Transcript 11. Interventions for Clients with Hematologic Problems

Interventions for Clients
with Hematologic Problems
Anemia
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Reduction in either the number
of red blood cells, the amount of
hemoglobin, or the hematocrit
Clinical sign (not a specific
disease); a manifestation of
several abnormal conditions
ANEMIA
1.) Anemia is reduction in either RBCs,
amount of hemoglobin, or hematocrit (%
of packed RBC per deciliter of blood)
“Anemia” is a symptom of an underlying
disease.
 Causes and types vary:
 1.)dietary problems—deficiency in
components necessary to make RBC—
iron, vitamin B12 (cyanocobalamin), folic
acid, or intrinsic factor
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2.)genetic disorders
3.)bone marrow disease
4.)excessive bleeding
5.)Immune reactions
6.)Changes in blood chemistry
7.)Toxins in the blood
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Chronic anemias develop gradually,
more subtle symptoms-- lethargy, pallor,
and anorexia (gastritis, hemorrhoids, menstrual
flow)
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Acute anemias do not allow the body
sufficient time to make physiologic
adjustments -- patients symptomatic
with shortness of breath, extreme
fatigue, and cardiac discomfort (trauma,
blood vessel rupture)
Hematologic Problems
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Anemias
Results in: reduction in oxygen
transport due to decrease in
hemoglobin production, a decrease in
erythrocytes, or a combination of
these factors.
Reduced oxygen leads to less energy
in all cells, reduced cell metabolism
and reproduction.
Compensation mechanisms include tachycardia
and peripheral vasoconstriction
Hematologic Problems
Anemias
General signs of anemia: fatigue, pallor,
dyspnea, and tachycardia
Severe anemia may lead to angina if
oxygen supply to the heart is insufficient
Chronic severe anemia may cause CHF
Other affects may include hair and skin
changes
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Key Features of Anemia
Integumentary manifestations
 Pallor, of ears, nail beds, palmar creases,
conjunctiva, and around mouth
 cool to touch
 intolerance of cold temperatures
 Nails become brittle, overtime become concave and
fingers are club like in appearance.
Cardiovascular Manifestations
 Tachycardia, murmurs, gallops when anemia severe
orthostatic hypotension
Key Features of Anemia
Respiratory Manifestations
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Dyspnea on exertion
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Decreased oxygen saturation levels
Neurologic Manifestations
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Increased somnolence and fatigue
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Headache
LABORATORY
PROFILE
Significance of abnormal finding
Test
Red blood cell count
Hemoglobin/Hematocrit
Mean cell hemoglobin (MCV)
cells,
Reticulocyte count
client
Decreased indicate possible
anemia/hemorrhage
Increased indicate possible chronic
hypoxia, or polycythemia vera
Increased levels indicate macrocytic
possible anemia. Decreased levels
indicate microcytic cells,
possible iron deficiency anemia
helpful in determining bone marrow function
(immature RBC) **Increased levels indicate
chronic blood loss—desireable in anemic
or after hemorrhage.
Hemoglobin electrophoresis
detects abnormal forms of hemoglobin, such as
hemoglobin S in sickle cell disease,
Prothrombin time /INR
assesses extrinsic clotting cascade,
reflects how much clotting
factors II, V, VII, X is functioning.
Increased=deficient in clotting factor
cascade.
Decreased=vitamin K excess.
(monitors Coumadin tx) 25-38 sec
PTT aPartial thromboplastin time assesses the intrinsic clotting cascade,
factors VIII, IX, XI, XII.
Prolonged w/hemophilia or disseminated
intravascular coagulation (DIC).
(monitors Heparin)
Level maintained 1.5 to 2.5 times their
baseline values
3.) Hypoproliferative anemia. Hypoproliferative anemia
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can be subdivided into three classes based upon the size of
the RBCs. The cells may be larger than normal (macrocytic),
normal (normocytic), or smaller than normal (microcytic).
Macrocytic anemia. Macrocytic anemia can be due
to several causes. The first is a deficiency in
vitamin B12 or folate, both important ingredients in
RBC production.
Microcytic anemia. Microcytic anemia is due to
abnormalities in the production of the essential
RBC protein, hemoglobin. This is often to due to
underlying disease, such as thalassemia, iron
deficiency anemia
Normocytic anemia. Normocytic anemia may be due
to chronic disease including malnutrition or mixed
anemia (combined macrocytic and microcytic
anemia).
