Management of Patients

with Nonmalignant
Hematologic Disorders
Frequently Used Lab Tests in
Hematology
Test Normal Range Description Indications/Comm
ents

RBC count Male: 4.7 – 6.1 Carries

million/mm3 hemoglobin;
survival time,
Female: 4.2 – 5.4 120 days
million/mm3
Hemoglobin (Hgb) M: 13.5–17.5 g/dL Delivers O2 Decreased in
F: 11.5–15.5 g/dL through circulation anemia; increased
to body tissues in
and returns CO2 polycythemia
from tissues to
lungs
Hematocrit (Hct) M: 40–52% Indicates relative Usually three times
F: 36–48% proportions of the Hgb
plasma and RBCs
(volume of
RBCs/L whole
blood)

Test Normal Range Description Indications/Comm

ents

Mean corpuscular 81–96 μm3 Indicates size of If < 80, cells

volume RBCs; very useful are microcytic;
(MCV) in differentiating if > 100, cells
types of anemia are macrocytic
Mean corpuscular 33–36 g/dL Average
hemoglobin concentration of
concentration Hgb in
(MCHC) RBCs; independent
of cell size
Red cell 11–14.5% Measures degree
distribution width of variation in size
(RDW) of RBCs
Reticulocyte 0.5–1.5% Measure of Indicates marrow’s
count marrow production response to
of erythrocytes; anemia
1% of RBC mass is (when anemia is
produced daily (to present,
replace the 1% of reticulocyte level
old cells that should rise)
die)
Test Normal Range Description Indications/Comm
ents

Platelets 150,000– Total number of Thrombocytopenia

400,000/mm3 platelets in : < 20,000/mm3,
circulation; serious; <
average life span, 10,000/mm3,
7–10 days potentially life-
threatening

White blood cells 4,500–11,000/mm3 Total WBC count Increased during

(WBCs) an infection.
Prothrombin time Varies (compare Measure time Increased in liver
(PT) with control), elapsed until clot disease,
11–12.5 sec forms; measures disseminated
extrinsic and intravascular
common pathways coagulation
(DIC),
obstructive
biliary disease,
clotting factor
depletion,
warfarin
(Coumadin) use
Test Normal Range Description Indications/Comm
ents

Partial Varies (compare Surface active Increased in

thromboplastin with control): agent added to clotting factor
time 25–35 sec plasma; measures depletion,
(PTT) time elapsed DIC, liver disease,
until clot forms; biliary
measures obstruction,
intrinsic and circulating
common anticoagulants
(heparin)
pathways
Thrombin time Varies (compare Tests conversion Time to clot is
(TT) with control), of fibrinogen to inversely
8–11 sec fibrin proportional to
fibrinogen level
Fibrinogen 170–340 mg/100 Measurement of Decreased in
mL fibrinogen bleeding
concentration disorders,
within plasma pregnancy,
available for malignancy,
conversion to inflammatory
fibrin clot disease

Test Normal Range Description Indications/Comments

Lymphocytes 20–50% (1,500– Integral component <1,500: lymphopenia; >4,000:

5,500/mm3) of immune lymphocytosis; increased in
system convalescent phase after bacterial or
viral infection, lymphoproliferative
disease
Test Normal Range Description Indications/Comments

D-dimer 0–0.5μg/mL Measures the Increased with fibrinolytic

amount of activity, rheumatoid
fragments arthritis, ovarian cancer
of fibrin when it is
lysed (broken
down); useful for
distinguishing
fibrinolysis from
fibrinogenolysis
Fibrin <10 μg/mL Byproduct of >40 μg/mL indicates DIC
degradation fibrinolysis
products
(FDP)
Monocytes 1–10% (100–800/mm3) Enter tissue as Increased in acute and chronic infection,
macrophages; inflammation, some
phagocytosis myeloproliferative disorders,
chronic myelomonocytic
leukemia (CMML)
Neutrophils 40–75% (2,500– Essential in If >8,000: infection, some
7,500/mm3) preventing/limitin inflammatory states, stress,
g bacterial steroids, other drugs,
infection; myeloproliferative disease
average Absolute neutrophil count (ANC)
life span: <500: increased risk for infection;
2–4 hr ANC <100: infection certain

Eosinophils 0–6% (0–440/mm3) Involved in Increased in allergic states, medications,

allergic reactions parasites, chronic myeloid leukemia
(neutralizes (CML), metastatic/ necrotic tumors
histamine); digest
foreign proteins
Basophils 0–2% (0–200/mm3) Contain histamine; Increase is very rare (CML)
integral part of
hypersensitivity
reactions

Iron Deficiency Anemia

Iron Deficiency Anemia (IDA)

► Ittypically results when the intake of dietary iron is inadequate for hemoglobin
synthesis.
► Itis 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 menorrhaghia,
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

