Assessment of Hematologic Function and
Treatment Modalities
ANATOMIC AND PHYSIOLOGIC OVERVIEW
The hematologic system consists of the blood and
the sites where blood is produced, including the
bone marrow and the reticuloendothelial system
(RES)
Plasma is the fluid portion of blood contains various
proteins, as albumin, globulin, fibrinogen, and other
factors necessary for clotting, as well as electrolytes,
waste products, and nutrients
55% of blood volume is plasma
STRUCTURE AND FUNCTION OF THE
HEMATOLOGIC SYSTEM
★ Blood
Cellular component of blood consists of three
primary cell types:
● erythrocytes (red blood cells [RBCs], red
cells)
● Leukocytes (white blood cells [WBCs])
● thrombocytes (platelets)
40% to 45% of the blood volume
The primary site for hematopoiesis is the bone
marrow
Bone Marrow
Site of hematopoiesis, or blood cell formation
Marrow is one of the largest organs of the body,
making up 4% to 5% of total body weight
Within it are primitive cells called Stem Cells
These stem cells are committed to produce specific
types of blood cells
Lymphoid stem cells produce either T or B
lymphocytes
Myeloid stem cells differentiate into three broad cell
types: erythrocytes, leukocytes, and platelets
★ Blood Cells
Erythrocytes (Red Blood Cells)
Consist primarily of hemoglobin
Contains iron and makes up 95% of the cell mass
Marrow releases slightly immature forms of
erythrocytes, called reticulocytes
Important property of heme is its ability to bind to
oxygen loosely and reversibly
Oxygen readily binds to hemoglobin in the
lungs and is carried as oxyhemoglobin
Lifespan of a normal circulating erythrocyte
is 120 days
Leukocytes (White Blood Cells)
Leukocytes are divided into two general
categories:
● Granulocytes
● Lymphocytes
Total leukocyte count is 4000 to 11,000
cells/mm3
Granulocytes
● presence of granules in the
cytoplasm of the cell
● eosinophils, basophils, and
neutrophils
Agranulocytes
● Monocytes - remove debris as Macrophages
● Lymphocytes – Natural killer (NK) cells
Platelets (Thrombocytes)
Platelets play an essential role in the control of
bleeding
They adhere to the site of injury and to each other,
forming a platelet plug that temporarily stops
bleeding
Substances released from platelet granules activate
coagulation factors in the blood plasma and initiate
the formation of a stable clot composed of fibrin
★ Plasma and Plasma Proteins
Liquid portion of the blood is called plasma
90% of plasma is water.
The remainder consists primarily of plasma proteins;
● clotting factors(particularly fibrinogen);
nutrients, enzymes, waste products, and
gases
Plasma proteins consist primarily of albumin and
globulins
Globulins carry various substances in bound form in
the Circulation
Albumin is particularly important for the maintenance
of fluid balance within the vascular system
★ Reticuloendothelial System (RES)
Composed of special tissue called macrophages
Defend the body against foreign invaders (i.e.,
bacteria and other pathogens) via phagocytosis
Stimulates the inflammatory process and present
antigens to the immune system
The spleen is the site of activity for most
macrophages
Hemostasis
process of preventing blood loss from intact vessels
and of stopping bleeding from a severed vessel,
which requires adequate numbers of functional
platelets
severed blood vessel constricts Circulating platelets
aggregate at the site adhere to the vessel and to
one another unstable hemostatic plug is formed
coagulation process formation of fibrin
ASSESSMENT of the HEMATOLOGIC SYSTEM
HEALTH HISTORY
Family History Assessment Specific to Hematologic
Disorders
Collect family history information on maternal and
paternal relatives from three generations of the
family.
Assess family history for other family members with
histories of blood disorders or episodes of abnormal
bleeding.
If a family history or personal risk is suspected, the
person should be carefully screened for bleeding
disorders prior to surgical procedures.
Patient Assessment Specific to Hematologic
Disorders
Assess for specific symptoms of hematologic
diseases:
● Extreme fatigue (the most common symptom
of hematologic disorders)
● Delayed clotting of blood
● Easy or deep bruising
● Abnormal bleeding (e.g., frequent
nosebleeds)
● Abdominal pain (hemochromatosis) or joint
pain (sickle cell disease)
● Review blood cell counts for abnormalities.
Assess for presence of illness despite low risk for
the illness (e.g., a young adult with a blood clot)
PHYSICAL ASSESSMENT
The physical assessment should be comprehensive
and include careful attention to the skin, oral cavity,
lymph nodes, and spleen
BONE MARROW ASPIRATION AND BIOPSY
Bone marrow aspiration procedure. The posterior
superior iliac crest is the preferred site for bone
marrow aspiration and biopsy because no vital
organs or vessels are nearby.
The patient is placed either in the lateral position
with one leg flexed or in the prone position. The Therapeutic phlebotomy is the removal of a certain
anterior iliac crest or sternum may also be used amount of blood under controlled conditions.

Therapeutic Approaches to Hematologic Patients with elevated hematocrits or excessive iron

Disorders absorption can usually be managed by periodically
removing 1 unit (about 500 mL) of whole blood.
SPLENECTOMY
Over time, this process can produce iron deficiency,
The surgical removal of the spleen leaving the patient unable to produce as many
erythrocytes.
Enlarged spleen may be the site of excessive
destruction of blood cells

Enlarged spleens develop severe thrombocytopenia

THERAPEUTIC APHERESIS

Apheresis is a Greek word meaning “separation.”

Blood is taken from the patient and passed through

a centrifuge, where a specific component is
separated from the blood and removed

HEMATOPOIETIC STEM CELL

TRANSPLANTATION

Hematopoietic stem cells may be transplanted from

either allogeneic or autologous donors

For most hematologic diseases, allogeneic

transplant is more effective

here, stem cells are obtained from a donor whose

cells match those of the patient.

