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The Hematologic System



OVERVIEW OF ANATOMY AND PHYSIOLOGY

The structures of the hematologic or hematopoietic system include the blood, blood vessels, and blood forming organs
(bone marrow, spleen, liver, lymph nodes, and thymus gland). The major function of the blood is to carry necessary
materials (oxygen, nutrients) to cells and to remove carbon dioxide and metabolic waste products. The hematologic system
also plays an important role in hormone transport, the inflammatory and immune responses, temperature regulation, fluid-
electrolyte balance, and acid-base balance.

Bone Marrow
1. Contained inside all bones, occupies interior of spongy bones and center of long bones; collectively one of the
largest organs of the body (4-5% of total body weight).
2. Primary function is hematopoiesis (the formation of blood cells).
3. Two kinds of bone marrow, red and yellow
1. Red (functioning) marrow
o Carries out hematopoiesis; production site of erythroid, myeloid, and thrombocytic components of blood;
one source of lymphocytes and macrophages.
o Found in ribs, vertebral column, and other flat bones.
2. Yellow marrow: red marrow that has changed to fat; found in long bones; does not contribute to hematopoiesis.
4. All blood cells start as stem cells in the bone marrow; these mature into the different, specific types of cells,
collectively referred to as formed elements of blood or blood components: erythrocytes, leukocytes, and
thrombocytes.

Blood
1. Composed of plasma (55%) and cellular components (45%).
2. Hematocrit
Reflects portion of blood composed of red blood cells.
Centrifugation of blood results in separation into top layer of plasma, middle layer of leukocytes and platelets,
and bottom layer of erythrocytes.
Majority of formed elements is erythrocytes; volume of leukocytes and platelets is negligible.
3. Distribution
1300 mL in pulmonary circulation
1. 400 mL arterial
2. 60 mL capillary
3. 840 mL venous
3000 mL in systemic circulation
1. 550 mL arterial
2. 300 mL capillary
3. 2150 mL venous

Plasma
1. Liquid part of blood; yellow in color because of pigments.
2. Consists of serum (liquid portion of plasma) and fibrinogen.
3. Contains plasma proteins such as albumin, serum globulins, fibrinogen, prothrombin, plasminogen.
Albumin: largest of plasma proteins, involved in regulation of intravascular plasma volume and maintenance of
osmotic pressure.
Serum globulins: alpha, beta, gamma
1. Alpha: role in transport of steroids, lipid, bilirubin.
2. Beta: role in transport of iron and copper.
3. Gamma: role in immune response function of antibodies.
4. Fibrinogen, prothrombin, plasminogen.


Cellular Components

Cellular components of formed elements of blood are erythrocytes (red blood cells [RBCs]), which are responsible for oxygen
transport; leukocytes (white blood cells [WBCs]), which play a major role in defense against microorganisms; and
thrombocytes (platelets), which function in hemostasis.

Erythrocytes (red blood cells)
1. Biconcave disc shape, no nucleus, chiefly sacs of hemoglobin.
2. Cell membrane is highly diffusible to O and CO.
3. RBCs are responsible for oxygen transport via hemoglobin (Hgb).
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o Two portions: iron carried on heme portion; second portion is protein.
o Normal blood contains 12-18 g Hgb/100 mL blood; higher (14-18 g) in men than in women (12-14 g)
4. Production
o Start in bone marrow as stem cells, released as reticulocytes (immature cells), mature into erythrocytes.
o Erythropoietin stimulates differentiation; produced by kidneys and stimulated by hypoxia.
o Iron, vitamin B, folic acid, pyridoxine (vitamin B), and other factors required for erythropoiesis.
5. Hemolysis (destruction)
o Average life span 120 days
o Immature RBCs destroyed in either bone marrow or other reticuloendothelial organs (blood, connective
tissue, spleen, liver, lungs, and lymph nodes).
o Mature cells removed chiefly by liver and spleen.
o Bilirubin: by-product of Hgb released when RBCs destroyed, excreted in bile.
o Iron: freed from Hgb during bilirubin formation; transported to bone marrow via transferring and reclaimed
for new Hgb production.
o Premature destruction: may cause by RBC membrane abnormalities, Hgb abnormalities, extrinsic physical
factors (such as the enzyme defects found in G6PD)
o Normal age RBCs may be destroyed by gross damage as in trauma or extravascular hemolysis (in spleen,
liver, bone marrow).

Leukocytes: involved in protection from bacteria and other foreign substances.
1. Granulocytes:
o Eosinophils: involved in phagocytosis and allergic reactions.
o Basophils: involved in prevention of clotting in microcirculation and allergic reactions.
o Eosinophils: and Basophils are reservoirs of histamine, serotonin, and heparin.
o Neutrophils: involved in short-term phagocytosis.
1. mature neutrophils: polymorphonuclear leukocytes
2. immature neutrophils: band cells (bacterial infection usually produces increased numbers of band
cells).
2. Mononuclear cells: monocytes and lymphocytes: large nucleated cells
o Monocytes: involved on long-term phagocytosis; play a role in immune response.
1. largest leukocyte
2. produced by bone marrow: give rise to histiocytes (Kupffer cells of liver), macrophages, and other
components of reticuloendothelial system.
o Lymphocytes: immune cells; produce substances against foreign cells; produced primarily in lymph tissue
(B cells) and thymus (T cells).

Thrombocytes (platelets)
1. Fragments of megakaryocytes formed in bone marrow.
2. Production regulated by thrombopoietin.
3. Essential factor in coagulation via adhesion, aggregation, and plug formation.
4. Release substances involved in coagulation.

Blood Groups
Erythrocytes carry antigens, which determine the different blood groups.
Blood-typing systems are based on the many possible antigens, but the most important are the antigens of the ABO
and Rh blood groups because they are likely to be involved in transfusion reactions.

1. ABO typing
1. Antigens of system are labeled A and B.
2. Absence of both antigens results in type O blood.
3. Presence of both antigens is type AB.
4. Presence of either A or B results in type A and type B, respectively.
5. Nearly half the population is type O, the universal donor.
6. Antibodies are automatically formed against the ABO antigens not on persons own RBCs; transfusion with
mismatched or incompatible blood results in a transfusion reaction.
2. Rh typing
1. Identifies presence or absence of Rh antigen (Rh positive or Rh negative).
2. Anti-Rh antibodies not automatically formed in Rh-negative person, but if Rh-positive blood is given,
antibody formation starts and a second exposure to Rh antigen will trigger a transfusion reaction.
3. Important for Rh-negative woman carrying Rh-positive baby, first pregnancy not affected, but in subsequent
pregnancy with an Rh-positive baby, mothers antibodies attack babys RBCs.



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Blood Coagulation
Conversion of fluid blood into a solid clot to reduce blood loss when blood vessels are ruptured.

1. Systems that initiate clotting.
Intrinsic system: initiated by contact activation following endothelial injury (intrinsic to vessel itself).
1. Factor XII initiates as contact made between damaged vessel and plasma protein.
2. Factors VIII, IX, and XI activated.
Extrinsic system
1. Initiated by tissue thromboplastins, released from injured vessels (extrinsic to vessel).
2. Factor VII activated.
2. Common pathways: activated by either intrinsic or extrinsic pathways.
1. Platelets factor 3 (PF3) and calcium react with factors X and V.
2. Prothrombin converted to thrombin via thromboplastin.
3. Thrombin acts on fibrinogen, forming soluble fibrin.
4. Soluble fibrin polymerized by factor XIII to produce a stable, insoluble fibrin clot.
3. Clot resolution: takes place via fibrinolytic system by plasmin and proteolytic enzymes; clot dissolves as tissue
repairs.

