MANAGEMENT OF PT WITH

NONMALIGNAN HEMATOLOGIC
DISORDERS

Iron Deficiency Anemia (IDA)

 It typically results when the intake of

dietary iron is inadequate for
hemoglobin synthesis.
 It is the most common type of anemia
in all age groups and it is the most
common in the world, affecting 1 in 8
persons.
 The most common cause of IDA in
men and postmenopausal women is
bleeding from ulcers, gastritis,
inflammatory bowel disease, or GI
tumors.
 The most common cause of IDA in
premenopausal women are
menorrhagia and pregnancy with
inadequate iron supplementation.
 Patients with chronic alcoholism or
who take aspirin, steroids, or NSAIDs
often have chronic blood loss from the
GI tract, which causes iron loss and
eventual anemia.
 Clinical Manifestations
 Weakness, fatigue, and general malaise
are common
 Pallor of the skin and mucous
membranes
 If the deficiency is severe or prolonged,
the patients may have smooth, red
tongue.
 Brittle and ridged nails
 Angular cheilosis
Assessment and Diagnostic Findings
 The definitive method of establishing the
diagnosis of IDA is bone marrow
aspiration.
 Decreased Hgb, Hct, and MCV in CBC
Medical Management
constipation, but also cramping, nausea,
and vomiting).
 Advise patient to increase fiber intake.
 Use a straw when taking fluid
preparation of iron.
APLASTIC ANEMIA
 Rather rare disease caused by a
decrease in or damage to marrow stem
cells, damage to the microenvironment
within the marrow, and replacement of
the marrow with fat.
 It results in bone marrow aplasia
(markedly reduced hematopoiesis).
 Therefore, in addition to severe anemia,
significant neutropenia and
thrombocytopenia (a deficiency of
platelets) are also seen.

 Anemia may be a sign of a curable Causes

gastrointestinal cancer or of uterine
fibroid tumors. Stool specimens should  Aplastic anemia can be congenital or
be tested for occult blood. acquired, but most cases are idiopathic
 People 50 years of age or older should (ie, without apparent cause).
have a colonoscopy, endoscopy, or  Infections and pregnancy can trigger it,
other examination of the gastrointestinal or it may be caused by certain
tract to detect ulcerations, gastritis, medications, chemicals, or radiation
polyps, or cancer. damage.
 Several oral iron preparations—ferrous  Agents that regularly produce marrow
sulfate, ferrous gluconate, and ferrous aplasia include benzene and benzene
fumarate—are available for treating iron derivatives (eg, airplane glue).
deficiency anemia.  Certain toxic materials, such as
inorganic arsenic and several
Nursing Management pesticides (including DDT, which is no
longer used or available in the United
 Preventive education is important, States), have also been implicated as
because iron deficiency anemia is potential causes.
common in menstruating and pregnant
women. SUBSTANCES ASSOCIATED WITH
 Food sources high in iron include APLASTIC ANEMIA
organ meats (beef or calf’s liver, chicken
Analgesics
liver), other meats, beans (black, pinto, Antiseizure agents (mephenytoin, triethadione*)
and garbanzo), leafy green vegetables, Antihistamines
raisins, and molasses. Antimicrobials*
 Taking iron-rich foods with a source Antineoplastic agents (alkylating agents, antitumor
antibiotics, antimetabolites)
of vitamin C enhances the absorption of Benzene*
iron. Chloramphenicol*
 Because iron is best absorbed on an Gold compounds*
empty stomach, patients should be Heavy metals
advised to take the supplement an hour Hypoglycemic agents
Insecticided
before meals. Organic arsenicals*
 Other patients have difficulty taking Phenylbutazone*
iron supplements because of Sulfonamides*
gastrointestinal side effects (primarily Sedatives
*MOST COMMON
 Medical Management
 Bone marrow transplantation or
peripheral stem cell transplantation
together with immunosuppressant
therapy by giving antithymocyte globulin
and cyclophosphamide.
 Blood transfusion if needed.
Nursing Management
 Patients with aplastic anemia are
vulnerable to problems related to RBC,
WBC, and platelet deficiencies. They
should be assessed carefully for signs of
infection and bleeding.