Patient’s own stem cells are harvested and then

used in autologous transplant

THERAPEUTIC PHLEBOTOMY
BLOOD COMPONENT THERAPY
TRANSFUSION
Common Complications Resulting from Long-Term Packed Red
Administration of blood and blood components requires knowledge of Blood Cell Transfusion Therapy
correct administration
techniques and
possible
complications.

Refer to your
LECLAB on Blood
Transfusions for the
procedure!!!

POSTPROCEDURE

1. Obtain vital signs

and auscultate breath Nursing Management for Transfusion Reactions
sounds; compare with baseline measurements. If signs of increased
fluid overload present, consider obtaining prescription for diuretic, as Stop the transfusion. Maintain the IV line with normal saline solution
warranted. through new IV tubing, given at a slow rate.

2. Dispose of used materials properly.
3. Document procedure in patient’s medical record, including patient
assessment findings and tolerance to procedure.
4. Monitor patient for response to and effectiveness of procedure. A
CBC may be ordered 1-6 hours after transfusion to facilitate this
evaluation.
Assess the patient carefully. Compare the vital signs with baseline,
including oxygen saturation. Assess the patient’s respiratory status
carefully. Note the presence of adventitious breath sounds; the use of
accessory muscles; extent of dyspnea; and changes in mental status,
including anxiety and confusion.
Note any chills, diaphoresis, jugular vein distention, and reports of back
pain or urticaria.