► Anemia may be a sign of a curable gastrointestinal cancer or of uterine fibroid

tumors. Stool specimens should be tested for occult blood.
► People 50 years of age or older should have a colonoscopy, endoscopy, or other
examination of the gastrointestinal tract to detect ulcerations, gastritis,
polyps, or cancer.
► Severaloral iron preparations—ferrous sulfate, ferrous gluconate, and ferrous
fumarate—are available for treating iron deficiency anemia.
Nursing Management

► Preventiveeducation is important, because iron deficiency anemia is common in

menstruating and pregnant women.
► Food sources high in iron include organ meats (beef or calf’s liver, chicken liver),
other meats, beans (black, pinto, and garbanzo), leafy green vegetables, raisins,
and molasses.
► Taking iron-rich foods with a source of vitamin C enhances the absorption of iron.
► Because iron is best absorbed on an empty stomach, patients should be advised to
take the supplement an hour before meals.
► Other patients have difficulty taking iron supplements because of gastrointestinal
side effects (primarily constipation, but also cramping, nausea, and vomiting).
Advise patient to increase fiber intake.
► Use a straw when taking fluid preparation of iron.
Aplastic Anemia
Aplastic Anemia

► Aplasticanemia is a 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.
Causes

► Aplastic anemia can be congenital or acquired, but most cases are idiopathic
(ie, without apparent cause).
► Infections
and pregnancy can trigger it, or it may be caused by certain
medications, chemicals, or radiation damage.
► Agents that regularlyproduce marrow aplasia include benzene and benzene
derivatives (eg, airplane glue).
► Certain toxic materials, such as inorganic arsenic and several pesticides
(including DDT, which is no longer used or available in the United States),
have also been implicated as potential causes.
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
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.
► Inan 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.

Megaloblastic Anemia

► 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.
► Anothercause 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

► Patientswith 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

► The classic method of determining the cause of vitamin B12 deficiency is the
Schilling test, in which the patient receives a small oral dose of radioactive
vitamin B12, followed in a few hours by a large, nonradioactive parenteral
dose of vitamin B12 (this aids in renal excretion of the radioactive dose).
► Ifthe 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 ileal
disease or pernicious anemia.
Assessment and Diagnostic Findings

► 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

► Folatedeficiency is treated by increasing the amount of folic acid in the diet

and 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.
► Ifsensation 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.
► Patientsmust 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

► Sicklecell anemia is a severe hemolytic anemia that results from inheritance

of the sickle hemoglobin gene.
► Thisgene 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
► Ifischemia 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.

► Jaundiceis 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.
► Aplasticcrisis 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.
► Sideeffects 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 erythropoiesis from
hemolysis.
► Infections must be treated promptly with appropriate antibiotics;
infection remains a major cause of death in these patients.

Medical Management

► NSAIDssuch as aspirin is given to relieve the patient from pain. It also relieves
potential thrombosis.
Nursing Management
► Painmanagement – NSAIDs administration, relaxation techniques, and
breathing exercises.
► Prevent the occurrence of infection by giving appropriate antibiotics.
► Providingthe patient with opportunities to make decisions about daily care
may increase the patient’s feelings of control.
► Monitor and manage potential complications such as:
► Leg ulcers
► Priapism leading to impotence
► Chronic pain and substance abuse

Thalassemia
Thalassemia
► The thalassemias 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.
► This increases the rigidity of the RBCs and thus the premature
destruction of these cells.

Types of Thalassemia
1. Alpha Thalassemia -
► Thalassemias occur mainly in people 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.
Thalassemia Major

► Thalassemiamajor (Cooley’s anemia) is characterized by severe anemia,

marked hemolysis, and ineffective erythropoiesis (production of RBCs).
► With early regular transfusion therapy, growth and development through
childhood are facilitated.
► Organ dysfunction due to iron overload results from the excessive amounts of
iron obtained through the RBC transfusions.
► Regularchelation therapy (eg, via subcutaneous deferoxamine) has reduced
the complications of iron overload and prolonged the life of these patients.
► Thisdisease is potentially curable by BMT if the procedure can be performed
before damage to the liver occurs (ie, during childhood).
Glucose 6 Phosphate Dehydrogenase
Deficiency
G6PDD
► Theabnormality in this disorder is in the G-6-PD gene; this gene
produces an enzyme within the RBC that is essential for membrane
stability.
►A few patients have inherited an enzyme so defective that they have a
chronic hemolytic anemia; however, the most common type of defect
results in hemolysis only when the RBCs are stressed by certain
situations, such as fever or the use of certain medications.
► Thetype 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.
► Alltypes of G-6-PD deficiency are inherited as X-linked defects;
therefore, many more men are at risk than women.