MEGALOBLASTIC ANEMIA
 Caused by deficiencies of vitamin B12
or folic acid, identical bone marrow and
peripheral blood changes occur,
because both vitamins are essential for
normal DNA synthesis.
 A bone marrow analysis reveals
hyperplasia (abnormal increase in the
number of cells), and the precursor
erythroid and myeloid cells are large
and bizarre in appearance.
 Many of these abnormal RBCs and
myeloid cells are destroyed within the
marrow, however, so the mature cells
that do leave the marrow are actually
fewer in number.
 Thus, pancytopenia (a decrease in all
myeloid derived cells) can develop.
 In an advanced situation, the
hemoglobin value may be as low as 4 to
5 g/dL, the WBC count 2,000 to
3,000/mm3, and the platelet count less
than 50,000/mm3.
 The classic method of determining the
 Those cells that are released into the
circulation are often abnormally shaped.
 The neutrophils are hypersegmented.
 The platelets may be abnormally large.
 The RBCs are abnormally shaped, and
the shapes may vary widely
(poikilocytosis).
 Because the RBCs are very large, the 
MCV is very high, usually exceeding 110
μm3.
Causes
Two types of causes:
1. Folic acid deficiency – occurs in people
who rarely eat vegetables
2. Vitamin B12 deficiency
 Can occur in strict vegetarianism
since Vitamin B12 is obtained through
eating meat.
 Another cause would be the inability of
the patient to produce Intrinsic Factor in
the stomach which binds to Vitamin B12
in order for it to be absorbed – this type
of anemia is known as pernicious
anemia.
Clinical Manifestations
 Typical anemia signs and symptoms
 Patients with pernicious anemia develop
a smooth, sore, red tongue and mild
diarrhea.
 They are extremely pale, particularly in
the mucous membranes.
 They may become confused; more often
they have paresthesias in the extremities
(particularly numbness and tingling in the
feet and lower legs).
 They may have difficulty maintaining
their balance because of damage to the
spinal cord, and they also lose position
sense (proprioception).
Assessment and Diagnostic Findings
cause of vitamin B12 deficiency is the
Schilling test, in which the patient receives
a small oral dose of radioactivevitamin B12,
followed in a few hours by a large,
nonradioactive parenteral dose of vitamin
B12 (this aids in renal excretion of the
radioactive dose).
If the oral vitamin is absorbed, more than
8% will be excreted in the urine within 24
hours; therefore, if no radioactivity is
present in the urine (ie, the radioactive
vitamin B12 stays within the gastrointestinal
 tract), the cause is gastrointestinal
malabsorption of the vitamin B12.
 Conversely, if the urine is radioactive, the
cause of the deficiency is not ilealdisease
or pernicious anemia.
 Later, the same procedure is repeated, but
this time intrinsic factor is added to the oral
radioactive vitamin B12. If radioactivity is
now detected in the urine (ie, the B12 was
absorbed from the gastrointestinal tract in
the presence of intrinsic factor), the
diagnosis of pernicious anemia can be
made.
 Another useful, easier test is the intrinsic
factor antibody test. A positive test
indicates the presence of antibodies that
bind the vitamin B12–intrinsic factor complex
and prevent it from binding to receptors in
the ileum, thus preventing its absorption.
Medical Management
 Folate deficiency is treated by increasing
the amount of folic acid in the dietand
administering 1 mg of folic acid daily.
 Vitamin B12 deficiency is treated by
vitamin B12 replacement. Vegetarians
can prevent or treat deficiency with oral
supplements through vitamins or fortified
soy milk.
 When, as is more common, the
deficiency is due to defective absorption
or absence of intrinsic factor,
replacement is by monthly intramuscular
injections of vitamin B12, usually at a
dose of 1000 μg.
 The reticulocyte count rises within 1
week, and in several weeks the blood
counts are allnormal.
Nursing Management
 The nurse needs to pay particular
attention to ambulation and should
assess the patient’s gait and stability as
well as the need for assistive devices
(eg, canes, walkers) and for assistance
in managing daily activities.