Spleen
1. Largest lymphatic organ: function as blood filtration system and reservoir
2. Vascular, bean shaped; lies beneath the diaphragm, behind and to the left of the stomach; composed of a fibrous
tissue capsule surrounding a network of fiber.
3. Contains two types of pulp
Red pulp: located between the fibrous strands, composed of RBCs, and macrophages.
White pulp: scattered throughout the red pulp, produces lymphocytes and sequesters lymphocytes,
macrophages, and antigens.
4. 1-2% of red cell mass or 200 mL blood/minute stored in spleen; blood comes via the splenic artery to the pulp for
cleansing, then passes into splenic venules that are lined with phagocytic cells, and finally to the splenic vein to the
liver.
5. Important hematopoietic site in fetus; postnatally produces lymphocytes and monocytes.
6. Important in phagocytosis; removes misshapen erythrocytes, unwanted parts of erythrocytes.
7. Also involved in antibody production by plasma cells and iron metabolism (iron released from Hgb portion of
destroyed erythrocytes returned to bone marrow).
8. In the adult, functions of the spleen can be taken over by the reticuloendothelial system

Liver
1. Involved in bile production (via erythrocyte destruction and bilirubin production) and erythropoiesis (during fetal life
and when bone marrow production is insufficient).
2. Kupffer cells of liver have reticuloendothelial function as histiocytes; phagocytic activity and iron storage.
3. Liver also involved in synthesis of clotting factors, synthesis of antithrombins.

Physical Examination
A. Auscultate for heart murmurs; bruits (cerebral, cardiac, carotid); pericardial or pleural friction rubs; bowel sounds.
B. Inspect for
1. Flush or pallor of mucous membranes, nail beds, palms, soles of feet.
2. Infection or pallor of sclera, conjunctiva
3. Cyanosis
4. Jaundice of skin, mucous membranes conjunctiva.
5. Signs of bleeding, petechiae, ecchymoses, oral mucosal bleeding (especially gums), epistaxis, hemorrhage from
any orifice.
6. Ulcerations or lesions.
7. Swelling or erythema.
8. Neurologic changes: pain and touch, position and vibratory sense, superficial and deep tendon reflexes.
C. Palpate lymph nodes; note location, size, texture, sensation, fixation; palpate the ribs for sterna bone tenderness.
D. Evaluate joint range of motion and tenderness.
E. Percuss for lung excursion, splenomegaly, hepatomegaly.

Laboratory/Diagnostic Tests
A. Blood
1. Complete blood count (CBC) with differential and peripheral smear.
a. White blood cell count (WBC) with differential.
Normal Value: 4.8 10.8 K/mL
Mean corpuscular volume (MCV): 81 99 fL
Mean corpuscular hemoglobin (MCH): 27 34 pg
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Mean corpuscular hemoglobin concentration (MCHC): 32 36 g/dl
Neutrophils: 35 70%
Lymphocytes: 25 45%
Monocytes: 0 12%
Eosinophils: 0 7%
Basophils: 0 2%
b. Hemoglobin (Hgb)
Normal Value male: 14.0 17.5 g/dl
Normal Value female: 12.3 15.3 g/dl
c. Hematocrit (HCT)
Normal Value male: 41.5 50.4%
Normal Value female: 35.9 44.6%
d. Platelet and reticulocyte count
Normal value: 150 400 K/mL
e. Red blood cell count (RBC) with peripheral smear.
Normal Value: 4.7 6.1 M/mL
2. Coagulation studies
a. Prothrombin time (PT)
b. Partial thromboplastin time (PTT)
c. Fibrin split products (FSP)
d. Lee-White clotting time (whole blood clotting time)
3. Blood chemistry
a. Blood urea nitrogen (BUN)
b. Creatinine
c. Bilirubin: direct and indirect
d. Uric acid
4. Miscellaneous
a. Erythrocyte sedimentation rate (ESR)
b. Serum protein electrophoresis
c. Serum iron and total iron-binding capacity.
d. Plasma protein assay
e. Direct and indirect Coombs tests
B. Urine and stool
1. Urinalysis
2. Hematest
3. Bence-Jones protein assay (urine)
C. Radiologic
1. Chest or other X-ray as indicated by history and physical exam.
2. Radionuclide scan (e.g., bone scan).
3. Lymphangiography.
D. Bone marrow aspiration and biopsy.
1. Puncture of iliac crest (preferred site), vertebrae body, sternum, or tibia (in infants) to collect tissue from bone
marrow.
2. Purpose: study cells involved in blood production.
3. Nursing care
a. Confirm that consent form has been signed.
b. Allay client anxiety; prepare client for a sharp, brief pain when bone marrow is aspirated into syringe.
c. Position client and assist physician to maintain sterile field.
d. Immediately after the aspiration, apply pressure to the site for at least 5 minutes and longer, if necessary.
e. Check the site frequently for signs of bleeding or infection.
f. Send specimen to laboratory.

Blood Transfusion and Component Therapy
Purpose:
1. improve oxygen transport (RBCs)
2. volume expansion (whole blood, plasma, albumin)
3. provision of proteins (fresh frozen plasma, albumin, plasma, fresh whole blood)
4. provision of platelets (platelet concentrate, fresh whole blood)
Blood and blood products
1. Whole blood; provides all components
o Large volume: 12-24 hours for Hgb and HCT to rise.
o Complications: volume overload, transmission of hepatitis or AIDS, transfusion reaction, infusion of excess
potassium and sodium, infusion of anticoagulant (citrate) in massive transfusion therapy.
2. Red blood cells
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o Provide twice the amount of Hgb as an equivalent amount of whole blood.
o Indicated in cases of blood loss, pre and post-op clients, and those with incipient congestive failure.
o Complications: transfusion reaction (less common than with whole blood due to removal of plasma
proteins).
3. Fresh frozen plasma
o Contains all coagulation factors including V and VIII.
o Can be stored frozen for 12 months; takes 20 minutes to thaw.
o Hang immediately upon arrival to unit (loses its coagulation factors rapidly)
4. Platelets
o Will raise recipients platelet count by 10,000/mm
o Pooled from 4-8 units of whole blood.
o Single-donor platelet transfusions may be necessary for clients who have developed antibodies;
compatibility testing may be necessary
5. Factor VIII fractions (cryoprecipitate): contains Factors VIII, fibrinogen, and XIII.
6. Granulocytes
o Do not increase WBC; increase marginal pool (at tissue level) rather than circulating pool.
o Premedication with steroids, antihistamines, and acetaminophen.
o Respiratory distress with shortness of breath, cyanosis, and chest pain may occur; requires cessation of
transfusion and immediate attention.
o Shaking chills or rigors common, require brief cessation of therapy, administration of meperidine IV until
rigors are diminished, and resumption of transfusion when symptoms relieved.
7. Volume expanders: albumin; percentage concentration varies (50-100 mL/unit); hyperosmolar solutions should
not be used in dehydrated clients.
Nursing care
1. Assess client for history of previous blood transfusions and any adverse reactions.
2. Ensure that the adult client has an 18-20 gauge IV catheter in place.
3. Use 0.9% sodium chloride.
4. At least two nurses should verify the ABO group, RH type, client and blood numbers, and expiration date.
5. Take baseline vital signs before initiating transfusion.
6. Start transfusion slowly (2 mL/minute).
7. Stay with the client during the first 15 minutes of the transfusion and take vital signs frequently.
8. Maintain the prescribed transfusion rate.
a. Whole blood: approximately 3-4 hours.
b. RBCs: approximately 2-4 hours
c. Fresh frozen plasma: as quickly as possible
d. Platelets: as quickly as possible
e. Cryoprecipitate: rapid infusion
f. Granulocytes: usually over 2 hours
g. Volume expanders: volume-dependent rate
9. Monitor for adverse reactions
10. Document the following:
a. Blood component unit number (apply sticker if available).
b. Date infusion starts and ends.
c. Type of component and amount transfused.
d. Client reaction and vital signs.
e. Signature of transfusionist.
f. If a reaction occurs, follow facility protocol for blood packaging and assessing client.

