH
MACROCYTOSIS
BILIRUBIN LEMON YELLOW
COLOR SKIN

INCREASED CHRONIC/
DARK COLORED
UROBILINOGEN IN UNCONTROLLED:
STOOL
URINE BILIRUBIN STONES
SITE OF HEMOLYSIS

INTRAVASCULAR
HAPTOGLOBIN

HEMOGLOBINEMIA

ACUTE TUBULAR NECROSIS

HEMOGLOBINURIA
 *INTRAVASCULAR HEMOLYSIS*
INCREASED HYPERCELLULAR BONE
ANEMIA
RETICULOCYTE COUNT MARROW

SLIGHT
HEMOGLOBINEMIA HEMOGLOBINURIA
MACROCYTOSIS

CHRONIC/
DECREASED OR
UNCONTROLLED:
ABSENCE OF
HAPTOGLOBIN ACUTE TUBULAR
NECROSIS
 ANEMIA DUE TO INCREASE RED CELL
DESTRUCTION
HEMOLYTIC ANEMIA
CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

INHERITED ACQUIRED IMMUNE MEDIATED

NON IMMUNE
MEMBRANE DEFECT
MEDIATED

ENZYME DEFECT
(G6PD)

HEMOGLOBIN DEFECT
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

DARK
S/Sx OF
JAUDICE COLORED
ANEMIA
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

LOW HAPTOGLOBIN HEMOGLOBINEMIA HEMOGLOBINURIA

ACUTE TUBULAR
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

ASPIRIN PARACETAMOL?
SOME FOODS THAT SHOULD BE AVOIDED

FAVA BEANS ARTIFICIAL

AND OTHER MENTHOL BLUE FOOD
LEGUMES COLORING

GLUTEN FREE CHINESE

PRODUCTS AMPALAYA HERBS

BLACK/GREEN
TEA SOYA
 LIST OF ANTIOXIDANTS

SUNFLOWER
BERRIES WALNUT GINGER
SEEDS

GRAPES ORANGE PINEAPPLE PRUNES

SPINACH TOMATOES GARLIC

 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.
More than Drops On A Card,
Save lives
 ANEMIA DUE TO INCREASE RED CELL
DESTRUCTION
HEMOLYTIC ANEMIA
CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

INHERITED ACQUIRED IMMUNE MEDIATED

NON IMMUNE
MEMBRANE DEFECT
MEDIATED

ENZYME DEFECT

HEMOGLOBIN DEFECT
(SICKLE CELL & THALASSEMIAS)
 HEMOLYTIC ANEMIA
QUALITATIVE HEMOGLOBINOPATIES SICKLE CELL DISEASE

GLOBIN CHAIN
DEFECT
αTHALASSEMIAS

QUANTITATIVE THALASSEMIAS

β THALASSEMIAS
REVIEW – ANATOMY & PHYSIOLOGY
REVIEW – ANATOMY & PHYSIOLOGY
 DIFFERENT TYPES OF GLOBIN CHAIN

EPSILON ZETA
CHAIN () CHAIN (ɀ)

ALPHA BETA
CHAIN () CHAIN ()

GAMMA DELTA
CHAIN () CHAIN ()
ZETA CHAIN (ɀ) +
EPSILON CHAIN ()
GOWER’S HEMOGLOBIN

ALPHA CHAIN ()+

ALPHA CHAIN ()+ BETA CHAIN ()
GAMMA CHAIN () ADULT 1 HEMOGLOBIN (HbA1)
FETAL HEMOGLOBIN (HbF)

ALPHA CHAIN ()+

DELTA CHAIN ()
ADULT 1 HEMOGLOBIN (HbA2)
ELECTROPHORESIS
SICKLE CELL DISEASE
 SICKLE CELL DISEASE
• Autosomal recessive genetic disorder

• The most common form of an inherited blood disorder that causes the
production of abnormal hemoglobin

• In the United States about 1,000 babies are born with sickle cell
disease each year.

• 70,000-100,000 Americans have sickle cell anemia

• 1 out of every 365 Black or African-American births.