Sickle Cell Disease
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Genetic disorder resulting in
chronic anemia, pain, disability,
organ damage, increased risk for
infection, and early death
Formation of abnormal
hemoglobin chains
Conditions causing sickling:
hypoxia, dehydration, infections,
venous stasis, low environmental
body temperatures, acidosis,
strenuous exercise, and
anesthesia.
Clinical Manifestations
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Cardiovascular changes
Skin changes
Abdominal changes
Musculoskeletal changes
Central nervous system changes
Interventions
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Pain is the most common
problem.
• Drug therapy: 48 hours of
intravenous analgesics
• Oral hydration
• Complementary and alternative
therapies
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Glucose-6-Phosphate
Dehydrogenase (G6PD)
Deficiency Anemia
Most common type of
congenital hemolytic
anemia
Hydration
Screening for this
deficiency necessary
before donating blood,
because cells deficient in
G6PD can be hazardous
Iron Deficiency Anemia
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This common type of anemia can
result from blood loss, poor
intestinal absorption, or
inadequate diet.
Evaluate adult clients for
abnormal bleeding.
Supplemental iron is the
treatment.
Блідість конюнктив,
шкірних покривів
Vitamin B12 Deficiency
Anemia
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Anemia is caused by inhibiting
folic acid transport and reducing
DNA synthesis in precursor cells.
Vitamin B12 deficiency is a result
of poor intake of foods
containing vitamin B12.
Pernicious anemia is anemia
caused by failure to absorb
vitamin B12 and lack of intrinsic
factor;
Folic Acid Deficiency Anemia
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Can cause megaloblastic anemia
Manifestations similar to those of
vitamin B12 deficiency, but
nervous system functions remain
normal
(Continued)
Folic Acid Deficiency Anemia
(Continued)
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Caused by:
• Poor nutrition and chronic alcohol
abuse
• Malabsorption syndromes, such as
Crohn’s disease
• Drugs, including anticonvulsants
and oral contraceptives, that slow
or prevent absorption of folic acid
Polycythemia Vera
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Disease with a sustained
increase in blood hemoglobin
Massive production of red blood
cells
Excessive leukocyte production
Excessive production of platelets
Phlebotomy
Increased hydration
Anticoagulants are part of
therapy
Polycythemia Vera
Due to hyperviscous (thicker than normal blood) the following
may occur:
 Key features:
 Client’s facial skin and mucous membranes have a dark,
flushed (plethoric) appearance
 Distention of superficial veins
 Weight loss
 Intense itching
 Hypertension
 Fatigue, enlarged hemorrhoids
 Swollen painful joints
 Enlarged firm spleen
 Infarctions of the heart (chest pain, heart failure), kidneys
 Strokes
 Bleeding tendency
Polycythemia Vera
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Diagnostic Tests
Blood cell counts and hematocrit
markedly elevated
Hyperuricemia due to high cell
destruction
Bone marrow hypercellular
Hgb levels to 18 g/dl
Hct of 55%
or >
RBC count of 6 mil/mm3
Polycythemia vera
Collaborative management:
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Phlebotomy (treatment) blood drawing
Increase hydration
Anticoagulants are part of therapy to
prevent clot formation
Chemotherapy to suppress bone
marrow activity
Radiation therapy
Bone marrow transplantation
Significant number of individuals with
PV go on to develop acute leukemia
Polycythemia vera
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Client education guide
Drink at least 3 L day
Avoid tight or constrictive clothing,
especially garters or girdles
Wear gloves when outdoors in
temperature lower than 50 degrees
Contact physician first sign of infection
Use soft-bristled toothbrush
Do not floss teeth
Polycythemia vera
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Take anticoagulants as prescribed
Wear support hose while awake and up
Elevate feet when you are seated
Exercise slowly and only on the advice
of your physician
Stop activity at the first sign of chest
pain
Use electric shaver
Polycythemia
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Production and presence of increased
RBCs
2 types:
Primary polycythemia =
Polycythemia Vera
Secondary Polycythemia = a.)
hypoxia driven = high altitude,
cardiopulmonary disease, defection
O2 transport
b.)Hypoxia independent= renal
cysts or tumors
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It is a cancer of the RBCs with 3 major
hallmarks:
1.) Massive production of red blood cells
2.) Excessive leukocyte production
3.) Excessive production of platelets
or >
Hgb levels to 18 g/dl
RBC count of 6 mil/mm3
Hct of 55%
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Aplastic Anemia (macrocytic)
Cause: deficiency of circulation RBC due to
failure of bone marrow to produce these RBC
cells, may occur alone or with
Leukopenia (decreased WBC) and
thrombocytopenia (decreased platelets).