5. If patient is at risk for transfusion-associated circulatory overload Notify the primary provider of the assessment findings, and implement
(TACO), monitor closely for 6 hours after transfusion if possible. any treatments prescribed. Continue to monitor the patient’s vital signs
and respiratory, cardiovascular, and renal status.
COMPLICATIONS
● Febrile Nonhemolytic Reaction Notify the blood bank that a suspected transfusion reaction has
● Acute Hemolytic Reaction occurred. Send the blood container and tubing to the blood bank for
● Allergic Reaction repeat typing and culture. The patient’s identity and blood component
● Transfusion-Associated Circulatory Overload (TACO) identifying tags and numbers are verified.
● Bacterial Contamination
● Transfusion-Related Acute Lung Injury (TRALI) If a hemolytic transfusion reaction or bacterial infection is suspected,
● Delayed Hemolytic Reaction the nurse does the following:
● Disease Acquisition
 ● Obtains appropriate blood specimens from the patient
● Collects a urine sample as soon as possible to detect
hemoglobin in the urine
● Documents the reaction according to the institution’s policy
DISEASES POTENTIALLY TRANSMITTED BY BLOOD
TRANSFUSION
● Hepatitis (Viral Hepatitis B, C)
- There is greater risk from pooled blood products and
blood of paid donors than from volunteer donors.
- A screening test detects most hepatitis B and C.
- Transmittal risk for Hepatitis B is estimated at 1:350,000
donated units.
● AIDS (HIV and HTLV)
- Donated blood is screened for antibodies to HIV.
- Transmittal risk is estimated at 1:1.5 million donated
units.
- People with high-risk behaviors (multiple sex partners,
anal sex, IV/injection drug use) and people with signs
and symptoms that suggest AIDS should not donate
blood.
● Cytomegalovirus (CMV)
- Transmittal risk is greater for premature newborns with
CMV antibody–negative mothers and for
immunocompromised recipients who are CMV negative
(e.g., those with acute leukemia, organ or tissue
transplant recipients).
- Blood products rendered “leukocyte reduced” help
reduce transmission of virus.
● Graft-Versus-Host Disease (GVHD)
- GVHD occurs only in severely immunocompromised
recipients (e.g., Hodgkin disease, bone marrow
transplantation).
- Transfused lymphocytes engraft in recipient and attack
host lymphocytes or body tissues; signs and symptoms
are fever, diffuse reddened skin rash, nausea, vomiting,
and diarrhea.
- Preventive measures include irradiating blood products
to inactivate donor lymphocytes (no known radiation
risks to transfusion recipient) and processing donor
blood with leukocyte reduction filters.
● Creutzfeldt-Jakob Disease (CJD)
- CJD is a rare, fatal disease that causes irreversible
brain damage.
- There is no evidence of transmittal by transfusion.
- All blood donors must be screened for positive family
history of CJD.
- Potential donors who spent a cumulative time of 5 years
or more (January 1980 to present) in certain areas of
Europe cannot donate blood; blood products from a
donor who develops CJD are recalled.
PHARMACOLOGIC ALTERNATIVES TO BLOOD TRANSFUSIONS
Growth Factors
- Recombinant technology has provided a means to produce
hematopoietic growth factors necessary for the production of
blood cells within the bone marrow
Erythropoietin
- Effective alternative treatment for patients with chronic anemia
secondary to diminished levels of erythropoietin, as in chronic
renal disease
Granulocyte Colony-Stimulating Factor (G-CSF)
- Cytokine that stimulates the proliferation and differentiation of
myeloid stem cells; a rapid increase in neutrophils is seen
within the circulation
Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)
- Cytokine that is naturally produced by a variety of cells,
including monocytes and endothelial cells
Thrombopoietin
- Cytokine that is necessary for the proliferation of
megakaryocytes and subsequent platelet formation
ANEMIA
Hemoglobin concentration is lower than normal
Reflects the presence of fewer than the normal number of erythrocytes
(i.e., red blood cells [RBCs]) within the circulation
Amount of oxygen delivered to body tissues is also diminished
Not a specific disease state but a sign of an underlying disorder
Most common hematologic condition
CLASSIFICATION
Classification of Anemias
HYPOPROLIFERATIVE ANEMIAS
● Iron Deficiency Anemia - intake of dietary iron is inadequate
● Anemias in Renal Disease - lack of erythropeitin in the kidneys
● Anemia of Inflammation- chronic diseases of inflammation,
infection, and malignancy as causes for this type of anemia
● Aplastic Anemia - decrease in or damage to marrow stem cells
● Megaloblastic Anemias - caused by deficiencies of vitamin B12
or folic acid
HEMOLYTIC ANEMIAS
Sickle Cell Disease - inheritance of the sickle
hemoglobin (HbS) gene, which causes the
hemoglobin molecule to be defective
Thalassemias - group of hereditary anemias
characterized by hypochromia (an abnormal
decrease in the hemoglobin content of
erythrocytes), extreme microcytosis
(smaller-than-normal erythrocytes), hemolysis,
and variable degrees of anemia.
Glucose-6-Phosphate Dehydrogenase
Deficiency - The G-6-PD gene is the source of
the abnormality in this disorder; this gene
produces an enzyme within the erythrocyte that
is essential for membrane stability
Immune Hemolytic Anemias - anemias can result
from exposure of the erythrocyte to antibodies
CLINICAL MANIFESTATIONS
Symptoms may vary on the rapidity,
duration(i.e., its chronicity), the metabolic
requirements of the patient, other concurrent
disorders or disabilities (e.g., cardiac or
pulmonary disease), and complications or
concomitant features of the condition that produced the anemia
More rapidly an anemia develops, the more severe its symptoms
A person who has become gradually anemic, with hemoglobin levels
between 9 and 11 g/dL, usually has fewer or no symptoms other than
slight tachycardia on exertion and possibly fatigue.
Patients with hypothyroidism with decreased oxygen needs may be
completely asymptomatic
Patients with coexistent cardiac, vascular, or pulmonary disease may
develop more pronounced symptoms(e.g., dyspnea, chest pain,
muscle pain or cramping)
ASSESSMENT AND DIAGNOSTIC FINDINGS
● Hematologic studies
● Bone marrow aspiration
● Diagnostic studies may be performed to determine the
presence of underlying chronic illness, such as malignancy, or
the source of any blood loss, such as polyps or ulcers within the
gastrointestinal (GI) tract
COMPLICATIONS
● Heart failure
● Paresthesias
● Delirium
● Patients with underlying heart disease are far more likely to
have angina or symptoms of heart failure
MEDICAL MANAGEMENT
● Directed toward correcting or controlling the cause of the
anemia
● May be replaced with a transfusion of packed red blood cells
ASSESSMENT
Physical Assessment
Weakness, fatigue, and general malaise are common, as are pallor of
the skin and mucous membranes, jaundice; angular cheilosis
(ulcerated corners of the mouth); and brittle, ridged, concave nails may
be present in patients with megaloblastic anemia (characterized by the
presence of abnormally large, nucleated RBCs) or hemolytic anemia,
tongue may be beefy red and sore in megaloblastic anemia, or smooth
and red in iron deficiency anemia
Patients with iron deficiency anemia may infrequently crave ice, starch,
or dirt; this craving is known as pica
Health History
Should include a medication history, history of alcohol intake, Family
history, athletic endeavors, nutritional assessment
● Cardiac status should be carefully assessed.
● GI system
● Neurologic examination
NURSING DIAGNOSES
Based on the assessment data, major nursing diagnoses may include:
Fatigue related to decreased hemoglobin and diminished oxygen
carrying capacity of the blood
Imbalanced nutrition, less than body requirements, related to
inadequate intake of essential nutrients
Activity intolerance related to inadequate hemoglobin and hematocrit
Noncompliance with prescribed therapy
Collaborative Problems/Potential Complications
● Potential complications may include the following:
● Heart failure
● Angina
● Paresthesias
● Confusion
● Injury related to falls
● Depressed mood
Planning and Goals
The major goals for the patient may include decreased fatigue,
attainment or maintenance of adequate nutrition, maintenance of
adequate tissue perfusion, compliance with prescribed therapy, and
absence of complications.