DISORDERS OF THE HEMATOLOGIC SYSTEM

Anemias
Iron-Deficiency Anemia
Chronic microcytic, hypochromic anemia caused by either inadequate absorption or excessive loss of iron.
Causes:
1. Trauma, dysfunctional uterine bleeding, and GI bleeding
2. Inadequate intake of iron-rich foods or by inadequate absorption of iron (from chronic diarrhea, malabsorption
syndromes, high cereal-product intake with low animal protein ingestion, partial or complete gastrectomy, pica).
3. Incidence related to geographic location, economic class, age group, and sex
o More common in developing countries and tropical zones (blood-sucking parasites).
o Women between ages 15 and 45 and children affected more frequently, as are the poor.
4. In iron-deficiency states, iron stores are depleted first, followed by a reduction in Hgb formation.

Assessment findings:
1. Mild cases usually asymptomatic
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2. Palpitations, dizziness, and cold sensitivity
3. Brittleness of hair and nails; pallor
4. Dysphagia, stomatitis, and atrophic glossitis
5. Dyspnea weakness
Laboratory findings:
1. RBCs small (microcytic) and pale (hypochromic)
2. Decreased: Hgb, HCT, serum iron, serum ferritin and reticulocyte count
3. Hemosiderin absent from bone marrow
Nursing interventions:
1. Monitor for signs and symptoms of bleeding through hematest of all elimination including stool, urine, and gastric
contents.
2. Provide for adequate rest: plan activities so as not to overtire.
3. Provide a thorough explanation of all diagnostic tests used to determine sources of possible bleeding (helps allay
anxiety and ensure cooperation).
4. Administer iron preparations as ordered.
Oral iron preparations: route of choice
1. Give following meals or snack.
2. Dilute liquid preparations well and administer using a straw to prevent staining teeth.
3. When possible administer with orange juice as vitamin C (ascorbic acid) enhances iron absorption.
4. Warn clients that iron preparations will change stool color and consistency (dark and tarry) and
may cause constipation.
5. Antacid ingestion will decrease oral iron effectiveness; milk products and eggs inhibit absorption.
Parental iron preparations: used in clients intolerant to oral preparations, who are noncompliant with
therapy, or who have continuing blood losses.
1. Use one needle to withdraw and another to administer iron preparations as tissue staining and irritation
are a problem.
2. Use the Z-track injection technique to prevent leakage into tissues.
3. Do not massage injection site but encourage ambulation as this will enhance absorption; advice against
vigorous exercise and constricting garments.
4. Observe for local signs of complications: pain at the injection site, development of sterile abscesses,
lymphadenitis as well as fever, headache, urticaria, hypotension, or anaphylactic shock.
5. Provide dietary teaching regarding food high in iron (meats, fortified cereals, nuts, seeds, dried beans, dried fruit).
6. Encourage ingestion of roughage and increase fluid intake to prevent constipation if oral iron preparations are being
taken.

Pernicious Anemia
Chronic progressive, macrocytic anemia caused by a deficiency of intrinsic factor; the result is abnormally
large erythrocytes and hypochondria (a deficiency of hydrochloric acid in gastric secretions).
Characterized by neurologic and GI symptoms; death usually results if untreated.
Causes:
Lack of intrinsic factor is cause by gastric mucosal atrophy (possibly due to heredity, prolonged iron deficiency, or an
autoimmune disorder)
Had total gastrectomy if vitamin B not administered.
Usually occurs in men and women over age 50
With an increase in blue-eyed persons of Scandinavian descent.

Pathophysiology
1. Intrinsic factor is necessary for the absorption of vitamin B into the small intestine.
2. B deficiency diminishes DNA synthesis, which results in defective maturation of cells (particularly rapidly dividing
cells such as blood cells and GI tract cells).
3. B deficiency can alter structure and function of peripheral nerves, spinal cord, and the brain.

Assessment findings
1. Anemia, weakness, pallor, dyspnea, palpitations, fatigue
2. GI symptoms: sore mouth; smooth, beefy, red tongue; weight loss; dyspepsia; constipation or diarrhea; jaundice.
3. CNS symptoms: tingling, paresthesias of hands and feet, paralysis, depression, psychosis.
Laboratory tests
1. Erythrocyte count decreased
2. Blood smear: oval, macrocytic erythrocytes with a proportionate amount of Hgb
3. Bone marrow
o Increased megaloblasts (abnormal erythrocytes)
o Few normoblasts or maturing erythrocytes
o Defective leukocyte maturation
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4. Biliburin (indirect): elevated unconjugated fraction.
5. Serum LDH elevated.
6. Positive Schilling test
o Measures absorption of radioactive vitamin B both before and after parenteral administration of intrinsic
factor.
o Definitive test for pernicious anemia
o Used to detect lack of intrinsic factor
o Fasting client is given radioactive vitamin B by mouth and nonradioactive vitamin B IM
to saturate tissue binding sites and to permit some excretion of radioactive vitamin B in
the urine if it is absorbed.
o 24-48 hour urine collection is obtained; client is encouraged to drink fluids.
o If indicated, second stage Schilling test performed 1 week after first stage. Fasting client is
given radioactive vitamin B combined with human intrinsic factor and test is repeated.
7. Gastric analysis: decreased free hydrochloric acid.
8. Large numbers of reticulocytes in the blood following parenteral vitamin B administration.

Nursing interventions
1. Provide a nutritious diet high in iron, protein and vitamins (fish, meat, milk/milk products, and eggs).
2. Avoid highly seasoned, coarse or very hot foods if client has mouth sores.
3. Provide mouth care before and after meals using a soft toothbrush and nonirritating rinses.
4. Bed rest may be necessary if anemia is severe.
5. Provide safety when ambulating (especially if carrying hot items, etc.).
6. Administer blood products

Discharge teaching:
1. Dietary instruction
2. Importance of lifelong vitamin B therapy.
3. Rehabilitation and physical therapy for neurologic deficits, as well as instruction regarding safety.

