Interventions for Clients with Hematologic Problems

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Interventions for Clients with Hematologic
Problems

• RED BLOOD CELL DISORDERS

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• Disorders of the hematologic system can occur as
  a result of problems in the production, function,
  or normal destruction of any type of blood cell.
• The type and severity of the specific disorder
  determine the degree of threat to the client's
  well-being

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RED BLOOD CELL DISORDERS

• The major cellular population of the blood
  consists of red blood cells (RBCs), or
  erythrocytes.
• Adequate tissue oxygenation depends on
  maintaining the circulating number of RBCs within
  the normal range for the person's age and gender
  and ensuring that the cells can perform their
  normal functions. RBC disorders include problems
  in production, function, and destruction.
• These problems may result in an insufficient
  number or insufficient function of RBCs (anemia)
  or an excess of RBCs (polycythemia).

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RED BLOOD CELL DISORDERS

• Anemia is a reduction in either the number of
  RBCs, the quantity of hemoglobin, or the
  hematocrit (percentage of packed RBCs per
  deciliter of blood).
• Anemia is a clinical sign, not a diagnosis,
  because it is a manifestation of a number of
  abnormal conditions.
• Despite the many causes of anemia, the effects of
  anemia on the client and the corresponding
  nursing care are similar for all types of anemia

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Sickle cell disease is a condition in which
  chronic anemia is one of many problems causing
  pain, disability, increased risk for disease, and
  early death.
• Once considered a childhood disorder, clients
  with sickle cell disease who receive appropriate
  supportive care may live into their 30s and 40s.
• In addition, there is great variation among
  clients in the severity of the disease and the
  onset of complications.

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Pathophysiology
• Hereditary disorder - formation of abnormal beta
  chains in the hemoglobin molecule.
• The normal hemoglobin molecule of adults is
  composed partially of the globin protein,
  consisting of two alpha chains and two beta
  chains of amino acids (hemoglobin A (HbA)). The
  total hemoglobin of normal healthy adults is
  usually 98 to 99 HbA, with a small percentage
  of a fetal form of hemoglobin (HbF).
• In sickle cell disease, at least 40 of the total
  hemoglobin contains an abnormality of the beta
  chains, known as hemoglobin S (HbS).

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• HbS is sensitive to changes in the oxygen content
  of the RBC. When RBCs containing large amounts of
  HbS are exposed to conditions of decreased
  oxygen, the abnormal beta chains contract and
  pile together within the cell, distorting the
  overall shape of the RBC.
• These cells assume a sickle shape, become rigid,
  clump together, and form clusters that block
  capillary blood flow.
• Capillary obstruction leads to further tissue
  hypoxia (reduced oxygen supply) and more
  sickling, causing blood vessel obstructions and
  infarctions in the locally affected tissues.
• Situations that lead to sickling include hypoxia,
  dehydration, infections, vascular stasis, low
  environmental or body temperatures, acidosis,
  strenuous exercise, and anesthesia

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Red blood cell actions under conditions of low
tissue oxygenation. (HbS, Hemoglobin S HbA,
hemoglobin A.)
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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Usually, sickled cells resume a normal shape when
  the precipitating condition is removed and proper
  oxygenation occurs.
• The membranes of the cells become damaged over
  time, and cells become irreversibly sickled.
• The membranes of cells with HbS are more fragile
  and more easily destroyed in the spleen and in
  other organs that have long, twisted capillary
  pathways. The average life span of an RBC
  containing 40 or more of HbS is approximately 20
  days. This reduced life span is responsible for
  hemolytic (blood cell-destroying) anemia in
  clients with sickle cell disease.

