Pleural effusion, a collection of fluid in the pleural space, is rarely a primary disease process; it is

usually secondary to other diseases. Normally, the pleural space contains a small amount of fluid (5 to
15 mL), which acts as a lubricant that allows the pleural surfaces to move without friction (Fig. 23-4).
Pleural effusion may be a complication of heart failure, TB, pneumonia, pulmonary infections
(particularly viral infections), nephrotic syndrome, connective tissue disease, pulmonary embolus, and
neoplastic tumors. The most common malignancy associated with a pleural effusion is bronchogenic
carcinoma.

Pathophysiology

In certain disorders, fluid may accumulate in the pleural space to a point at which it becomes
clinically evident. This almost always has pathologic significance. The effusion can be a relatively clear
fluid, or it can be bloody or purulent. An effusion of clear fluid may be a transudate or an exudate. A
transudate (filtrate of plasma that moves across intact capillary walls) occurs when factors influencing
the formation and reabsorption of pleural fluid are altered, usually by imbalances in hydrostatic or
oncotic pressures. The finding of a transudative effusion generally implies that the pleural membranes
are not diseased. A transudative effusion most commonly results from heart failure. An exudate
(extravasation of fluid into tissues or a cavity) usually results from inflammation by bacterial products or
tumors involving the pleural surfaces.

Clinical Manifestations

Usually, the clinical manifestations are caused by the underlying disease. Pneumonia causes
fever, chills, and pleuritic chest pain, whereas a malignant effusion may result in dyspnea, difficulty lying
flat, and coughing. The severity of symptoms is determined by the size of the effusion, the speed of its
formation, and the underlying lung disease. A large pleural effusion causes dyspnea (shortness of
breath). A small to moderate pleural effusion causes minimal or no dyspnea.

Assessment and Diagnostic Findings

Assessment of the area of the pleural effusion reveals decreased or absent breath sounds,
decreased fremitus, and a dull, flat sound on percussion. In the case of an extremely large pleural
effusion, the assessment reveals a patient in acute respiratory distress. Tracheal deviation away from
the affected side may also be apparent.

Physical examination, chest x-ray, chest CT, and thoracentesis confirm the presence of fluid. In
some instances, a lateral decubitus x-ray is obtained. For this x-ray, the patient lies on the affected side
in a side-lying position. A pleural effusion can be diagnosed because this position allows for the “layering
out” of the fluid, and an air–fluid line is visible.

Pleural fluid is analyzed by bacterial culture, Gram stain, acid-fast bacillus stain (for TB), red and
white blood cell counts, chemistry studies (glucose, amylase, lactate dehydrogenase, protein), cytologic
analysis for malignant cells, and pH. A pleural biopsy also may be performed as a diagnostic tool.

Medical Management
 The objectives of treatment are to discover the underlying cause of the pleural effusion; to
prevent reaccumulation of fluid; and to relieve discomfort, dyspnea, and respiratory compromise.
Specific treatment is directed at the underlying cause (eg, heart failure, pneumonia, cirrhosis). If the
pleural fluid is an exudate, more extensive diagnostic procedures are performed to determine the cause.
Treatment for the primary cause is then instituted.

Thoracentesis is performed to remove fluid, to obtain a specimen for analysis, and to relieve
dyspnea and respiratory compromise (see Chapter 21). Thoracentesis may be performed under
ultrasound guidance. Depending on the size of the pleural effusion, the patient may be treated by
removing the fluid during the thoracentesis procedure or by inserting a chest tube connected to a
water-seal drainage system or suction to evacuate the pleural space and re-expand the lung.

However, if the underlying cause is a malignancy, the effusion tends to recur within a few days
or weeks. Repeated thoracenteses result in pain, depletion of protein and electrolytes, and sometimes
pneumothorax. Once the pleural space is adequately drained, a chemical pleurodesis may be performed
to obliterate the pleural space and prevent reaccumulation of fluid. Pleurodesis may be performed using
either a thoracoscopic approach or a chest tube. A chemically irritating agent (eg, talc or another
chemical irritant) is instilled or aerosolized into the pleural space. With the chest tube approach, after
the agent is instilled, the chest tube is clamped for 60 to 90 minutes and the patient is assisted to
assume various positions to promote uniform distribution of the agent and to maximize its contact with
the pleural surfaces. The tube is unclamped as prescribed, and chest drainage may be continued several
days longer to prevent reaccumulation of fluid and to promote the formation of adhesions between the
visceral and parietal pleurae.

Other treatments for pleural effusions caused by malignancy include surgical pleurectomy,
insertion of a small catheter attached to a drainage bottle for outpatient management (Pleurx catheter
[Denver Biomedical]), or implantation of a pleuroperitoneal shunt. A pleuroperitoneal shunt consists of
two catheters connected by a pump chamber containing two one-way valves. Fluid moves from the
pleural space to the pump chamber and then to the peritoneal cavity. The patient manually pumps on
the reservoir daily to move fluid from the pleural space to the peritoneal space.