• 1 in 13 Black or African-American babies is born with sickle cell trait

• Nigeria - 45,000-90,000 babies with sickle cell disease are born each
year.
 SICKLE CELL DISEASE

HB AA HB AS HB AS HB SS
 SICKLE CELL DISEASE PATHOPHYSIOLOGY
VALINE IS INSERTED
POINT MUTATION IN ALTERATION IN THE INSTEAD OF GLUTAMIC
BETA GLOBIN STUCTURE
THE BETA CHAIN- 1 TRANSCRIPTION OF ACID ON THE 6TH
ABNORMALITY
NUCLEOTIDE AMINO ACID POSITION OF AMINO
ACID
CAUSES - HYPOXIC CONDITION

TEMPERATURE
HIGH ALTITUDE SEVERE EXECISE
CHANGES

UNPRESSURIZED ANESTHESIA –
CABIN OPERATION INFECTION

DEHYDRATION INFLAMMATION
 SICKLE CELL DISEASE PATHOPHYSIOLOGY
HEMOGLOBIN
BETA GLOBIN HEMOGLOBIN
ANY HYPOXIC STRUCTURES HEMOYSIS - SICKLE
ABNORMAL WILL PRECIPITATE
CONDITION FORMED FIBROUS CELL ANEMIA
PROTRUSION INTO GEL
POLYMER

HIGH
SPLENOMEGALY & LEMON YELLOW
UNCONJUGATED DARK STOOL BILI STONES
HEPATOMEGALY COLOR
BILIRUBIN

ENLARGEMENT OF
THE BONES IN THE RETICULOCYTOSIS PAIN ATTACKS INFARCTS
FACE AND SKULL
SICKLE CELL DISEASE S/Sx
PAIN ULCERS

DELAYED GROWTH VISUAL PROBLEMS

FREQUENT
AUTOSPLENECTOMY INFECTIONS
SEVERE HYPOXIC CONDITION

VASO
PAIN ATTACKS OCCLUSIVE
ATTACK

RBM
EXPANSION
 SICKLE CELL CRISIS

VASO
OCCLUSIVE APLASTIC CRISIS
CRISIS

SEQUESTRATION HEMOLYTIC
CRISIS CRISIS
SEVERE HYPOXIC CONDITION
 TREATMENT

FOLIC ACID AND

HYDRATION PENICILLIN ANALGESICS

EXCHANGE OR
BLOOD
CHELATION HYDROXYUREA
TRANSFUSION –
IRON LOADING?

BONE MARROR OR
ARGININE STEMCELL
TRANSPLANT
 NURSING INTERVENTIONS

1. HYDRATION
2. Managing pain
3. Preventing and managing infections
4. Vaccinations
5. Minimizing deficient knowledge
6. Promoting coping skills
7. Monitoring and managing potential complications
8. Promoting home and community based care
9. Stem cell transplant and gene therapy
THALASSEMIA
 THALASSEMIA
• Autosomal recessive genetic disorder

• Thalassemia affects approximately 4.4 of every

10,000 live births throughout the world

• African-american, Mediterranean countries

 THALASSEMIA

AFFECTE
NORMAL TRAIT TRAIT D
ALPHA THALASSEMIA PATHOPHYSIOLOGY
APLHA THALASSEMIA PATHOPHYSIOLOGY
 ALPHA THALASSEMIA
THALASSEMIA HB H DISEASE HEMOGLOBIN
SILENT CARRIER
TRAIT (4) BARTS (4)

SEVERE ANEMIA
ASYMPTOMATIC MILD ANEMIA (EXTRAVASCULAR HYDROPS FETALIS
HEMOLYSIS)
 TREATMENT
EXCHANGE OR
BLOOD
FOLIC ACID
TRANSFUSION –
IRON LOADING?