NURSING INTERVENTIONS
● Managing fatigue
● Maintaining adequate nutrition
● Maintaining adequate nutrition
● Promoting adherence with prescribed therapy
● Monitoring and managing potential complications
EVALUATION
1. Reports less fatigue
a. Follows a progressive plan of rest, activity, and exercise
b. Prioritizes activities
c. Paces activities according to energy level
2. Attains and maintains adequate nutrition
a. Eats a healthy diet
b. Develops a meal plan that promotes optimal nutrition
c. Maintains adequate amounts of iron, vitamins, and protein from diet
or supplements
d. Adheres to nutritional supplement therapy when prescribed
e. Verbalizes understanding of rationale for using recommended
nutritional supplements
f. Verbalizes understanding of rationale for avoiding nonrecommended
nutritional supplements
3. Maintains adequate activity level
a. Has vital signs within baseline for patient
b. Has pulse oximetry (arterial oxygenation) value within normal limits
4. Absence of complications
a. Avoids or limits activities that trigger dyspnea, palpitations,
dizziness, or tachycardia
b. Uses rest and comfort measures to alleviate dyspnea
c. Has vital signs within baseline for patient
d. Has no signs of increasing fluid retention
e. Remains oriented to time, place, and situation
f. Remains engaged in social situations, exhibits no signs of
depression
g. Ambulates safely, using assistive devices as necessary
h. Remains free of injury
i. Verbalizes understanding of importance of serial CBC measurements
j. Maintains safe home environment; obtains assistance as necessary
POLYCYTHEMIA
Refers to an increased volume of RBCs
Hematocrit is elevated (more than 55% in males, more than 50% in
females)
Dehydration (decreased volume of plasma) can cause an elevated
hematocrit but not typically to the level to be considered polycythemia.
Polycythemia is classified as either primary or secondary.
Primary polycythemia, also called polycythemia vera, is a proliferative
disorder
SECONDARY POLYCYTHEMIA
Caused by excessive production of erythropoietin.
Occur in response to a reduced amount of oxygen, which acts as a
hypoxic stimulus, as in heavy cigarette smoking, obstructive sleep
apnea (OSA), chronic obstructive pulmonary disease (COPD), or
cyanotic heart disease, or with conditions such as living at a high
altitude or exposure to low levels of carbon monoxide.
It can also result from certain hemoglobinopathies (e.g., hemoglobin
Chesapeake), in which the hemoglobin has an abnormally high affinity
for oxygen or from genetic mutations.
Secondary polycythemia can also occur from neoplasms (e.g., renal
cell carcinoma) that stimulate erythropoietin production, excess
erythropoietin-stimulating agent use, or androgen use.
MEDICAL MANAGEMENT
● Mild, treatment may not be necessary
● When treatment is necessary, it involves treating the primary
condition. Therapeutic phlebotomy may be necessary in
 symptomatic patients to reduce blood viscosity and volume as
well as when the hematocrit is significantly elevated.
● Therapeutic phlebotomy should not be used when the cause for
an elevated
● RBC level is an appropriate, compensatory response to tissue
hypoxia
● Mild, treatment may not be necessary
● When treatment is necessary, it involves treating the primary
condition.
● Therapeutic phlebotomy may be necessary in symptomatic
patients to reduce blood viscosity and volume as well as when
the hematocrit is significantly elevated.
● Therapeutic phlebotomy should not be used when the cause for
an elevated
● RBC level is an appropriate, compensatory response to tissue
hypoxia
NEUTROPENIA
● A neutrophil count of less than 2000/mm3
● results from decreased production of neutrophils or increased
destruction of these cells
● Neutrophils are essential in preventing and limiting bacterial
infection.
● A patient with neutropenia is at increased risk for infection from
both exogenous and endogenous sources
CAUSES OF NEUTROPENIA
DECREASED PRODUCTION OF NEUTROPHILS
Aplastic anemia, due to medications or toxins
Chemotherapy
Metastatic cancer, lymphoma, leukemia
Myelodysplastic syndromes
Radiation therapy
INEFFECTIVE GRANULOCYTOPOIESIS
Megaloblastic anemia
INCREASED DESTRUCTION OF NEUTROPHILS
Bacterial infections
Hypersplenism
Immunologic disorders (e.g., systemic lupus erythematosus)
Medication induceda
Viral disease (e.g., infectious hepatitis, mononucleosis)
CLINICAL MANIFESTATIONS
There are no definite symptoms of neutropenia until the patient
develops an infection. A routine CBC with differential, as obtained after
chemotherapy treatment, can reveal neutropenia before the onset of
infection.
MEDICAL MANAGEMENT
Treatment of the neutropenia varies depending on its cause.
medication induced, the offending agent is stopped immediately, if
possible.
Corticosteroids may be used if the cause is an immunologic disorder.
growth factors such as granulocyte colony-stimulating factor or
granulocyte-macrophage colonystimulating factor can be effective in
increasing neutrophil production
Withholding or reducing the dose of chemotherapy or radiation therapy
may be required
NURSING MANAGEMENT
Nurses in all settings have a crucial role in assessing the severity of
neutropenia and in preventing and managing complications, which
most often include infections.
The Patient at Risk for Infection
At the completion of education, the patient and/or caregiver will be able
to:
State the impact of alterations in neutrophils, lymphocytes,
immunoglobulins on physiologic functioning, ADLs, IADLs, roles,
relationships, and spirituality.
State changes in lifestyle (e.g., diet, activity) or home environment
necessary to decrease risk for infection.
Maintain good hand hygiene technique, oral hygiene, total body
hygiene, and skin integrity.
Avoid cleaning birdcages and litter boxes; consider avoiding garden
work (soil) and fresh flowers in stagnant water.
Maintain a high-calorie, high-protein diet, with fluid intake of 3000 mL
daily (unless fluids are restricted).
Avoid people with infections and crowds.
Perform deep breathing; use incentive spirometer every 4 hours while
awake if mobility is restricted.
Provide adequate lubrication with gentle vaginal manipulation during
sexual intercourse; avoid anal intercourse.
Identify signs and symptoms of infection
Demonstrate how to monitor for signs of infection.
Describe to whom, how, and when to report signs of infection.
Describe appropriate actions to take should infection occur
LYMPHOPENIA
Lymphocyte count less than 1500/mm3
Result from ionizing radiation, long-term use of corticosteroids, uremia,
infections (particularly viral infections), some neoplasms (e.g., breast
and lung cancers, advanced Hodgkin disease), and some
protein-losing enteropathies (in which the lymphocytes within the
intestines are lost)
Mild, it is often without sequelae;
Severe, it can result in bacterial infections (due to low B lymphocytes)
or in opportunistic infections (due to low T lymphocytes).
Tcell lymphocyte depletion is most common, typically due to viral
infection, such as the human immune deficiency virus (HIV).
BLEEDING DISORDERS
Failure of normal hemostatic mechanisms can result in bleeding,
Bleeding is commonly provoked by trauma; however, in certain
circumstances, it can occur spontaneously.
When the cause is platelet or coagulation factor abnormalities, the site
of bleeding can be anywhere in the body.
When the source is vascular abnormalities, the site of bleeding may be
more localized.
Some patients have simultaneous defects in more than one hemostatic
mechanism.
The bone marrow may be stimulated to increase platelet production
(thrombopoiesis) as a reactive response to bleeding
Sometimes, the increase in platelets does not result from increased
production but from a loss in platelet pooling within the spleen.
CLINICAL MANIFESTATIONS
Signs and symptoms of bleeding disorders vary according to the type
of defect.
Abnormalities of the vascular system give rise to local bleeding, usually
into the skin.
Patients with platelet defects develop petechiae, often in clusters;
Bleeding from platelet disorders can be severe.
In contrast, coagulation factor defects do not tend to cause superficial
bleeding, because the primary hemostatic mechanisms are still intact.
Instead, bleeding occurs deeper within the body (e.g., subcutaneous or
intramuscular hematomas, hemorrhage into joint spaces).