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• The client with sickle cell disease experiences
  periodic episodes of extensive cellular sickling,
  or crises.
• Repeated occlusions of progressively larger blood
  vessels have long-term negative effects on
  tissues and organs. Most effects are thought to
  occur as a result of capillary and blood vessel
  occlusion leading to tissue hypoxia, anoxia,
  ischemia, and cell death.
• Tissues and organs begin to have small infarcted
  areas that eventually destroy all healthy cells
  and lead to organ failure.
• Tissues and organs most commonly affected in this
  way are the spleen, liver, heart, kidney, brain,
  bones, and retina

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Etiology
• Sickle cell disease is a genetic disorder with an
  autosomal recessive pattern of inheritance.
• The formation of the beta chains of the
  hemoglobin molecule is dependent on a pair of
  genes.
• When the client inherits one abnormal gene of
  this pair, the condition is called sickle cell
  trait.
• When the client inherits two abnormal genes, the
  condition is called sickle cell disease (formerly
  sickle cell anemia), and the client has severe
  manifestations of the disease even under
  relatively mild precipitating conditions. In
  addition, if the client has children, each child
  will inherit one of the two abnormal genes and at
  least have sickle cell trait

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Cultural considerations
• Sickle cell disease occurs most often in African
  Americans, as well as in African, Mediterranean,
  Caribbean, Middle Eastern, and Central American
  populations.
• Approximately 1 of every 12 African Americans has
  the sickle cell trait.
• One of every 345 African-American infants
  inherits two abnormal genes (one from each
  parent) and has overt sickle cell disease

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• History
• An adult with sickle cell disease has a
  long-standing diagnosis of the disorder.
• The nurse asks the client about previous crises,
  including precipitating events, severity, and
  usual treatments.
• Recent activities and situations are explored to
  determine the probable precipitating condition or
  event.
• The nurse also reviews all activities and events
  during the previous 24 hours, including food and
  fluid intake, exposure to temperature extremes,
  types of clothing worn, medications taken,
  exercise, trauma, stress, and ingestion of
  alcohol or other recreational drugs. This
  activity review provides important information
  about fatigue, activity tolerance, and
  participation in activities of daily living
  (ADLs).

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• The client is asked about changes in sleep and
  rest patterns, ability to climb stairs, and any
  activity that induces shortness of breath.
• Obtaining a subjective baseline assessment of the
  client's perceived energy level using a scale
  ranging from 0 to 10 (0 not tired with plenty
  of energy 10 total exhaustion) can be useful
  in evaluating the degree of fatigue and the
  effectiveness of later treatments

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Physical assessment/clinical manifestations
• Pain is the most common symptom experienced
  during sickle cell crisis.
• Jaundice may also be present as a result of
  increased red blood cell (RBC) destruction and
  release of bilirubin.
• Other clinical manifestations vary with the site
  of tissue damage.
• Cardiovascular assessment
• Compare peripheral pulses, temperature, and
  capillary refill in all extremities
• Extremities distal to blood vessel occlusion are
  cool to the touch with slow capillary refill and
  may have diminished or absent pulses.
• The heart rate may be rapid and the blood
  pressure low to average, with a decreased pulse
  pressure because breakage of RBCs leads to anemia

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Integumentary assessment
• The skin may be pale or cyanotic as a result of
  decreased perfusion and anemia. The nurse
  examines the lips, tongue, nail beds,
  conjunctivae, palms, and soles at regular
  intervals for subtle color changes. With
  cyanosis, the lips and tongue are gray, and the
  palms, soles, conjunctivae, and nail beds have a
  bluish tinge.
• Jaundice. The nurse assesses for jaundice in
  clients with darker skin by inspecting the oral
  mucosa, especially the hard palate, for yellow
  discoloration. Inspection of the conjunctivae and
  adjacent sclera may be misleading because of
  normal deposits of subconjunctival fat that
  produce a yellowish hue when seen in contrast to
  the dark periorbital skin. Therefore the nurse
  examines the sclera closest to the cornea to
  diagnose jaundice more accurately. Jaundice from
  excessive bilirubin may also cause intense
  itching.