 If sensation is altered, patients need to
be instructed to avoid excessive heat
and cold.
 Because mouth and tongue soreness
may restrict nutritional intake, the nurse
can advise patients and families to
prepare bland, soft foods and to eat
small amounts frequently.
 Patients must also be taught about the
chronicity of their disorder and the
necessity for monthly vitamin B12
injections even in the absence of
symptoms. Many patients can be
instructed to self administer their
injections.
Sickle Cell Anemia
 Sickle cell anemia is a severe hemolytic
anemia that results from inheritance of
the sickle hemoglobin gene.
 This gene causes the hemoglobin
molecule to be defective. The sickle
hemoglobin (HbS) acquires a crystal-like
formation when exposed to low oxygen
tension.
 The oxygen level in venous blood can be
low enough to cause this change;
consequently, the RBC containing (HbS)
loses its round, very pliable, biconcave
disk shape and becomes deformed,
rigid, and sickle-shaped).
 These long, rigid RBCs can adhere to
the endothelium of small vessels;
when they pile up against each other,
blood flow to a region or an organ may
be reduced
 If ischemia or infarction results, the
patient may have pain, swelling, and
fever.
 The sickling process takes time; if the
RBC is again exposed to adequate
amounts of oxygen (eg, when it travels
through the pulmonary circulation)
before the membrane becomes too
rigid, it can revert to a normal shape.
For this reason the “sickling crises”
are intermittent.
 Cold can aggravate the sickling process,
because vasoconstriction slows the
blood flow.
 Oxygen delivery can also be impaired
by an increased blood viscosity, with or
without occlusion due to adhesion of
 sickled cells; in this situation, the effects
are seen in larger vessels, such as
arterioles.n, the “sickling crises” are
intermittent.
 The HbS gene is inherited in people of
African descent and to a lesser extent in
people from the Middle East, the
Mediterranean area, and aboriginal
tribes in India
Clinical Manifestations
 Patients are always anemic, usually with
hemoglobin values of 7 to 10 g/dL.
 Jaundice is characteristic and is usually
obvious in the sclerae. The bone marrow
expands in childhood in a compensatory
effort to offset the anemia, sometimes
leading to enlargement of the bones of
the face and skull.
 The chronic anemia is associated with
tachycardia, cardiac murmurs, and often
an enlarged heart (cardiomegaly).
 Dysrhythmias and heart failure may
occur in adults.
Sickle Cell Crisis
 There are three types of sickle cell crisis
in the adult population.
 The most common is the very painful
sickle crisis, which results from tissue
hypoxia and necrosis due to inadequate
blood flow to a specific region of tissue
or organ.
 Aplastic crisis results from infection
with the human parvovirus. The
hemoglobin level falls rapidly and the
marrow cannot compensate, as
evidenced by an absence of
reticulocytes.
 Sequestration crisis results when other
organs pool the sickled cells. Although
the spleen is the most common organ
responsible for sequestration in children,
by 10 years of age most children with
sickle cell anemia have had a splenic
infarction and the spleen is then no
longer functional (autosplenectomy). In
adults, the common organs involved in
sequestration arethe liver and, more
seriously, the lungs.
Assessment and Clinical Findings
 The patient with sickle cell trait usually
has a normal hemoglobin level, a normal
hematocrit, and a normal blood smear.
 In contrast, the patient with sickle cell
anemia has a low hematocrit and sickled
cells on the smear.
 The diagnosis is confirmed by
hemoglobin electrophoresis - is a
blood test used to measure and identify
the different types of hemoglobin in your
bloodstream.
Prognosis
 Patients with sickle cell anemia are
usually diagnosed in childhood, because
they become anemic in infancy and
begin to have sickle cell crises at 1 or 2
years of age.
 Some children die in the first years of
life, typically from infection, but the use
of antibiotics and parent teaching have
greatly improved the outcomes for these
children.
 However, with current management
strategies, the average life expectancy is
still suboptimal, at 42 years.
 In some patients, the symptoms and
complications diminish by 30 years of
age; these patients live into the sixth
decade or longer.