MEDICAL MANAGEMENT
Management varies based on the underlying cause of the bleeding
disorder.
transfusions of blood products may be indicated.
Hemostatic agents such as aminocaproic acid (Amicar) can be used to
inhibit this process.
A patient scheduled for an invasive procedure, including a dental
extraction, may need a transfusion prior to the procedure to minimize
the risk of excessive bleeding.
NURSING MANAGEMENT
Patients must be educated to observe themselves carefully and
frequently for signs of bleeding
They need to understand the importance of avoiding activities that
increase the risk of bleeding, such as contact sports.
Examine the skin for petechiae and ecchymoses (bruises) and the
nose and gums for bleeding.
If severe, patients who are hospitalized are monitored for bleeding by
testing all drainage and excreta (feces, urine, emesis, and gastric
drainage) for occult blood as well as obvious blood.
Patients must be educated to observe themselves carefully and
frequently for signs of bleeding
They need to understand the importance of avoiding activities that
increase the risk of bleeding, such as contact sports.
Examine the skin for petechiae and ecchymoses (bruises) and the
nose and gums for bleeding.
If severe, patients who are hospitalized are monitored for bleeding by
testing all drainage and excreta (feces, urine, emesis, and gastric
drainage) for occult blood as well as obvious blood.
SECONDARY THROMBOCYTOSIS
Increased platelet production is the primary mechanism of secondary,
or reactive, thrombocytosis.
The platelet count is above normal, an increase of more than 1
million/mm3 Platelet function is normal; survival time is normal or
decreased.
disorders or conditions can cause a reactive increase in platelets,
including infection, iron deficiency, chronic inflammatory disorders,
malignant disease, acute hemorrhage, and splenectomy.
Treatment is aimed at the underlying disorder. With successful
management, the platelet count usually returns to normal.
Thrombocytopenia (low platelet level) can result from various factors:
1. Decreased production of platelets within the bone marrow
2. Increased destruction of platelets
3. Increased consumption of platelets (e.g., the use of platelets in
clot formation).
CLINICAL MANIFESTATIONS
Bleeding and petechiae usually do not occur with platelet counts
greater than 50,000/mm3
When the platelet count drops to less than 20,000/mm3, petechiae can
appear, along with nasal and gingival bleeding, excessive menstrual
bleeding, and excessive bleeding after surgery or dental extractions.
When the platelet count is less than 5000/mm3, spontaneous,
potentially fatal central nervous system or GI hemorrhage can occur.
If the platelets are dysfunctional as a result of disease (e.g., MDS) or
medications (e.g.,aspirin), the risk of bleeding may be much greater
even when the actual platelet count is not significantly reduced,
because the function of the platelets is altered.
MEDICAL MANAGEMENT
The management of secondary thrombocytopenia is usually treatment
of the underlying disease.
If platelet production is impaired, platelet transfusions may increase the
platelet count and stop bleeding or prevent spontaneous hemorrhage.
In some instances, splenectomy can be a useful therapeutic
intervention, but often it is not an option.
NURSING MANAGEMENT
the nurse considers the cause of the thrombocytopenia, the likely
duration, and the overall condition of the patient.
Education is important, as are interventions to promote patient,safety,
particularly fall prevention in the older adult or patient who is frail.
The interventions for a patient with thrombocytopenia are the same as
those for a patient with cancer who is at risk for bleeding
the nurse considers the cause of the thrombocytopenia, the likely
duration, and the overall condition of the patient.
Education is important, as are interventions to promote patient,safety,
particularly fall prevention in the older adult or patient who is frail.
The interventions for a patient with thrombocytopenia are the same as
those for a patient with cancer who is at risk for bleeding
IMMUNE THROMBOCYTOPENIC PURPURA
More common among children and young women.
Also called idiopathic thrombocytopenic purpura and immune
thrombocytopenia.
Primary ITP occurs in isolation
Secondary ITP often results from autoimmune diseases (e.g.,
antiphospholipid antibody syndrome), viral infections (e.g., hepatitis C,
HIV), and various drugs (e.g., sulfa drugs).
Primary ITP is defined as a platelet count less than 100 × 109/L with
an inexplicable absence of a cause for thrombocytopenia
PATHOPHYSIOLOGY
Antiplatelet antibodies develop in the blood and bind to the patient’s
platelets ingested and destroyed by the reticuloendothelial system
(RES) or tissue macrophages body attempts to compensate for this
destruction by increasing platelet production within the marrow platelet
production also be impaired as the antibodies may also induce cell
death (via apoptosis) of the megakaryocytes inhibit platelet production
within the bone marrow
CLINICAL MANIFESTATIONS
Many patients have no symptoms, and the low platelet count is an
incidental finding (often less than 30,000/mm3; less than 5000/mm3 is
not uncommon).
Common physical manifestations are easy bruising, heavy menses,
and petechiae on the extremities or trunk
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.
Severe thrombocytopenia (platelet count less than 20,000/mm3)
ASSESSMENT AND DIAGNOSTIC FINDINGS
A careful history and physical assessment must be obtained to exclude
causes of the thrombocytopenia and identify evidence of bleeding.
Patients should be tested for hepatitis C and HIV, if not previously done
to rule out these potential causes.
If a bone marrow aspirate is performed, an increase in megakaryocytes
may be seen. The severity of the thrombocytopenia is highly variable,
but a platelet count that is less than 20,000/mm3 is a common finding.
MEDICAL MANAGEMENT
The primary goal of treatment is a “safe” platelet
Count exceeds 30,000/mm3 to 50,000/mm3 may be carefully observed
without additional intervention.
less than 30,000/mm3 or if bleeding occurs, the goal is to improve the
patient’s platelet count rather than to cure the disease.
basis is not of the patient’s platelet count but on the severity of
bleeding
Aminocaproic acid—a fibrinolytic enzyme inhibitor that slows the
dissolution of clots—may be useful for patients with significant mucosal
bleeding refractory to other treatments
The mainstay of short-term therapy is the use of immunosuppressive
agents.
IVIG is also commonly used to treat ITP
NURSING MANAGEMENT
Nursing care includes an assessment of the patient’s lifestyle to
determine the risk of bleeding from activity.
A careful medication history is also obtained, including use of
over-the-counter (OTC) medications, herbs, and nutritional
supplements.
The nurse must be alert for sulfa-containing medications and others
that alter platelet function (e.g., aspirin-based or other NSAIDs).
The nurse assesses for any history of recent viral illness and reports of
headache or visual disturbances, which could be initial symptoms of
intracranial bleeding.
Patients who are admitted to the hospital with wet purpura and low
platelet counts should have a neurologic assessment incorporated into
their routine vital sign measurements.
All injections or rectal medications should be avoided, and rectal
temperature measurements should not be performed, because they
can stimulate bleeding.
PLATELET DEFECTS
Qualitative defects, the number of platelets may be normal but the
platelets do not function normally.
The morphology of platelets is often hypogranular and pale, and may
be larger than normal.
Aspirin may induce a platelet disorder.
NSAIDs can also inhibit platelet function, but the effect is not as
prolonged as with aspirin (Other causes of platelet dysfunction include
end-stage renal disease, possibly from metabolic products affecting
platelet function;
MDS; multiple myeloma (due to abnormal protein interfering with
platelet function); cardiopulmonary bypass; herbal therapy; and other
medications
CLINICAL MANIFESTATIONS
Bleeding may be mild or severe.
elevated PT in the setting of a normal aPTT and platelet count may Hemophilia A is caused by a genetic defect that results in deficient or
suggest factor VII deficiency, defective factor VIII.