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Abdominal assessment
• Abdominal organs are usually the first to be
  damaged as a result of multiple episodes of
  hypoxia and ischemia. The nurse inspects the
  abdomen for asymmetry or bulging areas, gently
  palpating it. Affected organs, such as the liver
  or spleen, may be firm and enlarged with a
  nodular texture in later stages of the disease
• Musculoskeletal assessment
• Extremities are a common site of vascular
  occlusion among clients who have sickle cell
  disease. In addition, joints may be damaged from
  frequent hypoxic episodes and undergo necrotic
  degeneration.
• The nurse inspects the extremities for symmetry
  and records any areas of swelling or color
  difference. Clients are asked to move all joints,
  and the nurse notes the range of motion and any
  accompanying pain

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Central nervous system assessment
• During crises, clients may have a low-grade
  fever. If the CNS sustains infarcts or repeated
  episodes of hypoxia, they may have seizure
  activity or clinical manifestations of a stroke.
  Hand grasps are assessed bilaterally. The nurse
  assesses gait and coordination in those clients
  who are permitted to walk.
• Laboratory assessment
• Large percentage of hemoglobin S (HbS) present on
  electrophoresis. A person who has sickle cell
  trait usually expresses less than 40 HbS, and
  the client with sickle cell disease may express
  85 to 95 HbS. This percentage does not change
  during crises.
• Another indicator of sickle cell disease is the
  percentage of RBCs showing irreversible sickling.
  This value is less than 1 among people who do
  not have sickle cell disease, is 5 to 50 among
  people with sickle cell trait, and may exceed 90
  among clients with sickle cell disease

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• The hematocrit of clients with sickle cell
  disease is usually low (between 20 and 30).
  This value decreases even more dramatically
  during vascular occlusive crises, or aplastic
  crises, when the bone marrow temporarily fails to
  produce cells during stressful periods (such as
  infection).
• The reticulocyte count is elevated, indicating
  anemia of long duration. Often the mean
  corpuscular hemoglobin concentration (MCHC) and
  total bilirubin level are elevated in the client
  who has sickle cell disease.
• The total white blood cell (WBC) count is usually
  above normal among clients who have sickle cell
  disease. It is thought that this elevation is
  related to chronic inflammation resulting from
  tissue hypoxia and ischemia

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Radiographic assessment
• Bone changes occur as a result of chronically
  stimulated marrow and hypoxic bone tissue. The
  skull may show radiographic changes resulting
  from chronic bone surface resorption and
  regeneration, giving the skull a "crew cut"
  appearance. Joint necrosis and degeneration also
  are obvious on x-ray examination.
• Other diagnostic assessment
• Electrocardiographic (ECG) changes document
  cardiac infarcts and tissue damage.
• Ultra-sonography, computed tomography (CT),
  positron emission tomography (PET), and magnetic
  resonance imaging (MRI) may reveal soft-tissue
  and organ degenerative changes resulting from
  inadequate oxygenation and chronic inflammation

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Interventions
• PAIN DRUG THERAPY. Clients in acute sickle cell
  crisis often require at least 48 hours of
  parenteral analgesics.
• Morphine and hydromorphone (Dilaudid) are the
  medications of choice
• For sickle cell crisis, these agents should be
  administered intravenously on a routine schedule.
  Once relief is obtained, the intravenous (IV)
  dose can be tapered and then administered orally
• Meperidine (Demerol) is also used for sickle cell
  crisis, but long-term use of this agent can cause
  neurologic symptoms, including anxiety and
  seizures
• Intramuscular (IM) injections are avoided because
  frequent injections lead to sclerosing of tissue
  (and absorption may be impaired by poor
  circulation).
• Moderate pain may be treated with oral doses of
  codeine, morphine sulfate, or nonsteroidal
  antiinflammatory drugs (NSAIDs)

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• Complementary therapies and other
  nonpharmacologic measures, such as keeping the
  room warm, using distraction and relaxation
  techniques, proper positioning with support for
  painful areas, aroma therapy, therapeutic touch,
  and warm soaks or compresses, have all been
  useful in decreasing pain.
• The nurse must not assume, however, that these
  methods alone will provide adequate pain relief.
  Analgesics are required to treat sickle cell pain