Drug therapy
1. Vitamin B injections: monthly maintenance
2. Iron preparations (if Hgb level inadequate to meet increased numbers of erythrocytes)
3. Folic acid
Controversial
Reverses anemia and GI symptoms but may intensify neurologic symptoms
May be safe if given in small amounts in addition to vitamin B

Aplastic Anemia
o Pancytopenia or depression of granulocyte, platelet, and erythrocyte production due to fatty replacement of the
bone marrow.
o Bone marrow destruction may be idiopathic or secondary.
o Secondary aplastic anemia may be caused by
1. Chemical toxins (e.g., benzene)
2. Drugs (e.g., chloramphenicol, cytotoxic drugs)
3. Radiation
4. Immunologic injury

Assessment findings
1. Fatigue, dyspnea, pallor
2. Increased susceptibility to infection
3. Bleeding tendencies and hemorrhage

Laboratory findings:
1. normocytic anemia, granulocytopenia, thrombocytopenia
2. Bone marrow biopsy: marrow is fatty and contains very few developing cells.

Nursing interventions
1. Administer blood transfusions as ordered.
2. Provide nursing care for client with bone marrow transplantation.
3. Administer medications as ordered.
4. Monitor for signs of infection and provide care to minimize risk.
a. Maintain neutropenic precautions.
b. Encourage high-protein, high-vitamin diet to help reduce incidence of infection.
c. Provide mouth care before and after meals.
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5. Monitor for signs of bleeding and provide measures to minimize risk.
a. Use a soft toothbrush and electric razor.
b. Avoid intramuscular injections.
c. Hematest urine and stool.
d. Observe for oozing from gums, petechiae, or ecchymoses.
6. Provide client teaching and discharge planning concerning
a. Self-care regimen
b. Identification of offending agent and importance of avoiding it (if possible) in future.

Medical management
1. Blood transfusions: key to therapy until clients own marrow begins to produce blood cells.
2. Aggressive treatment of infection.
3. Bone marrow transplantation
Drug therapy
1. Corticosteroids and/ or androgens to stimulate bone marrow function and to increase capillary resistance (effective
in children but usually not in adults).
2. Estrogen and/or progesterone to prevent amenorrhea in female clients.
3. Identification and withdrawal of offending agent or drug.

Hemolytic Anemia
A category of diseases in which there is an increased rate of RBC destruction.
The degree of anemia is determined by the lag between erythrocyte hemolysis and the rate of bone marrow
erythropoiesis.

Types
1. Congenital: includes hereditary spherocytosis, G6PD deficiency, sickle cell anemia, thalassemia
2. Acquired: includes transfusion incompatibilities, thrombotic thrombocytopenic purpura; disseminated intravascular
clotting, spur cell anemia.

Causes: Often unknown, but erythrocyte life span is shortened and hemolysis occurs at a rate that the bone marrow cannot
compensate for.

Diagnosis: Based on laboratory evidence of an increased rate of erythrocyte destruction and a corresponding compensatory
effort by bone marrow to increase production.

Assessment findings
1. Clinical manifestations vary depending on severity of anemia and the rate of onset (acute vs. chronic)
2. Pallor, sclera icterus, and slight jaundice (chronic)
3. Chills, fever, irritability, precordial spasm, and pain (acute)
4. Abdominal pain and nausea, vomiting, diarrhea, melena
5. Hematuria, marked jaundice, and dyspnea
6. Splenomegaly and symptoms of cholelithiasis, hepatomegaly

Laboratory tests
1. Hgb and HCT decreased
2. Reticulocyte count elevated (compensatory)
3. Coombs test (direct): positive if autoimmune features present
4. Biliburin (indirect): elevated unconjugated fraction

Nursing interventions
1. Monitor for signs and symptoms of hypoxia including confusion, cyanosis, and shortness of breath, tachycardia, and
palpitations.
2. Note that the presence of jaundice may make assessment of skin color in hypoxia unreliable.
3. If jaundice and associated pruritus are present, avoid soap during bathing and use cool or tepid water.
4. Frequent turning and meticulous skin care are important as skin friability is increased.
5. Teach clients about the nature of the disease and identification of factors that predispose to episodes of hemolytic
crisis.

Medical management:
1. Corticosteroids in autoimmune types of anemia
2. Folic acid supplements
3. Blood transfusion therapy
4. Splenectomy

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Splenectomy
Indications
1. Rupture of the spleen caused by trauma, accidental tearing during surgery, diseases causing softening or damage
(e.g., infectious mononucleosis).
2. Hypersplenism: excessive splenic damage of cellular blood components as the spleen is a major source of antibody
formation in children, splenectomy is not recommended during the early years of life; if absolutely necessary, client
should receive prophylactic antibiotics post-op.
3. Primary hypersplenism can be alleviated with splenectomy; procedure is palliative only in secondary
hypersplenism

Nursing interventions: preoperatively
1. Provide routine preoperative care and explain what to expect postoperatively.
2. Administer pneumococcal vaccine as ordered since client will be at increased risk for pneumococcal infections for
several years after splenectomy.

Nursing intervention: postoperatively
1. Be aware that it is crucial to monitor carefully for hemorrhage and shock as clients with pre-op bleeding tendencies
will remain at risk post-op.
2. Monitor post-op temperature elevation: fever may not be the best indicator of post-op complications such as
pneumonia or urinary tract infection, as fever without concomitant infection is common following splenectomy.
3. Observe for abdominal distension and discomfort secondary to expansion of the intestines and stomach; an
abdominal binder may reduce distension.
4. Know that postoperative infection in a child is considered life threatening; administer prophylactic antibiotics as
ordered.
5. Ambulate early and provide chest physical therapy as location of the incision makes post-op atelectasis or
pneumonia a risk.
6. Emphasize to client the need to report even minor signs or symptoms of infection immediately to the physician.

Polycythemia Vera
o An increase in both the number of circulating erythrocytes and the concentration of Hgb within the blood.
o Classified as a myeloproliferative disorder (bone marrow overgrowth)
o Cause unknown, but thought to be a form of malignancy similar to leukemia.
o Usually develops in middle age, common in Jewish men