whereas an elevated partial thromboplastin time (PTT) in the setting of
a normal PT and platelet count may suggest hemophilia or von
Willebrand disease (vWD).
Ecchymoses are common, particularly on the extremities. Patients with
platelet dysfunction may be at risk for significant bleeding after trauma
or invasive procedures
MEDICAL MANAGEMENT
If the platelet dysfunction is caused by medication, its use should be
stopped, if possible
If platelet dysfunction is marked, bleeding can often be prevented by
transfusion of normal platelets before invasive procedures.
Hemophilia B (also called Christmas disease) stems from a genetic
defect that causes deficient or defective factor IX
Both types of hemophilia are inherited as X-linked traits, so most
affected people are males; females can be carriers but are almost
always asymptomatic
Hemophilia is recognized in early childhood, usually in the toddler age
group. However, patients with mild hemophilia may not be diagnosed
until they experience severe trauma (e.g., a high school football injury)
or surgery.
CLINICAL MANIFESTATIONS
Hemorrhages into various parts of the body

Antifibrinolytic agents (e.g., aminocaproic acid) may be required to
prevent significant bleeding after such procedures;
Desmopressin (DDAVP) can decrease the duration of bleeding in some
situations and improve hemostasis (Levi et al., 2016).
NURSING MANAGEMENT
Severe and can occur even after minimal trauma.
The frequency and severity of the bleeding depend on the degree of
factor deficiency as well as the intensity of the precipitating trauma.
About 75% of all bleeding in patients with hemophilia occurs into joints.
The most commonly affected joints are the knees, elbows, ankles,
shoulders, wrists, and hips.

Instruct to avoid substances that can diminish platelet function, such as

certain OTC medications, some herbal therapies, nutritional
supplements, and alcohol.

inform their health care providers (including dentists) of the underlying

condition before any invasive procedure is performed so that
appropriate steps can be initiated to diminish the risk of bleeding.

Maintaining good oral hygiene is very important so that gingival

bleeding can be minimized.