Nursing Management

The nurse’s role in the care of patients with a pleural effusion includes implementing the
medical regimen. The nurse prepares and positions the patient for thoracentesis and offers support
throughout the procedure. The nurse is responsible for making sure the thoracentesis fluid amount is
recorded and sent for appropriate laboratory testing. If a chest tube drainage and water-seal system is
used, the nurse is responsible for monitoring the system’s function and recording the amount of
drainage at prescribed intervals. Nursing care related to the underlying cause of the pleural effusion is
specific to the underlying condition. Care of the patient with a chest tube is discussed in Chapter 25.

If a chest tube is inserted for talc instillation, pain management is a priority and the nurse helps
the patient assume positions that are the least painful. However, frequent turning and movement are
important to facilitate adequate spreading of the talc over the pleural surface. The nurse evaluates the
patient’s pain level and administers analgesic agents as prescribed and as needed.

If the patient is to be managed as an outpatient with a pleural catheter for drainage, the nurse
educates the patient and family about management and care of the catheter and drainage system

Bleeding disorders

Failure of normal hemostatic mechanisms can result in bleeding, which may be severe. This
bleeding is commonly provoked by trauma, but in certain circumstances it can occur spontaneously.
When the source 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). This may
be a reactive response, as in a compensatory response to significant bleeding, or a more general
response to increased hematopoiesis, as in iron deficiency anemia. Sometimes, the increase in platelets
does not result from increased production but from a loss in platelet pooling within the spleen. The
spleen typically holds about one third of the circulating platelets at any time. If the spleen is absent (eg,
splenectomy), the platelet reservoir is also lost, and an abnormally high number of platelets enters the
circulation. In time, the rate of thrombopoiesis slows to reestablish a more normal platelet level.

Clinical Manifestations

Signs and symptoms of bleeding disorders vary depending on the type of defect. A careful
history and physical examination can be useful in determining the source of the hemostatic defect.
Abnormalities of the vascular system give rise to local bleeding, usually into the skin. Because platelets
are primarily responsible for stopping bleeding from small vessels, patients with platelet defects develop
petechiae, often in clusters; these are seen on the skin and mucous membranes but also occur
throughout the body (see Fig. 33-5). Bleeding from platelet disorders can be severe. Unless the platelet
disorder is severe, bleeding can often be stopped promptly when local pressure is applied; it does not
typically recur when the pressure is released.

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 (eg,
subcutaneous or IM hematomas, hemorrhage into joint spaces). External bleeding diminishes very
slowly when local pressure is applied; it often recurs several hours after pressure is removed. For
example, severe bleeding may start several hours after a tooth extraction. Risk factors for bleeding are
listed in Chart 33-4.

Medical Management

Management varies based on the underlying cause of the bleeding disorder. If bleeding is
significant, transfusions of blood products are indicated. The specific blood product used is determined
by the underlying defect and the extent of the blood loss. If fibrinolysis is excessive, hemostatic agents
such as aminocaproic acid (Amicar) can be used to inhibit this process. This agent must be used with
caution, because excessive inhibition of fibrinolysis can result in thrombosis. 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 who have bleeding disorders or who have the potential for development of such
disorders as a result of disease or therapeutic agents must be taught to observe themselves arefully and
frequently for bleeding (Chart 33-10). They need to understand the importance of avoiding activities
that increase the risk of bleeding, such as contact sports. It is necessary to examine the skin for
petechiae and ecchymoses (bruises) and the nose and gums for bleeding. Hospitalized patients are
monitored for bleeding by testing all drainage and excreta (feces, urine, emesis, and gastric drainage) for
occult as well as obvious blood. Outpatients are often given fecal occult blood screening cards to detect
occult blood in stools.

Primary Thrombocythemia

Primary thrombocythemia (also called essential thrombocythemia) is a stem cell disorder within
the bone marrow. A marked increase in platelet production occurs, with the platelet count consistently
greater than 600,000/mm3 . Platelet size may be abnormal, but platelet survival is typically normal.
Occasionally, the platelet increase is accompanied by an increase in erythrocytes, leukocytes, or both;
however, these cells are not increased to the extent that they are in polycythemia vera, CML, or
myelofibrosis. Although the exact cause is unknown, primary thrombocythemia is similar to other
myeloproliferative disorders, particularly polycythemia vera. However, unlike the other
myeloproliferative disorders, it rarely evolves into acute leukemia.
 Primary thrombocythemia, which affects women twice as often as men, tends to occur later in
life (median age at diagnosis is 65 to 70 years). Survival does not appear to differ from the general
population (Johansson, 2006), although conflicting findings have been reported (Brière, 2007).