Three forms:
1. Primary polycythemia vera unknown
2. Secondary polycythemia increase level of erythropoietin
3. Relative polycythemia - increase hematocrit due to decrease in plasma
Pathophysiology
1. A pronounced increase in the production of erythrocytes accompanied by an increase in the production of
myelocytes (leukocytes within bone marrow) and thrombocytes.
2. The consequences of this overproduction are an increase in total blood volume (2-3 times greater than
normal), and severe congestion of all tissues and organs with blood.
Assessment findings
1. Ruddy complexion and duskiness of mucosa secondary to capillary congestion in the skin and mucous membranes.
2. Hypertension associated with vertigo, headache, and fullness in the head secondary to increased blood volume.
3. Symptoms of HF secondary to overwork of the heart.
4. Thrombus formation: CVA, MI, gangrene of the extremities, DVT, and pulmonary embolism can occur.
5. Bleeding and hemorrhage secondary to congestion and overdistension of capillaries and venules.
6. Hepatomegaly and splenomegaly.
7. Peptic ulcer secondary to increased gastric secretions.
8. Gout secondary to increased uric acid released by nucleoprotein breakdown.
9. Night sweats, epigastric and joint pain
10. Visual: scotomas, double vision, blurred vision
11. Pleuritic chest pain
Laboratory tests
1. CBC: increase in all mature cell forms (erythrocytes, leukocytes, and platelets)
2. HCT: increased
3. Bone marrow: increase in immature cell forms.
4. Bilirubin (indirect): increase to unconjugated fraction.
5. Liver enzymes may be increased
6. Uric acid increased.
7. Hematuria and melena possible.
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8. Decreased serum iron concentration
9. Panmyelosis
Nursing interventions
1. Monitor for signs and symptoms of bleeding complications.
2. Force fluids and record I&O.
3. Prevent development of DVT.
4. Monitor for signs and symptoms of CHF.
5. Provide care for the client having a phlebotomy.
o 350 500 ml removed every other day
o Complications: iron deficiency anemia
6. Prevent/provide care for the bleeding or infection complications.
7. Administer medications as ordered.
o Radioactive phosphorus (P): of erythrocyte production produces a remission of 6 months to 2 years.
o Nitrogen mustard, busulfan (Myleran), chlorambucil, cyclophosphamide to effect myelosuppression.
o Antigout and peptic ulcer drugs as needed.
8. Decrease in activity tolerance: need to space activity with periods of rest.
9. Avoidance of iron-rich foods to avoid counteracting the therapeutic effects of phlebotomy.
10. Pheresis technology: removal of RBCs, WBCs, platelets
11. Splenectomy

Disorders of Platelets and Clotting Mechanism

Thrombocytopenia
Decrease in number of circulating platelets or platelet count of less than 100,000/mL blood
A decrease in number of functional platelets leads to bleeding disorders; high risk for bleeding if platelet count is
below 20,000/mm3; cerebral and pulmonary hemorrhages can occur when platelet counts drop below 10,000/mm3

3 Mechanisms of Thrombocytopenia
1. Decreased production
2. Increased destruction
3. Increased consumption

Types
1. Inherited autoimmune or idiopathic thrombocytopenia purpura (ITP)
follows a viral infection such as measles, rubella, or chicken pox
2. Acquired
infection or drug-induced effects

Assessment
1. Petechiase (pinpoint hemorrhages on skin and mucous membranes) and purpura (purplish discolored areas) most
commonly found in mucous membranes, anterior thorax, arms, and neck.
2. Epistaxis, gingival bleeding, menorrhagia, hematuria, and gastrointestinal bleeding (bloody or tarry stools)
3. Signs of internal hemorrhage
4. Decreased hemoglobin and hematocrit if bleeding is present
5. Decreased platelet count; platelet antibodies present if ITP
6. Prolonged bleeding time
7. Bone marrow examination may reveal decreased platelet activity or increased megakaryocytes.

Interventions
1. Treat underlying cause
2. Platelet transfusions for active bleeding; little benefit in ITP
3. Splenectomy if medications are not effective
4. Bleeding precautions
Avoid intramuscular or subcutaneous injection
Avoid indwelling catheters
Use smallest gauge needles for injections or venipunctures
Apply pressure on injection sites for 5 minutes or until bleeding stops
Avoid straining at stool, vigorous coughing, and nose blowing
No rectal manipulation: rectal temperatures, suppositories, enemas
Use electric shavers and soft bristled toothbrush
Avoid flossing
No aspirin and drugs that interfere with blood coagulation
5. Monitor CBC, platelet counts, signs of bleeding
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Drugs
1. Steroids and immunoglobulins for ITP
2. Immunosuppressive agents: vincristine (Oncovin) and cyclophosphamide (Cytoxan)
3. Platelet growth factor: oprelvekin (Neumega)


Disseminated Intravascular Coagulation (DIC)
Diffuse fibrin deposition within arterioles and capillaries with widespread coagulation all over the body and
subsequent depletion of clotting factors.
Hemorrhage from kidneys, brain, adrenals, heart, and other organs.
Cause unknown
Clients are usually critically ill with an obstetric, surgical, hemolytic, or neoplastic disease.
May be linked with enemy of thromboplastic substances into the blood.
Mortality rate is high, usually because underlying disease cannot be corrected.

Pathophysiology
1. Underlying disease (e.g., toxemia of pregnancy, cancer) causes release of thromboplastic substances that promote
the deposition of fibrin throughout the microcirculation.
2. Microthrombi form in many organs, causing microinfarcts and tissue necrosis.
3. RBCs are trapped in fibrin strands and are hemolysed.
4. Platelets, prothrombin, and other clotting factors are destroyed, leading to bleeding.
5. Excessive clotting activates the fibrinolytic system, which inhibits platelet function, causing further bleeding.

Assessment findings
1. Petechiae and ecchymoses on the skin, mucous membranes, heart, lungs, and other organs.
2. Prolonged bleeding from breaks in the skin (e.g., IV or venipuncture sites)
3. Severe and uncontrollable hemorrhage during childbirth or surgical procedures.
4. Mental status changes
5. Oliguria and acute renal failure
6. Convulsions. Coma, death

Laboratory findings
1. PT prolonged
2. PTT usually prolonged
3. Thrombin time usually prolonged
4. Fibrinogen level usually decreased
5. Platelet count usually depressed
6. Fibrin split products elevated
7. Protamine sulfate test strongly positive
8. Factor assays (II, V, VII) depressed

Nursing interventions
1. Monitor blood loss and attempt to quantify.
2. Observe for signs of additional bleeding or thrombus formation.
3. Monitor appropriate laboratory data.
4. Prevent further injury.
o Avoid IM injections.
o Apply pressure to bleeding sites.
o Turn and position client frequently and gently.
o Provide frequent nontraumatic mouth care (e.g., soft toothbrush or gauze sponge)
5. Provide emotional support to client and significant others.
6. Administer blood transfusions and medications as ordered.
7. Teach client the importance of avoiding aspirin or aspirin-containing compounds.

Medical management
1. Identification and control of underlying disease is key.
2. Blood transfusions: include whole blood, packed RBCs. Platelets, plasma, cryoprecipitate, and volume expanders.
3. Heparin administration
o Somewhat controversial
o Inhibits thrombin thus preventing further clot formation, allowing coagulation factors to accumulate.


12

IMMUNOLOGIC DISORDERS

Functions of the Immune System
The immune system provides protection against invasion by microorganisms from outside the body.
The immune system protects the body from internal threats and maintains the internal environment by removing
dead or damaged cells.

Immune Response
3. T lymphocytes and B lymphocytes
Lymphocytes migrate to lymphoid tissue where they remain dormant until they need to form sensitized
lymphocytes for cellular immunity or antibodies for humoral immunity.
Some B lymphocytes lie dormant until a specific antigen enters the body, at which time they greatly increase
in number and are available for defense.
T lymphocytes are responsible for rejection of transplanted tissue.
T and B lymphocytes are necessary for a normal immune response
4. Humoral Response
Humoral response is immediate
This type of response provides protection against acute, rapidly developing bacterial and viral infections.
5. Cellular Response
Cellular response is delayed; this is also called delayed hypersensitivity
This type of response is active against slowly developing bacterial infections and is involved in autoimmune
responses, some allergic reactions, and rejection of foreign cells.