HEMOPHILIA

CLINICAL MANIFESTATIONS
● Bleeding is commonly associated with dental extraction
● Spontaneous hematuria and GI bleeding can also occur
● Surgical procedures typically result in excessive bleeding at the
surgical site
● Falls carry significant risk for morbidity in adults
MEDICAL MANAGEMENT
Recombinant forms of factor VIII and X concentrates are available and
decrease the need for using factor concentrates, or, more infrequently,
fresh-frozen plasma.
Other therapeutic options include administering recombinant factor VIIa
(Novoseven, Novo Nordixk) or activated prothrombin complex
concentrates (IaPCC, FEIBA)
Aminocaproic acid inhibits fibrinolysis and therefore stabilizes the clot
NURSING MANAGEMENT
Diagnosed as children, hey often require assistance in coping with the
condition because it is chronic, places restrictions on their lives, and is
an inherited disorder that can be passed to future generations
Patients need extensive education about activity restrictions and
self-care measures to diminish the chance of hemorrhage and
complications of bleeding
Patients and family members are instructed how to administer the
factor concentrate at home at the first sign of bleeding so that bleeding
is minimized and complications avoided
During hemorrhagic episodes, the extent of bleeding must be assessed
Carefully. Close observation and systematic assessment for emergent
complications
Warm baths promote relaxation, improve mobility, and lessen pain
Genetic testing and counseling regarding having children
VON WILLEBRAND DISEASE (VWD)
Inherited as a dominant trait, vWD is a common bleeding disorder that
affects males and females equally
Deficiency of vWF, which is necessary for factor VIII activity
Type 1, the most common, is characterized by decreases in structurally
normal vWF.
Type 2 shows variable qualitative defects based on the specific vWF
subtype involved.
Type 3 is very rare (less than 5% of cases) and is characterized by a
severe vWF deficiency as well as significant deficiency of factor VIII
CLINICAL MANIFESTATIONS
Bleeding tends to be mucosal.
Recurrent nosebleeds, easy bruising, heavy menses, prolonged
bleeding from cuts, and postoperative bleeding.
Massive soft tissue or joint hemorrhages are not often seen, unless the
patient has severe type 3 vWD.
As the laboratory values fluctuates, so does the bleeding.
Assessment and Diagnostic Findings
The diagnosis is established by the patient meeting all of these criteria:
1. A history of bleeding since childhood
2. Reduced vWF activity in plasma
3. History of bleeding within the family
Laboratory test results show a normal platelet count but a prolonged
bleeding time and a slightly prolonged aPTT.
Ristocetin cofactor, or vWF collagen binding assay, which measures
vWF activity
vWF antigen, factor VIII, and, for patients with suspected type 2
defects, vWF multimers, which measure specific subtypes of vWF.
MEDICAL MANAGEMENT
The goal of treatment is to replace the deficient protein (e.g., vWF or
factor VIII) at the time of spontaneous bleeding or prior to an invasive
procedure to prevent subsequent bleeding.
Desmopressin, a synthetic vasopressin analogue, can be used to
prevent bleeding associated with dental or surgical procedures
Replacement products include Humate-P and Alphanate, which are
commercial concentrates of vWF and factor VIII
Aminocaproic acid is useful in managing mild forms of mucosal
bleeding
Estrogen–progesterone compounds may diminish the extent of
menses.
Platelet transfusions are useful when there is significant bleeding.
While rich in vWF and Factor VIII, cryoprecipitate is typically used only
in emergent situations
ACQUIRED COAGULATION DISORDERS
Liver Disease
Hepatic dysfunction (due to cirrhosis, tumor, or hepatitis) can result in
diminished amounts of the factors needed to maintain coagulation and
hemostasis.
Prolongation of the PT, unless it is caused by vitamin K deficiency, may
indicate severe hepatic dysfunction. Although bleeding is usually minor
(e.g., ecchymoses), these patients are also at risk for significant
bleeding, related especially to trauma or surgery.
Transfusion of fresh-frozen plasma may be required to replace clotting
factors and to prevent or stop bleeding.
Patients may also have life-threatening hemorrhage from peptic ulcers
or esophageal varices. In these cases, replacement with fresh-frozen
plasma, PRBCs, and platelets is usually required.
VITAMIN K DEFICIENCY
The synthesis of many coagulation factors depends on vitamin K.
Vitamin K deficiency is common in malnourished patients.
Prolonged use of some antibiotics decreases the intestinal flora that
produces vitamin K
Administration of vitamin K (phytonadione [Mephyton]), either orally or
as a subcutaneous injection, can correct the deficiency quickly;
adequate synthesis of coagulation factors is reflected by normalization
of the PT.
DISSEMINATED INTRAVASCULAR COAGULATION
Disseminated intravascular coagulation (DIC) is not an actual disease
but a sign of an underlying condition.
DIC may be triggered by sepsis, trauma, cancer, shock, abruptio
placentae, toxins, allergic reactions, and other conditions the vast
majority (two thirds) of cases of DIC are initiated by an infection or a
malignancy
The severity of DIC is variable, but it is potentially life-threatening.
CLINICAL MANIFESTATIONS

ASSESSMENT AND DIAGNOSTIC FINDINGS

 Nurses need to be aware of which patients are at risk for DIC
Medical Management
Patients need to be assessed thoroughly and frequently forsigns and
The most important factor in managing DIC is aggressively treating the symptoms of thrombi and bleeding and monitored for any progression
underlying cause of these signs
Lab values must be monitored frequently, not only for the actual result
Correcting the secondary effects of tissue ischemia by improving but to note trends over time as well as the rate of change in values.
oxygenation, replacing fluids, correcting electrolyte imbalances, and
administering vasopressor medications is also important. Assessment and interventions should target potential sites of
end-organ damage
If serious hemorrhage occurs, the depleted coagulation factors and
platelets may be replaced to reestablish the potential for normal THROMBOTIC DISORDERS
hemostasis and thereby diminish bleeding.
Altered balance within the normal hemostasis process, causing
Cryoprecipitate is given to replace fibrinogen and factors V and VII. excessive thrombosis that may be arterial (due to platelet aggregation)
Administering fresh frozen plasma replaces coagulation factors. or venous (composed of platelets, red cells, and thrombin).
Platelets are transfused to correct severely low platelet levels, control
bleeding Decreased clotting inhibitors within the circulation, altered hepatic, lack
of fibrinolytic enzymes, and
All management strategies must be tortuous or atherosclerotic
individualized to the specific patient, vessels
underlying cause of DIC, and response to
interventions. Thrombosis may occur as
an initial manifestation of
NURSING MANAGEMENT an occult malignancy or as
a complication from a pre-
Nurses need to be aware of which patients existing cancer.
are at risk for DIC
Hyperhomocysteinemia,
Patients need to be assessed thoroughly antithrombin (AT)
and frequently for signs and symptoms of deficiency, protein C
thrombi and bleeding and monitored for any deficiency, protein S
progression of these signs deficiency, activated protein
C (APC) resistance, and
Lab values must be monitored frequently, factor V Leiden deficiency -
not only for the actual result but to note hypercoagulable states
trends over time as well as the rate of
change in values.