Medical Management
 Hydroxyurea (Hydrea), a chemotherapy
agent, has been shown to be effective in
increasing hemoglobin F levels in
patients with sickle cell anemia, thereby
decreasing the permanent formation of
sickled cells.
 Side effects of hydroxyurea include
chronic suppression of WBC formation,
teratogenesis, and potential for later
development of a malignancy.
 Chronic transfusions with RBCs have
been shown to be highly effective in
several situations: in an acute
exacerbation of anemia (eg, aplastic
crisis), in the prevention of severe
complications from anesthesia and
surgery, and in improving the response
to infection (when it results in
exacerbated anemia).
 Patients with sickle cell anemia require
daily folic acid replacements to maintain
 the supply required for increased  This increases the rigidity of the RBCs
erythropoiesis from hemolysis. and thus the premature destruction of
 Infections must be treated promptly with these cells.
appropriate antibiotics; infection remains
a major cause of death in these patients. Types of Thalassemia
1. Alpha Thalassemia

 Thalassemias occur mainly in people

Medical Management
 NSAIDs such as aspirin is given to
relieve the patient from pain. It also
relieves potential thrombosis.
Nursing Management
 Pain management – NSAIDs
administration, relaxation techniques,
and breathing exercises.
 Prevent the occurrence of infection by
giving appropriate antibiotics.
 Providing the patient with opportunities
to make decisions about daily care may
increase the patient’s feelings of control.
 Monitor and manage potential
complications such as:
o Leg ulcers
o Priapism leading to impotence
o Chronic pain and substance abuse
THALASSEMIA
from Asia and the Middle East;
 The alpha-thalassemias are milder
than the beta forms and often occur
without symptoms. The RBCs are
extremely microcytic, but the anemia,
if present, is mild.
2. Beta Thalassemia
 are most prevalent in Mediterranean
populations but also occur in people
from the Middle East or Asia.
 The severity of beta-thalassemia
varies depending on the extent to
which the hemoglobin chains are
affected.
 If left untreated, severe beta-
thalassemia (thalassemia major, or
Cooley’s anemia) can be fatal within
the first few years of life. If it is treated
with regular transfusion of RBCs,
patients may survive into their 20s
and 30s.

 Are a group of hereditary disorders

associated with defective hemoglobin-
chain synthesis.
 These anemias occur worldwide, but the
highest prevalence is found in people of
Mediterranean, African, and Southeast
Asian ancestry.
 Thalassemias are characterized by
hypochromia (an abnormal decrease in
the hemoglobin content of RBCs),
extreme microcytosis (smaller-than-
normal RBCs), destruction of blood
elements (hemolysis), and variable
degrees of anemia.
 In thalassemia, the production of one or
more globulin chains within the
hemoglobin molecule is reduced. When
this occurs, the imbalance in the
configuration of the hemoglobin causes it
to precipitate in the erythroid precursors
or the RBCs themselves. Thalassemia Major
 Thalassemia major (Cooley’s anemia)  Thiazide diuretics (eg,
is characterized by severe anemia, hydrochlorothiazide [Hydro- DIURIL],
marked hemolysis, and ineffective chlorothiazide [Diuril]),
erythropoiesis (production of RBCs).  Oral hypoglycemic agents (eg,
 With early regular transfusion glyburide [Micronase], metformin
therapy, growth and development [Glucophage]),
through childhood are facilitated.  Chloramphenicol (Chloromycetin)
 Organ dysfunction due to iron overload  Vitamin K (phytonadione [Aqua-
results from the excessive amounts of Mephyton]).
iron obtained through the RBC  In affected people, a severe hemolytic
transfusions. episode can result from ingestion of fava
 Regular chelation therapy (eg, via beans.
subcutaneous deferoxamine) has Clinical Manifestations
reduced the complications of iron
overload and prolonged the life of these  Patients are asymptomatic and have
patients. normal hemoglobin levels and
 This disease is potentially curable by reticulocyte counts most of the time.
BMT if the procedure can be performed However, several days after exposure to
before damage to the liver occurs (ie, an offending medication, they may
during childhood). develop pallor, jaundice, and
hemoglobinuria (hemoglobin in the
Glucose 6 Phosphate Dehydrogenase urine). The reticulocyte count rises, and
Deficiency (G6PD) symptoms of hemolysis develop.