Immunity
Natural Immunity
Natural immunity is also called native or innate immunity
It is present at birth and includes biochemical, physical, and mechanical barriers of defense, as well as the
inflammatory response.
Acquired Immunity
Acquired or adaptive immunity is received passively from the mothers antibodies, animal serum, or
antibodies produced in response to a disease.
Immunization produces active acquired immunity.

Laboratory Studies
1. Antinuclear antibody (ANA) titer determination
The ANA titer determination is a blood test used for the differential diagnosis of rheumatic diseases and for
the detection of antinucleoprotein factors and patterns associated with certain autoimmune diseases.
The test is positive at a titer of 1:20 or 1:40, depending on the laboratory.
A positive result does not necessarily confirm a disease.
The ANA titer is positive in most individuals diagnosed with systemic lupus erythematosus (SLE).
An ANA titer result can be false-positive in a small proportion of the normal population.
2. Anti-dsDNA antibody test
The anti-dsDNA (double-stranded DNA) antibody test is a blood test done specifically to identify or
differentiate DNA antibodies found in SLE.
The test supports a diagnosis, monitors disease activity and response to therapy, and establishes a prognosis
for SLE.
Values:
o Negative: lower than 70 units by enzyme-linked immunosorbent assay (ELISA)
o Borderline: 70 to 200 units
o Positive: higher than 200 units
3. Skin testing
Description
The administration of an allergen to the surface of the skin or into the dermis
Administered by patch, scratch, or intradermal techniques
Preprocedure interventions
Discontinue systemic corticosteroids or anti-histamine therapy 5 days before the test as prescribed.
Obtain informed consent
Have resuscitation equipment available if a scratch test is performed, because it may induce an
anaphylactic reaction.
Postprocedure interventions
Record the site, date, and time of the test
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Record the date and time for follow-up site reading
Inspect the site for erythema, papules, vesicles, edema, and wheal
Measure wheal and document size and other findings.
Provide the client with a list of potential allergens, if identified.

Immunodeficiency
The absence or inadequate production of immune bodies
The disorder can be congenital (primary) or acquired (secondary)
Treatment depends on the inadequacy of immune bodies and its primary cause.
Assessment:
1. Factors that decrease immune function
2. Frequent infections
3. Nutritional status
4. Medication history, such as use of corticosteroids for long periods
5. History of alcohol or drug abuse
Interventions:
1. Protect the client from infection
2. Promote a balanced diet with adequate nutrition.
3. Use strict aseptic technique for all procedures.
4. Provide psychosocial care regarding lifestyle changes and role changes.
5. Instruct the client in measures to prevent infection.

Hypersensitivity and allergy
An abnormal, individual response to certain substances that normally do not trigger such an exaggerated reaction.
In some types of allergies, a reaction occurs on a second and subsequent contact with the allergen.
Skin testing may be done to determine the allergen.

Types of Hypersensitivity Reactions
Type Causative
Component
Pathological Process Reaction
I: Immediate, anaphylactic IgE Mast cell degranulation
Histamine and Leukotrine release
Anaphylaxis, Atopic
diseases, Skin reactions
II: Cytolytic, cytotoxic IgG, IgM Complement fixation Cell lysis ABO incompatibility, Drug-
induced hemolytic anemia
III: Immune complex Antigen-antibody
complexes
Deposition in vessels and tissue
walls Inflammation
Arthus reaction, serum
sickness, SLE, AGN
IV: Cell-mediated, delayed Sensitized T cells Lymphokine release Tuberculosis, Contact
dermatitis, Transplant
rejection

Assessment
1. History of exposure to allergens
2. Itching, tearing, and burning of eyes and skin
3. Rashes
4. Nose twitching nasal stuffiness
Interventions
1. Identification of the specific allergen.
2. Management of the symptoms with antihistamines, anti-inflammatory agents, or corticosteroids
3. Ointments, creams, wet compresses, and soothing baths for local reactions.
4. Desensitization programs may be recommended.

Anaphylaxis
A serious and immediate hypersensitivity reaction that releases histamine from the damaged cells.
Anaphylaxis can be systemic or cutaneous (localized)
Assessment
1. Neurologic: headache, dizziness, paresthesia, feeling of impending doom
2. Skin: pruritus, angioedema, erythema, urticarial
3. Respiratory: hoarseness, coughing, sensation of narrowed airway, wheezing, stridor, dyspnea, tachypnea,
respiratory arrest
4. Cardiovascular: hypotension, arrhythmias, tachycardia, cardiac arrest
5. Gastrointestinal: cramping, abdominal pain, nausea, vomiting, diarrhea
Interventions
1. Establish a patent airway
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2. Prepare for the administration of epinephrine (Adrenalin), diphenhydramine hydrochloride (Benadryl), or
corticosteroids.
3. Provide measures to control shock.
4. Provide emotional support.
5. Instruct the client to wear a Medic-Alert bracelet prior to discharge from emergent care.
6. Instruct the client in the use of prescribed medication such as epinephrine (Epipen) for immediate treatment of a
future reaction.

Latex Allergy
A hypersensitivity to latex
The source of the allergic reaction is thought to be the proteins in the natural rubber latex or the various chemicals
used in the manufacturing process of latex gloves.
Symptoms of the allergy can range from mild contact dermatitis to moderately severe symptoms of rhinitis,
conjunctivitis, urticarial, and bronchospasm to severe life-threatening anaphylaxis.

Common routes of exposure:
1. Cutaneous: natural latex gloves and latex balloons
2. Percutaneous and parenteral: intravenous lines and catheters; hemodialysis equipment
3. Mucosal: use of latex condoms, catheters, airways, and nipples
4. Aerosol: aerosolization of powder from latex gloves can occur when gloves are dispensed from the box or when gloves
are removed from the hands.

At risk individuals
1. Health care workers
2. Individuals having multiple surgeries
3. With spina bifida
4. Wear gloves frequently, such as food handlers, hairdressers, and auto mechanics
5. Allergic to kiwis, bananas, pineapples, tropical fruits, grapes, avocados, potatoes, hazelnuts and water chestnuts

Assessment
1. Anaphylaxis or type I hypersensitivity is a response to natural latex
2. A delayed type IV hypersensitivity reaction can occur; symptoms of contact dermatitis include pruritus, edema,
erythema, vesicles, papules, and crusting and thickening of the skin and can occur within 6 to 48 hours.

Autoimmune Disease
Body is unable to recognize its own cells as a part of itself.
Autoimmune disease can affect collagenous tissue.

Lupus Erythematosus
Chronic, progressive, systemic inflammatory disease that can cause major organs and systems to fail
Connective tissue and fibrin deposits collect in blood vessels on collagen fibers and on organs
The deposits lead to necrosis and inflammation in blood vessels, lymph nodes, gastrointestinal tract, and pleura
No cure for the disease is known

Causes
1. The cause of SLE is unknown, but it is believed to be a defect in immunological mechanisms, with a genetic origin
2. Precipitating factors include medications, stress, genetic factors, sunlight or ultraviolet light, and pregnancy.