Assessment and interventions should target

potential sites of end-organ damage
A recent study found that taking aspirin after completing standard
anticoagulation therapy for treating VTE reduced the risk of recurrent
thrombosis
With some conditions, or with repeated thrombosis, lifelong
anticoagulant therapy is necessary.
HYPERHOMOCYSTEINEMIA
Homocysteine can promote platelet aggregation.
Increased plasma levels of homocysteine are a significant risk factor
for VTE (e.g., deep vein thrombosis [DVT], pulmonary embolism [PE]),
recurrent VTE, and arterial thrombosis (e.g., ischemic stroke, ACS)
Hyperhomocysteinemia can be hereditary, or it can result from a
nutritional deficiency of folate and, to a lesser extent, of vitamins B12
and B6, because these vitamins are cofactors in homocysteine
metabolism.
In hyperhomocysteinemia, the endothelial lining of the vessel walls is
denuded, which can precipitate thrombus formation.
Smoking causes low levels of vitamin B6 and B12 and folate
ANTITHROMBIN DEFICIENCY
AT is a protein that inhibits thrombin and certain coagulation factors,
and it may also play a role in diminishing inflammation within the
endothelium of blood vessels.
Most commonly a hereditary condition that can cause venous
thrombosis common sites for thrombosis are the deep veins of the leg
and the mesentery.
Patients tend to exhibit heparin resistance; require greater amounts of
heparin
AT deficiency can also be acquired by four mechanisms:
1. Accelerated consumption of AT (as in DIC)
2. Reduced synthesis of AT (as in hepaticdysfunction)
3. Increased excretion of AT (as in nephrotic syndrome)
4. Medication induced (e.g., estrogens, L-asparaginase)
PROTEIN C DEFICIENCY
Protein C is a vitamin K–dependent enzyme synthesized in the liver;
when activated, it inhibits coagulation.
Deficient, the risk of thrombosis increases, and thrombosis can often
occur spontaneously.
Individuals with Protein C deficiency are at higher risk for recurrent PE
Complication of anticoagulation management is warfarin-induced skin
necrosis.
Treatment with purified protein C concentrate is sometimes indicated.
PROTEIN S DEFICIENCY
Protein S is another natural anticoagulant normally produced by the
liver.
Like patients with protein C deficiency, those with protein S deficiency
have a greater risk of recurrent venous thrombosis early in life, and
also with recurrent PE
Thromboses most commonly occur in the axillary, mesenteric, and
cerebral veins.
Warfarin-induced skin necrosis is possible.
Acquired protein S deficiency can also occur.
Pregnancy, DIC, liver disease, nephritic syndrome, HIV infection, and
the use of L-asparaginase have all been associated with reduced
protein S levels.
ACTIVATED PROTEIN C RESISTANCE AND FACTOR V LEIDEN
MUTATION
APC resistance is a common condition that can occur with other
hypercoagulable states.
APC is an anticoagulant, and resistance to APC increases the risk of
venous thrombosis.
Factor V Leiden mutation is the most common cause of inherited
hypercoagulability in Caucasians,
Factor V Leiden mutation synergistically increases the risk of
thrombosis in patients with other risk factors (e.g., the use of oral
contraceptives, hyperhomocysteinemia, increased age).
The duration of anticoagulation is based upon the coexistence of other
risk factors for thrombi formation
MEDICAL MANAGEMENT
The primary method of treating thrombotic disorders is anticoagulation.
However, in thrombophilic conditions, when to treat (prophylaxis or not)
and how long to treat (lifelong or not) can be controversial.
Anticoagulation therapy is not without risks; the most significant risk is
bleeding.
PHARMACOLOGIC THERAPY
1. Heparin
2. Warfarin (Coumadin)
3. Thrombin and Factor Xa Inhibitors
4. Aspirin
NURSING MANAGEMENT
Patients with thrombotic disorders should avoid activities that lead to
circulatory stasis (e.g., immobility, crossing the legs).
Exercise, especially ambulation, should be performed frequently
throughout the day, particularly during long trips by car or plane.
Anti-embolism stockings are often prescribed
Surgery further increases the risk of thrombosis.
Medications that alter platelet aggregation, such as low-dose aspirin or
clopidogrel (Plavix), may be prescribed.
Anticoagulants such as warfarin
Assessed for concurrent risk factors for thrombosis and should avoid
them if possible.
Tobacco and nicotine products should be avoided
In many instances, younger patients with thrombophilia may not
require prophylactic anticoagulation; however, with concomitant risk
factors (e.g., pregnancy), increasing age, or subsequent thrombotic
events,prophylactic or long-term anticoagulation therapy may be
required.
Accurate health history can be extremely useful and can help guide the
selection of appropriate therapeutic interventions.
Patients need to understand risk factors for thrombosis and what they
can do to diminish or reduce them, such asavoiding smoking, using
alternative forms of contraception, increasing mobility, and maintaining
a healthy weight.
Assessed for concurrent risk factors for thrombosis and should avoid
them if possible.
Tobacco and nicotine products should be avoided
In many instances, younger patients with thrombophilia may not
require prophylactic anticoagulation; however, with concomitant risk
factors (e.g., pregnancy), increasing age, or subsequent thrombotic
events,prophylactic or long-term anticoagulation therapy may be
required.
Accurate health history can be extremely useful and can help guide the
selection of appropriate therapeutic interventions.
Patients need to understand risk factors for thrombosis and what they
can do to diminish or reduce them, such asavoiding smoking, using
alternative forms of contraception, increasing mobility, and maintaining
a healthy weight.

When a patient with a thrombotic disorder is hospitalized, frequent

assessments should be performed for signs and symptoms of
beginning thrombus formation, particularly in the legs (DVT) and lungs
(PE).

Ambulation or range-of-motion exercises as well as the use of

antiembolism stockings

Properly fitted graduated compression stockings may reduce pain and

edema associated with the acute stage of DVT