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• POTENTIAL FOR SEPSIS. The client with sickle cell
  disease is more susceptible to bloodborne
  infections and infection by encapsulated
  microorganisms, such as Streptococcus pneumoniae
  and Haemophilus influenzae, as a result of
  decreased spleen function. Interventions aim at
  preventing or halting the process of infection,
  controlling infection, and initiating early,
  effective treatment regimens for specific
  infections.
• PREVENTION/EARLY DETECTION. Frequent, thorough
  handwashing is of the utmost importance. Any
  person with an upper respiratory tract infection
  who must enter the client's room wears a mask.
  Strict aseptic technique is used for all invasive
  procedures.
• The nurse continually assesses the client for the
  presence of infection and monitors a daily
  complete blood count (CBC) with differential WBC
  count. The oral mucosa is inspected during every
  nursing shift for lesions indicating fungal or
  viral infection. The lungs are auscultated every
  8 hours for crackles, wheezes, or diminished
  breath sounds. Each time the client voids,
  assistive nursing personnel inspect the urine for
  odor and cloudiness, and the client is asked
  about any sensation of urgency, burning, or pain
  during urination. Vital signs are taken at least
  every 4 hours to assess for fever

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• DRUG THERAPY. Prophylactic therapy with
  twice-daily administration of oral penicillin in
  the penicillin-tolerant client has resulted in
  dramatic reductions in the number of pneumonia
  and other streptococcal infections. Agents used
  depend on the sensitivity of the specific
  organism causing the infection, as well as the
  extent of the infection

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLS Sickle Cell Disease

• POTENTIAL FOR MULTIPLE ORGAN DYSFUNCTION
• The client in sickle cell crisis is admitted to
  the acute care hospital. The nurse assesses for
  adequacy of circulation to all body areas.
  Restrictive clothing is removed, and the client
  is instructed to avoid keeping the hips or knees
  in a flexed position.
• Dehydration perpetuates cell sickling and must be
  avoided. Nursing personnel assist the client in
  maintaining an adequate hydration status. The
  client in crisis requires an oral or parenteral
  intake of at least 200 mL/hr.
• Oxygen is ordered, and the nurse ensures that
  oxygen therapy is delivered appropriately,
  including nebulization to prevent dehydration.
• Transfusion therapy has been used to decrease the
  incidence of organ dysfunction and stroke. RBC
  transfusions are therapeutic because levels of
  hemoglobin A (HbA) are sustained, whereas levels
  of hemoglobin S (HbS) are diluted. Transfusions
  also suppress erythropoiesis, thereby decreasing
  the production of sickle cells. Transfusions may
  be administered in either the acute care or
  clinic setting by a registered nurse. The nurse
  monitors the client closely for complications of
  transfusion therapy
• In some treatment centers, bone marrow
  transplantation is being performed to correct
  abnormal hemoglobin permanently. Because bone
  marrow transplantation is expensive and may
  result in chronic and life-threatening
  complications, its risks and benefits need to be
  seriously considered for each client

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLSGlucose-6-Phosphate Dehydrogenase
Deficiency Anemia