Medications that have hemolytic effects

for people with G6PDD:
► Antimalarial agents (eg, chloroquine [Aralen]),
► Sulfonamides (eg, trimethoprim and sulfamethoxazole [Septra]),
► Nitrofurantoin (eg, Macrodantin),
► Analgesics (including aspirin in high doses),
► Thiazide diuretics (eg, hydrochlorothiazide [Hydro- DIURIL],
chlorothiazide [Diuril]),
► Oral hypoglycemic agents (eg, glyburide [Micronase], metformin
[Glucophage]),
► Chloramphenicol (Chloromycetin), and
► Vitamin K (phytonadione [Aqua-Mephyton]).
► Inaffected people, a severe hemolytic episode can result from
ingestion of fava beans.

Clinical Manifestations
► Patients are asymptomatic and have normal hemoglobin
levels and reticulocyte counts most of the time. However,
several days after exposure to an offending medication,
they may develop pallor, jaundice, and hemoglobinuria
(hemoglobin in the urine). The reticulocyte count rises,
and symptoms of hemolysis develop.
► Specialstains of the peripheral blood may then disclose
Heinz bodies - (degraded hemoglobin) within the RBCs.
► Hemolysisis often mild and self-limited. However, in the
more severe Mediterranean type of G-6-PD deficiency,
spontaneous recovery may not occur and transfusions may
be necessary.
Heinz Bodies
Assessment and Diagnostic Finding

► Diagnosis through Newborn Screening

Medical Management

►The treatment is to stop the offending

medication. Transfusion is necessary only in the
severe hemolytic state, which is more commonly
seen in the 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
Polycythemia Vera
► Polycythemia vera, or primary polycythemia, is a proliferative disorder
in which the myeloid stem cells seem to have escaped normal control
mechanisms.
► Thebone 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%.
► Thisphase 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

► Patientswith 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.
► Patientsneed to be instructed to avoid iron supplements, including those
within multivitamin supplements.
► Ifthe patient has an elevated uric acid concentration, allopurinol (Zyloprim) is
used to prevent gouty attacks.
► Ifthe 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.
Medical Management

► 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.
► Patientswith a history of bleeding are usually advised to
avoid aspirin and aspirin-containing medications, because
these 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)
What is ITP?

► ITPis a disease that affects people of all ages, but it is more common in
children and young women.
► Othernames 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 a destruction 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.
► Patientswith 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.
► Tellpatients 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 with significant mucosal bleeding
since it slows the dissolution of clots.
► IVIG is commonly used to treat ITP.
► Monoclonal antibodies (e.g. rituximab) since it may increase platelet counts for
up to 1 year in 20% to 35% of those treated.

Continuation…

► Thrombopoietin receptor agonists:

► Romiplostim (Nplate) is given weekly as a subcutaneous injection.
► Eltrombopag(Promacta) is given orally on an empty stomach since food may alter
drug metabolism.
Surgical Management

► Splenectomy is an alternative treatment and results in a sustained normal

platelet count approximately 50% of the time.
► Patientswho have undergone splenectomy are permanently at risk for sepsis
and should receive pneumococcal, haemophilus influenza type B, and
meningococcal 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.
► Allinjections 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
Description

►Defect in clotting mechanisms of blood

►Genetic disorder: X-linked recessive
transmission
►Usually occurs in males
►Classification
 ►Factor VIII deficiency (Classic hemophilia);
hemophilia A
►Factor IX deficiency (Christmas disease);
hemophilia B
Clinical findings

►Prolonged bleeding from any

wound
 ►Bleeding into the joints
(hemarthrosis), resulting in pain,
deformity, and retarded growth
►Intracranial hemorrhage
Severity of bleeding
►Mild
►Factor VIII activity of 5% to 50%
►Bleeding with severe trauma or surgery

►Moderate
 ►Factor VIII activity of 1% to 5%
►Bleeding with trauma

►Severe
►Factor VIII activity of 1%
►Spontaneous bleeding without trauma

Treatment

►Control of bleeding
►Prevention of bleeding with use of
factor replacement
►Drugs that replace deficient coagulation
factors
►Factor VIII concentrate from recombinant
DNA
►Factor IX complex contains factor II, VII, IX, X
(concentrated)
►Adjunctive measures
►Aminocaproic acid (Amicar): inhibits the
enzyme that destroy formed fibrin and
increases fibrinogen activity in clot formation
►Fibrinogen: maintain plasma fibrinogen levels
required for clotting materials
 ►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:

• Avoid using sharp objects

• Wear shoes at all times
• Avoid contact sports
 • Use electric razor
• Cut nails across
• Use soft-bristled toothbrush

Disseminated Intravascular Coagulation

(DIC)
What is DIC?

► DIC is not an actual disease but a sign of an underlying condition. It may be

triggered by sepsis, trauma, cancer, shock, abruptio placenta, toxins, allergic
reactions, and other conditions.
► Thevast majority of cases (two-thirds) of DIC are initiated by infection or
malignancy.
► The severity of DIC is variable, but potentially life-threatening.
Assessment and Diagnostic Findings
Medical Management

► Treat the underlying cause.

► Improve oxygenation, replacing fluids, correcting electrolyte imbalances, and
administering vasopressor medications is also 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,
betalactam 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.