When ALL three occur together it is called
“Pancytopenia”
Cause:
1.) Congenital in origin – chromosomal
abnormality
2.) Acquired--Long term exposure to toxic
agents, ionizing radiation or infection, (viral,
bacterial), medications, antiseizure,
antimicrobials) may cause Aplastic anemia.
Signs and Symptoms:
Onset insidius:
 manifestations include those of anemia
 Those of leukopenia (recurrent multiple
infections)
 Those related to thrombocytopenia (petechia,
tendency to bleed excessively,
especially in the mouth.
Aplastic Anemia (macrocytic)
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Diagnosis: definitive bone marrow aspiration
red bone marrow is replaced by fatty red
bone marrow
Treatment: blood transfusions,
immunosuppressive therapy (antilymphocyte
globulin (ALG), cyclosporine (Sandiuumne),
prednisone, cyclophosphamide (Cytoxan) can
bring about partial or complete remissions.
Splenectomy on clients with enlarged spleen
that is either destroying normal RBCs or
suppressing their development
Bone marrow transplant.
Hemolytic Anemia
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Destruction or hemolysis of RBCs at a rate that
exceeds production
Third major cause of anemia
Intrinsic hemolytic anemia
• Abnormal hemoglobin (sickle cell)
• Enzyme deficiencies
• RBC membrane abnormalities
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Extrinsic hemolytic anemia
• Acquired
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(mechanical injury heart bypass, toxins)
Sites of hemolysis
• Intravascular
• Extravascular
Hemolytic Anemia
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Jaundice
• Destroyed RBCs cause increased bilirubin
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Enlarged spleen and liver
• Hyperactive with macrophage phagocytosis
of the defective RBCs
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Accumulation of hemoglobin molecules
can obstruct renal tubules
• Tubular necrosis
2.) Hemolytic Anemias resulting
from increased destruction
(hemolysis) of RBCs
a.) Sickle cell disease —
b.) Glucose-6-Phosphate
Dehydrogenase Deficiency
anemia
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Thrombotic
Thrombocytopenia Purpura (TTP)
is a rare autoimmune reaction in
blood vessels disorder in which
platelets clump together
abnormally in the capillaries and
Hemochromatosis
Primary hemochromatosis is an inherited disorder
characterized by excessive iron accumulation due
to increaxed intestinal iron absorption causing
tissue damage.
 Symptoms do not develop until organ damage,
often irreversible, develops.
 Symptoms include fatigue, hepatomegaly, bronze skin
pigmentation, loss of libido, arthalgias, and
manifestations of cirrhosis, diabetes, or
cardiomyopathy.
 Diagnosis is based on serum iron studies and gene
assay.
 Treatment serial phlebotomies
Leukemia
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Type of cancer with uncontrolled
production of immature white
blood cells in the bone marrow
Acute or chronic
Classified by cell type
Risk factors: ionizing radiation,
exposure to certain chemicals
and drugs, bone marrow
hypoplasia, genetic factors,
immunologic factors,
environmental factors
Leukemia are grouped into 4 types--according to how quickly they progress
and the type of cell involved.
1.) Acute Lymphocytic Leukemia (ALL)**
(malignant cells are mainlyB
lymphocytes)
--most common in children
--Signs and symptoms may appear
abruptly
• Fever
• Bleeding
• CNS manifestations, common
2.) Acute Myeloid(myelogenous) Leukemia (AML)
Myeloblasts affected = precursor to
granulocytes
(malignant cells granulocytes (neutrophils, eosinophils,
basophils)
Also called acute nonlymphoblastic leukemia
(ANLL)
--stem cell of WBC proliferates, decreasing
stem cells availability for RBC and platelets
--result hyperplasia of bone marrow
--25% of all leukemias
• 85% of the acute leukemias in adults
--Abrupt, dramatic onset
• Serious infections or abnormal bleeding
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3.) Chronic Lymphocytic Leukemia (CLL)
--most common form of adult leukemia
( malignant cells are B lymphocytes)
* Lymph node enlargement is present
throughout body
• Increased incidence of infection
Pain, paralysis from pressure caused by enlarged
lymph nodes occurs in later stage of disease
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4.) Chronic Myeloid Leukemia (CML)--(malignant cells are granulocytes= eosinophis,
basophils, neutrophils)
*Philadelphia chromosome
• Genetic marker
* Move into peripheral blood in massive numbers
• Ultimately infiltrate liver and spleen
Clinical Manifestations
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Cardiovascular: heart rate is
increased; blood pressure is
decreased.