 The abnormality in this disorder is in the  Special stains of the peripheral blood
G-6-PD gene; this gene produces an may then disclose Heinz bodies -
enzyme within the RBC that is essential (degraded hemoglobin) within the RBCs.
for membrane stability.  Hemolysis is often mild and self-limited.
 A few patients have inherited an enzyme However, in the more severe
so defective that they have a chronic Mediterranean type of G-6-PD
hemolytic anemia; however, the most deficiency, spontaneous recovery may
common type of defect results in not occur and transfusions may be
hemolysis only when the RBCs are necessary.
stressed by certain situations, such as
fever or the use of certain medications.
 The type of deficiency found in the
Mediterranean population is more severe
than that in the African Caribbean
population, resulting in greater hemolysis
and sometimes in life-threatening
anemia.
 All types of G-6-PD deficiency are
inherited as X-linked defects; therefore,
many more men are at risk than women
Assessment and Diagnostic Finding
Medications that have hemolytic effects
for people with G6PDD:  Diagnosis through Newborn Screening
 Antimalarial agents (eg, chloroquine Medical Management
[Aralen]),
 Sulfonamides (eg, trimethoprim and  The treatment is to stop the offending
sulfamethoxazole [Septra]), medication. Transfusion is necessary
 Nitrofurantoin (eg, Macrodantin), only in thesevere hemolytic state, which
 Analgesics (including aspirin in high is more commonly seen in the
doses), Mediterranean variety of G-6-PD
deficiency.
 Nursing Management
 The patient should be educated about
the disease and given a list of
medications to avoid.
 If hemolysis does develop, nursing
interventions are the same as for
hemolysis from other causes.
POLYCYTHEMIA VERA
 Is a proliferative disorder in which the
myeloid stem cells seem to have
escaped normal control mechanisms.
 The bone marrow is hypercellular, and
the RBC, WBC, and platelet counts in
the peripheral blood are elevated.
However, the RBC elevation is
predominant; the hematocrit can exceed
60%.
 This phase can last for an extended
period (10 years or longer). The spleen
resumes its embryonic function of
hematopoiesis and enlarges.
 Over time, the bone marrow may
become fibrotic, with a resultant inability
to produce as many cells (“burnt out” or
spent phase).
 The disease evolves into myeloid
metaplasia with myelofibrosis or AML in
a significant proportion of patients; this
form of AML is usually refractory to
standard treatments.
 The median survival time exceeds 15
years
Clinical Manifestations
 Patients typically have a ruddy
complexion and splenomegaly (enlarged
spleen).
 The symptoms result from the increased
blood volume (headache, dizziness,
tinnitus, fatigue, paresthesias, and
blurred vision) or from increased blood
viscosity (angina, claudication, dyspnea,
and thrombophlebitis), particularly if the
patient has atherosclerotic blood
vessels.
 Another common and bothersome
problem is generalized pruritus, which
may be caused by histamine release due
to the increased number of basophils.
 Erythromelalgia, a burning sensation in
the fingers and toes, may be reported
and is only partially relieved by cooling.
Assessment and Diagnostic Findings
 Diagnosis is made by finding an elevated
RBC mass (a nuclear medicine
procedure), a normal oxygen saturation
level, and an enlarged spleen.
 Other factors useful in establishing the
diagnosis include elevated WBC and
platelet counts.
 The erythropoietin level is not as low as
would be expected with an elevated
hematocrit; it is normal or only slightly
low.
Complications
 Patients with polycythemia vera are at
increased risk for thromboses resulting
in a CVA (brain attack, stroke) or heart
attack (MI); thrombotic complications are
the most frequent cause of death.
 Bleeding is also a complication, possibly
due to the fact that the platelets (often
very large) are somewhat dysfunctional.
 The bleeding can be significant and can
occur in the form of nosebleeds, ulcers,
and frank gastrointestinal bleeding.
Medical Management
 Phlebotomy is an important part of
therapy and can be performed
repeatedly to keep the hematocrit within
normal range. This is achieved by
removing enough blood (initially 500 mL
once or twice weekly) to deplete the
patient’s iron stores, thereby rendering
the patient iron deficient and
consequently unable to continue to
manufacture RBCs excessively.