Assessment
1. Assess for precipitating factors
2. Erythema butterfly or rash of the face
3. Dry, scaly, raised rash on the face or upper body
4. Fever
5. Weakness, malaise, and fatigue
6. Anorexia
7. Weight loss
8. Photosensitivity
9. Joint pain
10. Erythema of the palms
11. Anemia
12. Positive ANA test and lupus erythematosus (LE) preparation
13. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein level

15
Interventions
1. Monitor skin integrity and provide frequent oral care.
2. Instruct the client to clean the skin with a mild soap, avoiding harsh and perfume substances.
3. Assist with the use of ointments and creams for the rash as prescribed.
4. Identify factors contributing to fatigue
5. Administer iron, folic acid, or vitamin supplements as prescribed if anemia occurs.
6. Provide a high-vitamin and high-iron diet.
7. Provide a high-protein diet if there is no evidence of kidney disease.
8. Instruct in measures to conserve energy, such as pacing activities and balancing rest with exercise.
9. Administer topical or systemic corticosteroids, salicylates, and nonsteroidal anti-inflammatory drugs as prescribed for
pain and inflammation.
10. Administer medications to decrease the inflammatory response as prescribed.
11. Instruct the client to avoid exposure to sunlight and ultraviolet light.
12. Monitor for proteinuria and red cell casts in the urine.
13. Monitor for bruising, bleeding, and injury.
14. Assist with plasmapheresis as prescribed to remove autoantibodies and immune complexes from the blood before organ
damage occurs.
15. Monitor for signs of organ involvement such as pleuritis, nephritis, pericarditis, coronary artery disease, hypertenstion,
neuritis, anemia, and peritonitis.
16. Lupus nephritis occurs early in the disease process

Scleroderma (Systemic Sclerosis)
A chronic connective tissue disease similar to SLE that is characterized by inflammation, fibrosis, and sclerosis.
Affects the connective tissue throughout the body.
Causes fibrotic changes involving the skin, synovial membranes, esophagus, heart, lungs, kidneys, and
gastrointestinal tract.
Treatment is directed toward forcing the disease into remission and slowing its progress.
Assessment
1. Pain
2. Stiffness and muscle weakness
3. Pitting edema of the hands and fingers that progresses to the rest of the body
4. Taut and shiny skin that is free from wrinkles
5. Skin tissue is tight, hard, and thick, loses its elasticity, and adheres to underlying structures.
6. Dysphagia.
7. Decreased range of motion
8. Joint contractures
9. Inability to perform activities of daily living
Interventions
1. Encourage activity as tolerated
2. Maintain a constant room temperature
3. Provide small frequent meals, eliminating foods that stimulate gastric secretions, such as spicy foods, caffeine, and
alcohol.
4. Advise the client to sit up for 1 to 2 hours after meals if there is esophageal involvement.
5. Provide supportive therapy as the major organs become affected.
6. Administer corticosteroids as prescribed for inflammation.
7. Provide emotional support and encourage the use of resources as necessary.

Infectious Mononucleosis
Organism: Epstein-Barr virus (EBV)
Mode of Transmission: direct contact with infected blood or secretions
Source: oral secretions
Incubation period: 4 to 6 weeks
Communicability: unknown; viral shedding occurs before onset of symptoms until 6 months or longer after recovery

Assessment
1. Fever, sore throat, headache
2. Malaise and fatigue
3. Nausea and abdominal pain
4. Lymphadenopathy and hepatosplenomegaly

Interventions
1. Supportive care
2. Rest
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3. Assess for abdominal pain. Left upper quadrant or left shoulder pain (signs of ruptured spleen)
4. No contact sport (prevent trauma to the spleen)
5. Avoid contact with oral secretions: no kissing, provide disposable eating utensils

Multiple Myeloma
- a neoplastic condition characterized by the abnormal proliferation of plasma cells in the bone marrow, causing the
development of single or multiple tumors composed of abnormal plasma cells. Disease disseminates into
lymph nodes, liver, spleen, and kidneys and causes bone destruction throughout the body.
- Also called malignant plasmacytoma, plasma cell myeloma, myelomatosis
- Cause unknown, but environment factors thought to be involved.
- Disease occurs after age 40; affects men twice as often as women.
Pathophysiology
- Bone demineralization and destruction with osteoporosis and a negative calcium balance.
- Disruption of erythrocyte, leukocyte, and thrombocyte production.
Assessment findings
1. CRAB: calcium increase, renal failure, anemia, bone fractures
2. Headache and bone pain increasing with activity.
3. Skeletal deformities of sternum and ribs
4. Loss of height (spinal column shortening)
5. Osteoporosis,
6. Renal calculi
7. Neurologic dysfunction: spinal cord compression and paraplegia.
Laboratory tests
1. Radiologic: diffuse bone lesions, widespread demineralization, osteoporosis, osteolytic lesions of skull.
2. Bone marrow; many immature plasma cells; depletion of other cell types.
3. CBC: reduced Hgb, WBC, and platelet counts.
4. Serum globulins elevated
5. Bence-Jones protein: positive (abnormal globulin that appears in the urine of clients with multiple myeloma and
other bone tumors)
Nursing interventions:
1. Comfort measures to help alleviate bone pain.
2. Encourage ambulation to slow demineralization process.
3. Move slowly causes clients are prone to pathologic and other fractures.
4. Encourage fluids: 3000-4000 mL/day to counteract calcium overload and to prevent protein from precipitating in
the renal tubules.
5. Bleeding precaution
6. Protect against infection
Medical management
1. Drug therapy
o Analgesics for bone pain.
o Chemotherapy (melphalan [Alkeran] and cyclophosphamide [Cytoxan]) to reduce tumor mass; may intensify the
pancytopenia to which these clients are prone; requires careful monitoring of laboratory studies.
o Antibiotics to treat infection.
o Gammaglobulin for infection prophylaxis.
o Corticosteroids and mithramycin for severe hypercalcemia.
2. Radiation therapy to reduce tumor mass and for palliation of bone pain.
3. Transfusion therapy
4. Laminectomy
5. Dialysis

RHEUMATOID ARTHRITIS
Chronic, symmetrical, systemic inflammatory disease
Destruction of connective tissue and synovial membrane within the joints that weakens the joint, leading to its
dislocation and permanent deformity
Signs and Symptoms
1. Inflammation, tenderness, and stiffness of the joints
2. Moderate to severe pain with morning stiffness lasting longer than 30 minutes
- Joint deformity Swan neck, Boutonniere, Ulnar deviation
3. Muscle atrophy, and ROM
4. Spongy, soft feeling in the joints
5. Low-grade temperature, fatigue, and weakness
6. Anorexia, weight loss, and anemia
7. Joint deterioration on CXR
8. Synovial tissue biopsy reveals inflammation
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9. Elevated ESR, CRP, ANA, + RA factor
RA factor blood test:
- Nonreactive: 0 39 IU/mL
- Weakly reactive: 40 79 IU/mL
- Reactive: higher than 80 IU/mL
10. Systemic manifestations:
- Acute pericarditis, valvular insufficiency, coronary arteritis
- Pleural effusion and interstitial fibrosis
- Wrist drop, foot drop, carpal tunnel syndrome
- Sjogren syndrome
- Felty syndrome
Management:
1. Balance rest and activity
2. warm & cold applications 15-20 min 3-4X/day
3. warm bath or shower in AM on arising
4. refer to the PT, OT
5. Diet: decrease overall fat intake
- Eating oil-rich, cold water fish @ least 2X/week
6. Adjunctive Aids: long-handled shoehorn, elastic shoelaces, Velcro fasteners, crutches, walker & cane, raised toilets,
splints
7. Adjunctive pain control: TENS, Progressive Skeletal Muscle Relaxation
Drugs:
1. NSAIDs: Ibuprofen (Motrin); Naproxen (Naprosyn)
- With food, assess for bruises & tinnitus
2. DMARDs (Disease modifying antirheumatic drugs)
Hydroxychloroquine (Plaquenil) refer to ophthalmologist
Leflunomide (Arava)
Etanercept (Enbrel) liver enzyme
- liver enzyme check Q monthly for 12 mos; then Q 6 mos.
- A/E: hair loss, wt. loss, GI distress, rash or itching
3. Steroids:
Prednisone (Deltasone), Methylprednisoline (Medrol)
- taper drug adrenal insufficiency
- A/E: cushingoid effect, mask signs of infection, glucose
4. Gold: Penicillamine (Cuprimine, Depen)
- CBC & UA