Respiratory rate increases.
Skin grows pale and cool to the
touch.
Intestinal manifestations include
weight loss, nausea, and
anorexia.
Central nervous system
disturbances include headache.
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Leukemia
Laboratory Assessment
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Decreased hemoglobin and
hematocrit levels
Low platelet count
Abnormal white blood cell
count, may be low, normal
or elevated, but is usually
quite high
Poorer prognosis: client
with high white blood cell
count at diagnosis
Laboratory Assessment
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Definitive test: examination of
cells obtained from bone marrow
aspiration and biopsy
Drug Therapy for Acute
Leukemia
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Induction therapy
Consolidation therapy
Maintenance therapy
New drug therapies
Drug therapy for infection
Infection Protection
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Frequent handwashing
Private room
HEPA filtration or laminar airflow
system
Mask for visitor with upper
respiratory infection
“Minimal bacteria diet” without
uncooked foods
Monitoring of daily laboratory
results
Bone Marrow Transplantation
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Standard treatment for leukemia
Purges present marrow of the
leukemic cells
After conditioning, new, healthy
marrow given to the client
toward a cure
Sources of stem cells
Conditioning regimen
Transplantation
Risk for Injury
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Nadir: period of greatest bone
marrow suppression
Bleeding precautions
Fatigue
Interventions:
• Diet therapy
• Blood replacement therapy
• Drug therapy
• Energy conservation
Hodgkin’s Lymphoma
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Cancer that starts in a single
lymph node or a single chain of
nodes
Marker: Reed-Sternberg cell
Large, painless lymph node
usually in the neck; fever,
malaise, night sweats
One of the most curable cancers
Treatment: external radiation
alone or with combination
chemotherapy
Non-Hodgkin’s Lymphoma
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All lymphoid cancers that do not
have the Reed-Sternberg cell
More than 12 types of nonHodgkin’s lymphoma
Low-grade lymphomas less
responsive to treatment; cures
are rare
Treatment: radiation therapy and
multiagent chemotherapy, or
single-agent therapy with
fludarabine
Multiple Myeloma
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White blood cell cancer that
involves a more mature
lymphocyte than either leukemia
or lymphoma
Uncommon cancer
Manifestations: fatigue, easy
bruising, bone pain, fractures,
hypertension, increased
infection, hypercalcemia, and
fluid imbalance
Treatment: chemotherapy
Autoimmune
Thrombocytopenic Purpura
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Large ecchymosis or petechial
rash on arms, legs, upper
chest, and neck
Diagnosed by decreased
platelet count and large
numbers of megakaryocytes in
the bone marrow
Interventions include:
• Therapy to prevent bleeding
• Drug therapy to suppress immune
function
Thrombotic
Thrombocytopenic Purpura
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Rare disorder; platelets clump
together abnormally in the
capillaries and too few platelets
remain in circulation
Inappropriate clotting, yet blood
fails to clot properly when
trauma occurs
Plasma pheresis, infusion of FFP
Aspirin, alprostadil, plicamycin
Immunosuppressive therapy
Hemophilia
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Hemophilia A is deficiency of
factor VIII and accounts for 80%
of cases.
Hemophilia B (Christmas
disease) is deficiency of factor IX
and accounts for 20% of cases.
For hemophilia A with blood
transfusion and factor VIII
therapy, survival time has
increased greatly.
Transfusion Therapy
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Pretransfusion responsibilities to
prevent adverse transfusion
reactions:
• Verify prescription.
• Test donor’s and recipient’s blood
for compatibility.
• Examine blood bag for
identification.
• Check expiration date.
• Inspect blood for discoloration, gas
bubbles, or cloudiness.
Transfusion Responsibilities
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Provide client education.
Assess vital signs.
Begin transfusion slowly and
stay with client first 15 to 30
minutes.
Ask client to report unusual
sensations such as chills,
shortness of breath, hives, or
itching.
Administer blood product per
protocol.
Types of Transfusions
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Red blood cell
Platelet transfusions
Plasma transfusions: fresh frozen
plasma
Cryoprecipitate
Granulocyte (white cell)
transfusions
Transfusion Reactions
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Clients can develop any of the
following transfusion reactions:
• Hemolytic
• Allergic
• Febrile
• Bacterial
• Circulatory overload
Autologous Blood
Transfusion
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Collection and infusion of client’s
own blood
Eliminates compatibility
problems; reduces risk for
transmission of bloodborne
disease
Preoperative autologous blood
donation
Acute normovolemic