Clinical Manifestations

Many patients with primary thrombocythemia are asymptomatic; the illness is diagnosed as the
result of finding an elevated platelet count on a CBC. Symptoms occur most often when the platelet
count exceeds 1 million/mm3 . However, they do not always correlate with the extent to which the
platelet count is elevated. When symptoms do occur, they result primarily from hemorrhage or vascular
occlusion. This occlusion can occur in large vessels (cerebrovascular, coronary, or peripheral arteries)
and deep veins, as well as in the microcirculation. Microvascular vasoocclusive manifestations are most
frequently seen in the form of erythromyalgia. The toxic effects of platelet substances include painful
burning, warmth, and redness in a localized distal area of the extremities. Headaches are the most
common neurologic manifestations; other manifestations include transient ischemic attacks and
diplopia (Brière, 2007). More common forms of venous thromboembolism include DVT and pulmonary
embolism. The spleen may also be enlarged but usually not to a significant extent.

In addition, because the platelets can be dysfunctional, minor or major hemorrhage may occur.
Bleeding is commonly limited to recurrent skin manifestations (ecchymoses, hematomas, epistaxis, gum
bleeding), although significant GI bleeding is also possible. Bleeding typically does not occur unless the
platelet count exceeds 1.5 million/mm3 . It results from a deficiency in von Willebrand factor (vWF) as
the platelet count increases (Brière, 2007).

Assessment and Diagnostic Findings

The diagnosis of primary thrombocythemia is made by ruling out other potential disorders—
either other myeloproliferative disorders or underlying illnesses that cause a reactive or secondary
thrombocytosis (see below). Iron deficiency should be excluded, because a reactive increase in the
platelet count often accompanies this deficiency. Occult malignancy should be excluded. The CBC shows
markedly large and abnormal platelets; the platelet count is persistently elevated (greater than
600,000/mm3 ). Analysis of the bone marrow (by aspiration and biopsy) may not be particularly useful.

No data reliably predict the development of complications. Risk factors for the development of
thrombotic complications include age older than 60 years and a history of prior thrombotic events.
Major bleeding tends to occur when the platelet count is very high (greater than 1,500,000/mm3 ) and
there is a prior history of major bleeding. A history of minor bleeding can also render the patient at risk
for further hemorrhage if the platelet count exceeds 1,000,000/mm3 and the duration of disease
exceeds 15 years (Brière, 2007). In contrast, patients who are younger than 40 years of age, have no
previous history of a thrombotic or hemorrhagic event, and have platelet counts of less than
1,000,000/mm3 are considered to be at low risk for developing thrombotic or hemorrhagic
complications.

Medical Management
 The management of primary thrombocythemia is highly controversial. The risk of significant
thrombotic or hemorrhagic complications may not be increased until the platelet count exceeds 1.5
million/mm3 . A careful assessment of other risk factors, such as history of peripheral vascular disease,
history of tobacco use, atherosclerosis, and prior thrombotic events, should be considered in developing
the treatment plan.

In younger patients with no risk factors, low-dose aspirin therapy may be sufficient to prevent
thrombotic complications. However, the use of aspirin can increase the risk of hmorrhagic complications
and is typically a contraindication in patients with a history of GI bleeding. Aspirin can relieve the
neurologic symptoms (eg, headache), erythromyalgias, and visual symptoms of primary
thrombocythemia.

In older patients and in those with concurrent risk factors, more aggressive measures may be
necessary. Hydroxyurea is effective in lowering the platelet count. This agent is taken orally and causes
minimal side effects other than dose-related leukopenia. (Its potential for leukogenesis diminishes its
utility in younger patients with risk factors.) The medication anagrelide is more specific in lowering the
platelet count than hydroxyurea but has more side effects. Severe headaches cause many patients to
stop taking the medication. Tachycardia and chest pain may also occur, and anagrelide is
contraindicated in patients with concurrent cardiac problems. Anagrelide is also carcinogenic.

Interferon-alfa-2b has been shown to lower platelet counts by an unknown mechanism. The
medication is administered subcutaneously at varying frequency, commonly three times per week.
Significant side effects, such as fatigue, weakness, memory deficits, dizziness, anemia, and liver
dysfunction, limit its usefulness.

Rarely, the occlusive symptoms are so great that the platelet count must be reduced
immediately. When necessary, platelet pheresis (see later discussion) can reduce the amount of
circulating platelets, but only transiently. The extent to which symptoms and complications (eg,
thromboses) are reduced by pheresis is unclear.