 Patients need to be instructed to avoid
iron supplements, including those within
multivitamin supplements.
 If the patient has an elevated uric acid
concentration, allopurinol (Zyloprim) is
used to prevent gouty attacks.
 If the patient develops ischemic
symptoms, dipyridamole (eg, Persantine)
is sometimes used.
 Radioactive phosphorus (32P) or
chemotherapeutic agents (eg,
hydroxyurea [Hydrea]) can be used to
suppress marrow function, but they may
increase the risk for leukemia. Patients
receiving hydroxyurea appear.
 Aspirin is also useful in diminishing pain
associated with erythromelalgia.
 Anagrelide (Agrylin) inhibits platelet
aggregation and can also be useful in
controlling the thrombocytosis
associated with polycythemia vera.
Nursing Management
 The nurse’s role is primarily that of
educator. Risk factors for thrombotic
complications should be assessed, and
patients should be instructed regarding
the signs and symptoms of thrombosis.
 Patients with a history of bleeding are
usually advised to avoid aspirin and
aspirin-containing medications,
becausethese medications alter platelet
function.
 Minimizing alcohol intake should also be
emphasized to further diminish any risk
for bleeding.
 For pruritus, the nurse may recommend
bathing in tepid or cool water, along with
applications of cocoa butter – based
lotions and bath products.
IMMUNE THROMBOCYTOPENIC
PURPURA
 ITP is a disease that affects people of all
ages, but it is more common in children
and young women.
 Other names for the disorder are
idiopathic thrombocytopenic purpura and
immune thrombocytopenia.
 Primary ITP occurs in isolation;
secondary ITP often results from
autoimmune disease, viral infections
(hepatitis C and HIV), and various drugs
(sulfa drugs).
 Primary ITP is defined as a platelet
count less than 100 x 109/L with an
inexplicable absence of a cause for
thrombocytopenia.
Pathophysiology
 ITP is an autoimmune disorder
characterized by adestruction of normal
platelets by an unknown stimulus.
 Antiplatelet antibodies develop in the
blood and bind to the patient’s platelets.
 These antibody-bound platelets are then
ingested and destroyed by the
reticuloendothelial system (RES) or
tissue macrophages.
 The body attempts to compensate for
this destruction by increasing platelet
production within the marrow.
 However, platelet production may also
be impaired as the antibodies may also
induce cell death (via apoptosis) of the
megakaryocytes and thus inhibit platelet
production in the bone marrow.
Clinical Manifestations
 Platelet count of less than 30,000/mm3;
less than 5000/mm3 is not uncommon.
 Easy bruising, heavy menses in females,
and petechiae on the extremities or
trunk.
 Patients with simple bruising or
petechiae (“dry purpura”) tend to have
fewer complications from bleeding than
those with bleeding from mucosal
surfaces, such as the GI tract (including
the mouth) and pulmonary system (e.g.
hemoptysis), which is termed wet
purpura.
 Patients with wet purpura have a greater
risk of life-threatening bleeding than
those with dry purpura.
Assessment and Diagnostic Findings
 Patients should be tested for hepatitis C
and HIV to rule out potential causes.
 Bone marrow aspiration may reveal an
increase in megakaryocytes.
 Platelet count less than 20,000/mm3 is a
common finding.
 Some patients may be infected with
helicobacter pylori, and eradicating the
infection may improve the platelet count.
It is unclear why H. pylori and ITP are
correlated.
Medical Management
 The primary goal of treatment is a “safe”
platelet count. Because the risk of
bleeding typically does not increase until
the platelet count is less than
30,000/mm3.
 Tell patients to stop taking sulfa-
containing medications if they have been
taking any.
 Transfusions are ineffective because the
patient’s antiplatelet antibodies bind with
the transfused platelets, causing them to
be destroyed.
 Aminocaproic acid may be useful for
patients withsignificant mucosal bleeding
since it slows the dissolution of clots.
 IVIG is commonly used to treat ITP.