BONE MARROR OR
CHELATION STEMCELL
TRANSPLANT
BETA THALASSEMIA
BETA THALASSEMIA
 BETA THALASSEMIA

THALASSEMIA THALASSEMIA THALASSEMIA

MINOR INTERMEDIA MAJOR

MODERATE ANEMIA SEVERE ANEMIA –

ASYMPTOMATIC
– MAY REQUIRE BLOOD
INCREASED HB A2
EPISODIC BLOOD TRANSFUSION FOR
& HB F
TRANSFUSION LIFE
 TREATMENT
EXCHANGE OR
BLOOD
FOLIC ACID
TRANSFUSION –
IRON LOADING

BONE MARROR,
GENE THERAPY OR
CHELATION STEMCELL
TRANSPLANT
 CHELATION THERAPY
• EXJADE
• DESFERAL
 ANEMIA DUE TO INCREASE RED CELL
DESTRUCTION
HEMOLYTIC ANEMIA
CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

INHERITED ACQUIRED INON-MMUNE MEDIATED

MEMBRANE DEFECT PNH IMMUNE MEDIATED

ENZYME DEFECT

HEMOGLOBIN DEFECT
 HEMOLYTIC ANEMIA
HEMOLYTIC ANEMIA
CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

NON IMMUNE
IMMUNE MEDIATED
MEDIATED

SPLENOMEGALY/
MECHANICAL CAUSES
INFECTION CHEMICAL SPLENIC
SEQUESTRATION

MAHA(Macroangiopathic
MALARIA LEAD EBV Hemolytic Anemia)

MAHA(Microangiopathic
CLOSTRIIDIUM CMV Hemolytic Anemia)
 MACROANGIOPATHIC HEMOLYTIC ANEMIA
(MAHA)

ILL FITTING
TRAUMATIC CALCIFIC AORTIC
PROSTHETIC
DAMAGE VALVE STENOSIS
CARDIAC VALVE

CONGENITAL
CARDIOVASCULAR MARCH
ATHLETES
ANOMALIES eg HEMOGLOBINURIA
coarctation of aorta
 MICROANGIOPATHIC HEMOLYTIC ANEMIA
(MAHA)
DIC
TRAUMATIC (Disseminated
DAMAGE Intravascular
Hemolysis)

TTP (Thrombocytopenic
Thrombotic Purpura) HUS
ITP (Hemolytic Uremic
(Idiopathic Syndrome)
Thrombocytopenic Purpura)
 HEMOLYTIC ANEMIA
HEMOLYTIC ANEMIA CAUSES

INTRINSIC FACTOR EXTRINSIC FACTOR

(INTRACORPUSCULAR) (EXTRACORPUSCULAR)

NON IMMUNE MEDIATED IMMUNE MEDIATED

AUTO ANTIBODY

ISO ANTIBODY
IMMUNE MEDIATED HEMOLYSIS

AUTO ANTIBODY
1. WARM ANTIBODY HEMOLYTIC
ANEMIA
2. COLD ANTIBODY HEMOLYTIC ANEMIA
3. COLD ANTIBODY AGGLUTINS
IMMUNE MEDIATED HEMOLYSIS

ISO ANTIBODY
1. ABO
INCOMPATIBILITY
2. RH INCOMPATIBILITY
TYPES OF ANTIBODY
ABO INCOMPATIBILITY
 PLASMA HAS ANTIBODY
FOR “B” ANTIGEN = YYY
RBC HAS “B” ANTIGEN
ON ITS SURFACE RBC HAS “A” ANTIGEN
ON ITS SURFACE
PLASMA HAS ANTIBODY
FOR “A”ANTIGEN = YYY
 Rh BLOOD GROUP
• It was first discovered in Rhesus Monkey

85%

15%
ABO INCOMPATIBILITY
 _ _ _ _
YYY YYY YYY YYY
YYY YYY YYY
YYYYYY YYYYYY
YYY
+ + + YYY +
YYY
• 28 weeks AOG
• Within 72 hours post partum
COOMB’S TEST
COOMB’S REAGENT

COOMB’S
REAGENT
COOMB’S TEST

COOMB’S
REAGENT
COOMB’S TEST

COOMB’S
REAGENT