Nursing Management

Patients with primary thrombocythemia need to be instructed about the accompanying risks of
hemorrhage and thrombosis. The patient is informed about signs and symptoms of thrombosis,
particularly the neurologic manifestations, such as visual changes, numbness, tingling, and weakness.
Risk factors for thrombosis are assessed, such as obesity, hypertension, hyperlipidemia, and smoking;
measures to diminish these risk factors are encouraged. Patients taking aspirin should be informed
about the increased risk of bleeding. Patients who are at risk for bleeding should be instructed about
medications (eg, aspirin, NSAIDs) and other substances (eg, alcohol) that can alter platelet function.
Patients taking interferon are taught to self-administer the medication and manage side effects. Patients
taking hydroxyurea should have CBCs monitored; the dosage is adjusted based on the platelet and white
blood cell count.

Secondary Thrombocytosis
 Increased platelet production is the primary mechanism of secondary, or reactive,
thrombocytosis. The platelet count is above normal, but, in contrast to primary thrombocythemia, an
increase of more than 1 million/mm3 is rare. Platelet function is normal; the platelet survival time is
normal or decreased. Consequently, symptoms associated with hemorrhage or thrombosis are rare.
Many disorders or conditions can cause a reactive increase in platelets, including infection, chronic
inflammatory disorders, iron deficiency, malignant disease, acute hemorrhage, and splenectomy (see
previous discussion of primary thrombocythemia). Treatment is aimed at the underlying disorder. With
successful management, the platelet count usually returns to normal. Thrombocytopenia

Thrombocytopenia

Thrombocytopenia (low platelet level) can result from various factors: decreased production of
platelets within the bone marrow, increased destruction of platelets, or increased consumption of
platelets. Causes and treatments are summarized in Table 33-4.

Clinical Manifestations

Bleeding and petechiae usually do not occur with platelet counts greater than 50,000/mm3 ,
although excessive bleeding can follow surgery or other trauma. 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 (eg, MDS) or medications (eg, 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.

Assessment and Diagnostic Findings

A platelet deficiency that results from decreased production (eg, leukemia, MDS) can usually be
diagnosed by examining the bone marrow via aspiration and biopsy. Numerous genetic causes of
thrombocytopenia have been discovered, including autosomal dominant, autosomal recessive, and X-
linked mutations. If platelet destruction is the cause of thrombocytopenia, the marrow shows increased
megakaryocytes and normal or even increased platelet production as the body attempts to compensate
for the decreased platelets in circulation.

An important cause to exclude is “pseudothrombocytopenia.” Here, platelets aggregate and

clump in the presence of ethylenediamine tetra-acetic acid (EDTA), the anticoagulant present in the
tube used for CBC collection. This clumping is seen in 1:1000 people (Sekhon & Roy, 2006). A manual
examination of the peripheral smear can easily determine platelet clumping as the cause of
thrombocytopenia; newer cell counter machines can also detect this.

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. If excessive platelet destruction occurs, transfused
platelets are also destroyed, and the platelet count does not increase. The most common cause of
excessive platelet destruction is idiopathic thrombocytopenic purpura (see the following discussion). In
some instances splenectomy can be a useful therapeutic intervention, but often it is not an option; for
example, in patients in whom the enlarged spleen is due to portal hypertension related to cirrhosis,
splenectomy may cause more bleeding disorders.

Nursing Management The interventions for a patient with thrombocytopenia are listed in Chart 33-8.

Nursing Interventions

Prevent Complications • Avoid aspirin and aspirin-containing medications or other medications known
to inhibit platelet function, if possible. • Do not give intramuscular injections. • Avoid indwelling
catheters if at all possible. • Take no rectal temperatures; do not give suppositories, enemas. • Use stool
softeners, oral laxatives to prevent constipation. • Induce amenorrhea with oral contraceptives (do not
administer the placebo pills). • Use smallest possible needles when performing venipuncture. • Apply
pressure to venipuncture sites for 5 minutes or until bleeding has stopped. • Permit no flossing of teeth
and no commercial mouthwashes. • Use only soft-bristled toothbrush for mouth care. • Consider using
toothettes for mouth care if platelet count is 10,000/mm3 , or if gums bleed. • Lubricate lips with water-
soluble lubricant every 2 hours while awake. • Avoid suctioning if at all possible; if unavoidable, use only
gentle suctioning. • Discourage vigorous coughing or blowing of the nose. • Use only electric razor for
shaving. • Pad side rails as needed. • Prevent falls by ambulating with patient as necessary.

Control Bleeding • Apply direct pressure. • For epistaxis, position patient in high Fowler’s position; apply
ice pack to back of neck and direct pressure to nose. • Notify physician for prolonged bleeding (eg,
unable to stop within 10 minutes). • Administer platelets, fresh-frozen plasma, packed red blood cells,
as prescribed.