 Monoclonal antibodies (e.g. rituximab)
since itmay increase platelet counts for
up to 1 year in 20% to 35% of those
treated.
 Thrombopoietin receptor agonists:
o Romiplostim (Nplate) is given weekly
as a subcutaneous injection.
o Eltrombopag (Promacta) is given
orally on an empty stomach since
food may alter drug metabolism.
Surgical Management
 Splenectomy is an alternative treatment
andresults in a sustained normal platelet
count approximately 50% of the time.
 Patients who have undergone
splenectomy are permanently at risk for
sepsisand should receive
pneumococcal, haemophilus influenza
type B, andmeningococcal vaccines
preferably 2-3 weeks before
splenectomy.
Nursing Management
 Assessment of patient’s lifestyle to
determine the risk for bleeding from
activity.
 A careful medication history is also
obtained and be alert for sulfa-containing
medications and aspirin.
 All injections and rectal medications and
rectal temperature measurements
should be avoided because they can
stimulate bleeding.
 Encourage high-fiber diet to prevent
constipation.
 Avoid vigorous flossing of teeth; electric
razors should be used for shaving; soft-
bristled toothbrush should replace stiff-
bristled ones.
 Patient is also counseled to refrain from
vigorous sexual intercourse when the
platelet count is less than 10,000/mm3
HEMOPHILIA
 Defect in clotting mechanisms of blood
 Genetic disorder: X-linked recessive
transmission
 Usually occurs in males
 Classification
o Factor VIII deficiency (Classic
hemophilia); hemophilia A
o Factor IX deficiency (Christmas
disease); hemophilia B
Clinical findings
 Prolonged bleeding from any wound
 Bleeding into the joints (hemarthrosis),  DIC is not an actual disease but a sign of
resulting in pain, deformity, and retarded an underlying condition. It may
growth betriggered by sepsis, trauma, cancer,
 Intracranial hemorrhage shock, abruptio placenta, toxins, allergic
reactions, and other conditions.
Severity of bleeding  The vast majority of cases (two-thirds) of
DIC are initiated by infection or
 Mild
malignancy.
o Factor VIII activity of 5% to 50%
 The severity of DIC is variable, but
o Bleeding with severe trauma or
potentially life threatening.
surgery
 Moderate
o Factor VIII activity of 1% to 5%
o Bleeding with trauma
 Severe
o Factor VIII activity of 1%
o Spontaneous bleeding without
trauma
Treatment

 Control of bleeding
 Prevention of bleeding with use of factor
replacement
 Drugs that replace deficient coagulation
factors
o Factor VIII concentrate from
recombinant DNA
o Factor IX complex contains factor II,
VII, IX, X (concentrated)
 Adjunctive measures
o Aminocaproic acid (Amicar): inhibits
the enzyme that destroy formed fibrin
and increases fibrinogen activity in clot
formation
o Fibrinogen: maintain plasma fibrinogen
levels required for clotting materials
o Thrombin: supplies physiologic levels
of natural material at superficial
bleeding site to control bleeding
Nursing Management
 Advise the patient to watch signs of
bleeding.
 Patient teaching would include the ff:
o Avoid using sharp objects
o Wear shoes at all times
o Avoid contact sports
o Use electric razor
o Cut nails across
o Use soft-bristled toothbrush
Disseminated Intravascular Coagulation
Medical Management
 Treat the underlying cause.Improve
oxygenation, replacing fluids, correcting
electrolyte imbalances, and
administering vasopressor medications
is Qalso important.
 Blood transfusion of platelet concentrate
for serious hemorrhage.
 Cryoprecipitate is given to relace
fibrinogen and factors V and VII.
 Heparin is used for patients with
thrombotic manifestations.
Nursing Management
 Avoid procedures/activities that can
increase intracranial pressure.
 Monitor V/S closely including neurologic
checks.
 Avoid medications that interfere with
platelet function (e.g. aspirin, beta-
lactam antibiotics).
 Avoid rectal probes, and rectal
medications.
 Avoid injections.
 Monitor amount of bleeding.
 Perform oral hygiene carefully by using
sponge tipped swabs, salt/baking soda
mouth rinses.