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APLASTIC

ANEMIA

HAZEL ARVEE B. POSIS


The bone marrow (soft tissue
which is located within the
hard outer shell of the bones)
is responsible for the
production of all the types of
blood cells. The mature forms
of these cells include red
blood cells, which carry
oxygen throughout the body;
white blood cells, which fight
infection; and platelets, which
are involved in clotting. In
aplastic anemia, the basic
structure of the marrow
becomes abnormal, and those
cells responsible for
generating blood cells
(hematopoietic cells) are
greatly decreased in number
or absent. These
hematopoietic cells are
replaced by large quantities of
fat.
 Is a physiological and anatomical failure of the bone marrow characterized
by a marked decrease or absence of blood-forming elements in the marrow
and peripheral pancytopenia (decreased RBCs ,WBCs and platelets ).

 A rare and serious blood disorder in which bone marrow stops making
enough new blood cells. aplastic anemia, normal production of all blood
cells—red cells, white cells, and platelets—slows or stops. This is because
the stem cells have been damaged. The cause of this damage is often
unknown.

Aplastic anemia is caused by damage to stem cells in the bone marrow.


Stem cells normally develop into three types of blood cells: red blood cells,
white blood cells, and platelets. When stem cells are damaged, they do not
grow into healthy blood cells.
Causes Of Aplastic Anemia
Inherited Acquired
Fanconi anemia Idiopathic (70 % or more cases)
Familial aplastic anemia Secondary
Dyskeratosis congenita • Drugs
Shwachman Diamond Syndrome •6 –mecaptopurine
Dubowitz syndrome •Methotrexate
•Cyclophosphamide
•Chloremphenicol
Chemicals :insecticides
Toxin(e.g.,benzen ,carbon
tetrachloride)
Irradiation
Infection
•Viral hepatitis
•HIV
•Infectious mononucleosis
•CMV
Myelodysplastic syndrome
CAUSES

DAMAGE TO STEM CELL

DEPRESSION OR CESSATION OF ACTIVITY OF


REPEATED
ALL BLOOD PRODUCING ELEMENT
INFECTION;
FREQUENT
SICK DAY

LEUKOPENIA
BLEEDING THROMBOCYTOPENIA
TENDENCY AEB
ECCHYMOSIS,
DECREASE IN FORMATION OF RBC
PURPURA,
PETICHIAE,
BLEEDING FROM NOSE,
MOUTH, PALLOR OF SKIN & MUCOUS
VAGINA, RECTUM MEMBRANE, CYANOSIS
PANCYTOPENIA

APLASTIC ANEMIA
Clinical features of Aplastic Anemia
1. Anemia result in pallor ,easy fatigability ,weakness and loss of
appetite
2. Thrombocytopenia leads to petechiae ,easy bruising ,severe
nosebleeds and bleeding into the GIT and renal tract
3. Leukopenia leads to increased susceptibility to infections and oral
ulcer that response poorly to antibiotic therapy.
4. Hepatosplenomegaly and lympadenopathy do not occur ; their
presence suggest underlying leukemia
5. Hyperplastic gingivitis is also a symptom of aplastic anemia
6. Special features ;
I. Skin ; Hyperpigmentation, café –au-lait spots,erythematous
rash
II. Head ;Microcephaly ,micro-ophthalmia
III. Mouth ; cleft lip,leukoplakia
IV. General : small stature
Nursing care is based on carefull assessment and mngt. Of the
complication of pancytopenia

Primary focused on preventing infection and monitoring for


signs of bleeding
To prevent infection in the hospitalized patient who is
immunosupressed:
3. Provision of private room
4. Use of protective isolation
5. Meticulous hygiene
6. Protective oral care management.
7. Monitoring of invasive lines for signs of infection
8. Avoidance of bladder catheterization
9. Instruct family and visitors of careful handwashing.
Monitor for signs of bleeding and provide
measures to minimize risk
Use a soft toothbrush and electric razor
Avoid injections and venipuncture
Hematest urine and stool
Observe for oozing from gums, petechiae or ecchymoses
Avoiding rectal thermometer, medications, enema.
Monitor invasive lines sites.
Measures to avoid fatigue:

Frequent periods of rest


O2 carrying of the
Avoid fatigue producing
activities blood
Monitor patient on signs of
DOB.
O2 supply to
tissues