Acquired Immune Deficiency Syndrome (AIDS)
Characterized by severe deficits in cellular immune function; manifested clinically by opportunities infections
and/or unusual neoplasms
Epidemiology is similar to that of hepatitis B; increased incidence in population in which sexual promiscuity is
common in IV drug abusers.

Etiologic factors
1. Results from infection with human immunodeficiency virus (HIV), a retrovirus that preferentially infects helper T-
lymphocytes (T cells)
2. Transmissible through sexual contact, contaminated blood or blood products, and from infected woman to child in
utero or possibly through breast-feeding
3. HIV is present in an infected persons blood semen, and other body fluids.

Proposed strategies for prevention
1. Early detection; include HIV testing as routine part of medical care
2. Expand opportunities for testing outside of the medical settings.
3. Behavior modification with persons diagnosed with HIV and partners.
4. Reduce viral load in pregnant woman with HIV; reduce perinatal transmission of HIV disease to newborn.

Assessment findings
1. Fatigue, weakness, anorexia, weight loss, diarrhea, pallor, fever, night sweats
2. Shortness of breath, dyspnea, cough, chest pain, and progressive hypoxemia secondary to infection (pneumonia)
3. Progressive weight loss secondary to anorexia, nausea, vomiting, diarrhea and a general wasting syndrome; fatigue,
malaise.
4. Temperature elevations (persistent or intermittent); night sweats
5. Neurologic dysfunction secondary to acute meningitis, progressive dementia, encephalopathy, encephalitis
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6. Presence of opportunistic infection, for example
o Pneumocystic carinii pneumonia
o Herpes simplex, cytomegalovirus, and Epstein-Barr virus
o Candidiasis: oral or esophageal
o Mycobacterium-avium complex
7. Neoplasms
o Kaposis sarcoma
o CNS lymphoma
o Burkitts lymphoma
o Diffuse undifferentiated non-Hodgkins lymphoma

Laboratory findings:
1. Based on clinical criteria
2. Positive HIV antibody test-ELISA (enzyme-linked immunosorbent assay) confirmed by Western blot assay
3. Other lab findings may include
o Leukopenia with profound lymphopenia
o Anemia
o Thrombocytopenia
o Decreased circulatory CD4 lymphocyte cells
o Low CD4: CD8 lymphocyte ratio

Nursing interventions
1. Administer medications as ordered for concomitant disease; monitor for signs of medication toxicity/
2. Monitor respiratory status; provide care as appropriate for respiratory problems, e.g., pneumonia.
3. Assess neurological status; reorient client as needed; provide safety measures for the confused/disoriented client.
4. Assess for signs and symptoms of fluid and electrolyte imbalances; monitor lab studies; ensure adequate hydration.
5. Monitor clients nutritional intake; provide supplements, total parenteral nutrition, etc., as ordered.
6. Assess skin daily (especially perianal area) for signs of breakdown; keep skin clean and dry; turn q4 h while in bed.
7. Inspect oral cavity daily for ulceration, signs of infection; instruct client to rinse mouth with normal saline and
hydrogen peroxide or normal saline and sodium bicarbonate rinses.
8. Observe for signs and symptoms of infection; report immediately if any occur.
9. If severe leucopenia develops institute neutropenic precautions
o Prevent trauma to skin and mucous membranes, e.g., avoid enemas, rectal temperatures; minimize all
parenteral infections.
o Do not place client in a room with clients having infections.
o Screen visitors for colds, infections, etc.
o Do not allow fresh fruits, vegetables, or plants in clients room.
o Mask client when leaving room for walks, X-rays, etc.
10. Institute blood and body fluid precautions.
11. Provide emotional support for client/significant others; help to decrease sense of isolation.
12. Provide client teaching and discharge planning concerning
o Importance of observing for signs of infections and notifying physician immediately if any occur.
o Ways to reduce chance of infection
1. Clean kitchen and bathroom surfaces regularly with disinfectants.
2. Avoid direct contact with pets litter boxes or stool, bird cage droppings, and water in fish tanks.
3. Avoid contact with people with infections, e.g., cold, flu.
4. Importance of balancing activity with rest.
5. Need to eat a well-balanced diet with plenty of fluids.
o Prevention of disease transmission
1. Use safer sex practices, e.g., condoms for sexual intercourse.
2. Do not donate blood, semen, organs.
3. Do not share razors, toothbrushes, or other items that may draw blood.
4. Inform all physicians, dentists, sexual partners of diagnosis.

Medical management
1. No effective cure for AIDS at present; several categories of antiretroviral drugs are available (see table 4-21)
2. Highly active antiretroviral therapy (HAART) refers to combination of antiretroviral drugs to avoid development of
viral resistance to drugs; drug therapy is initiated as early as possible to reduce HIV RNS levels in blood and to
maintain or increase CD4+ levels to greater than 200 cells/uL.
3. Drugs used to treat Pneumocystis carinii pneumonia (PCP) include:
o PO or IV trimethropim-sulfamethoxazole (Bactrim, Septra ); side effects include rash, leucopenia, fever
o IM or IV pentamidine (Pentam 300); side effects include hepatotoxicity, nephrotoxicity, blood sugar
imbalances, abscess or necroses at IM injection site, hypotension.
o Corticosteroids are also frequently used.
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Antiretroviral Drug Classifications
1. Nucleoside Reverse Transcriptase Inhibitors (NRTIs)
o Zidovudine (AZT, ZDV, Retrovir) the first developed drug
o Didanosine (ddl, Videx)
o Stavudine (d4T, Zerit)
o Lamivudine (3TC, Epivir)
o Abacavir (Ziagen)
2. Nucleotide Reverse Transcriptase Inhibitor: Tenofovir DF (viread)
3. Nonnucleoside Reverse Transcriptase Inhibitors (NNRTIs)
o Nevirapine (Viramune)
o Delavirdne (Rescriptor)
o Efavirenz (Sustival)
4. Protease Inhibitors (Pls)
o Saquinavir (Fortovase)
o Indinavir (Crixivan)
o Ritonavir (Norvir)
o Nelfinavir (Viracept)
o Amprenavir (Agenerase)
o Kaletra (combination of lopinavir and ritonvir)
5. Fusion Inhibitor (Entry Inhibitor): Enfuvirtide (Fuzeon)