FATIGUE
Medical management
1. Blood transfusion: key to therapy until client’s own marrow begins to
produce blood cells

2. Aggressive treatment of infections

3. Bone marrow transplantation

4. Drug therapy

a. Corticosteroids and/or androgens to stimulate bone marrow


function and to increase capillary resistance
(Patients with aplastic anaemia are particularly vulnerable to damage to
large joints which may result from steroid treatment)
1. Bone marrow aspiration, also
called bone marrow sampling, is
the removal by suction of fluid
from the soft, spongy material that
lines the inside of most bones.
2. Bone marrow biopsy, or
needle biopsy, is the removal of a
small piece of bone marrow.
3. A sample of the patient's bone
marrow will need to be removed
by needle (usually from the hip
bone) and examined under a
microscope. If aplastic anemia is
present, this examination will
reveal very few or no
hematopoietic cells, and
replacement with fat.
The most successful treatment for aplastic anemia is
bone marrow transplantation. To do this, a marrow donor
(often a sibling) must be identified. There are a number of
tissue markers which must be examined to determine
whether a bone marrow donation is likely to be
compatible with the patient's immune system.
Compatibility is necessary to avoid complications,
including the destruction of the donor marrow by the
patient's own immune system.

Patients who cannot undergo bone marrow transplant


can be treated with a number of agents, including
• antithymocyte globulin (ATG)
• cyclophosphamide
• steroids
•cyclosporine.
ANTITHYMOCYTE GLOBULIN

An immunosuppressant which is used to reduce the body's natural


immunity in patients who receive a bone marrow transplant or
undergo treatment for Aplastic Anaemia It reduces the activity of the
T lymphocytes which are attacking bone marrow stem cells.

When a patient receives a bone marrow transplant , the


white blood cells produced by the new stem cells in the bone marrow
will try to get rid of (reject) the patients body. Antithymocyte globulin
works by eliminating the white blood cells which are doing this.
Before Having This Treatment
allergic reaction to horses or rabbits

Precautions:
increase the chance of getting other infections
fever, chills and shakes within a few hours after the first dose.

Side Effects of This Medicine


H20 retention
Report: heart working harder, bloating or swelling
cyclophosphamide
A drug used to treat cancers and to suppress the rejection of
transplanted tissue. It basically kills off all the white cells . It is the drug
of choice for those having a Bone Marrow Transplant.

Cyclophosphamide commonly causes nausea, vomiting and hair


loss and can affect the heart, lungs and liver. It can also cause
severe bladder damage in susceptible people because it produces
a toxic substance called acrolein (a colourless liquid with an
irritating odour and used in chemical warfare as a tear gas).
Another drug may be given before and after each dose of
cyclophosphamide to reduce its toxicity. Inform your doctor if you
find it painful to pass urine while you are on this drug. It can also
lead to abnormal bleeding due to lowered blood cells, and
increased risk of infection and reduced fertility in men. Drink lots of
water while taking this drug. This will usually prevent it from causing
bladder irritation.
STEROIDS

One of a group of hormones chemically related to cholesterol. They


include oestrogen, which controls female sexual development,
androgen , which produces male characteristics and builds up
muscular protein tissue, progesterone, which plays an important part in
the regulation of the menstrual cycle and in pregnancy, and the
corticosteroids , which are responsible for chemically making use of the
carbohydrates and fats we eat, controlling inflammation, and salt and
water regulation. Steroids may be naturally occurring or they may be
synthesised.
CYCLOSPORINE
A drug used with ATG / ALG treatment to further suppress the
immune system by inhibiting the activity of only the T lymphocytes .
It is also used in bone marrow transplants to help handle GVHD . It
also decreases the body's ability to fight off infections.
side effects
confusion
nervousness
unusual weakness or tiredness
difficulty breathing
shortness of breath
tender, enlarged, or bleeding gums
nausea or vomiting
stomach pain (severe)
irregular heartbeat
convulsions
numbness
when taking cyclosporine :
2. avoid eating excessive amounts of foods high in potassium such as
bananas, oranges, orange juice, milk, waffles, oatmeal, tomatoes,
and baked beans

2. avoid grapefruit juice as it may block the breakdown of


cyclosporine by the liver (the opposite problem to the above). If
this happens, blood levels of cyclosporine could be increased and
this could increase serious side effects.