• Overview
• Many forms of congenital hemolytic (blood
  cell-destroying) anemia result from defects or
  deficiencies of one or more enzymes within the
  red blood cell (RBC). More than 200 such
  disorders have been identified. Most of these
  enzymes are needed to complete some critical step
  in cellular energy production. The most common
  type of congenital hemolytic anemia is associated
  with a deficiency of the enzyme
  glucose-6-phosphate dehydrogenase (G6PD). This
  disease is inherited as an X-linked recessive
  disorder and affects about 10 of all African
  Americans.
• G6PD stimulates critical reactions in the
  glycolytic pathway. RBCs contain no mitochondria,
  so active glycolysis is essential for energy
  metabolism. Newly produced RBCs from clients with
  G6PD deficiency have relatively sufficient
  quantities of G6PD however, as the cells age,
  the concentration diminishes drastically. Cells
  that have reduced amounts of G6PD are more
  susceptible to breaking during exposure to
  specific drugs (e.g., phenacetin, sulfonamides,
  aspirin acetylsalicylic acid, quinine
  derivatives, thiazide diuretics, and vitamin K
  derivatives) and toxins.
• After exposure to any of these agents, clients
  experience acute intravascular hemolysis lasting
  from 7 to 12 days. During this acute phase,
  anemia and jaundice develop. The hemolytic
  reaction is self-limited because only older
  erythrocytes, containing less G6PD, are destroyed

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLSGlucose-6-Phosphate Dehydrogenase
Deficiency Anemia

• Collaborative management
• It is critical that the precipitating drug or the
  agent responsible for the hemolytic reaction be
  identified and totally removed. People should be
  screened for this deficiency before donating
  blood, because administration of cells deficient
  in G6PD can be hazardous for the recipient.
• During and immediately after an episode of
  hemolysis, adequate hydration is essential to
  prevent precipitation of cellular debris and
  hemoglobin in the kidney tubules, which can lead
  to acute tubular necrosis. Osmotic diuretics,
  such as mannitol (Osmitrol), may assist in
  preventing this complication. Transfusion therapy
  is indicated when anemia is present and kidney
  function is normal

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLSImmunohemolytic Anemia

• Overview
• Increased RBC destruction through hemolysis can
  occur in response to many situations, including
  trauma, infection (especially malarial
  infections), and autoimmune reactions. All
  increase the rate at which RBCs are destroyed by
  causing lysis (breakage) of the RBC membrane.
• In immunohemolytic anemia, immune system
  components attack a person's own RBCs. The exact
  mechanism that causes immune components to no
  longer recognize blood cells as self and to
  initiate destructive processes against RBCs is
  not known. Some hemolytic anemias are present
  with other autoimmune disorders (such as systemic
  lupus erythematosus) or lymphoproliferative
  disorders. Regardless of the cause, RBCs are
  viewed as non-self by the immune system and are
  destroyed.

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLSImmunohemolytic Anemia

• There are two types of immunohemolytic anemia
  warm antibody anemia and cold antibody anemia.
• Warm antibody anemia is usually associated with
  immunoglobulin G (IgG) antibody excess. These
  antibodies are most active at 98 F (37 C) and
  may be stimulated by drugs, chemicals, or other
  autoimmune problems.
• Cold antibody anemia is associated with fixation
  of complement proteins on immunoglobulin M (IgM)
  and occurs best at 86 F (30 C). This problem is
  commonly associated with a Raynaud-like response
  in which the arteries in the distal extremities
  constrict profoundly in response to cold
  temperatures or stress

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ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF
RED BLOOD CELLSImmunohemolytic Anemia

• Collaborative management
• Treatment depends on clinical severity. Steroid
  therapy for mild to moderate immunosuppression is
  the first line of treatment and is temporarily
  effective in most clients. Splenectomy and more
  intensive immunosuppressive therapy with
  cyclophosphamide (Cytoxan, Procytox) and
  azathioprine (Imuran) may be instituted if
  steroid therapy fails. Plasma exchange therapy to
  remove attacking antibodies is effective for
  clients who do not respond to immunosuppressive
  therapy

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Indications for treatment with blood components
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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSIron Deficiency Anemia

• Overview
• The adult body contains between 2 and 6 g of
  iron, depending on the size of the person and the
  amount of hemoglobin in the cells.
• Approximately two thirds of this iron is
  contained in hemoglobin the other third is
  stored in the bone marrow, spleen, liver, and
  muscle.
• If a person has an iron deficiency, the iron
  stores are depleted first, followed by the
  hemoglobin stores. As a result, RBCs are small
  (microcytic), and the client has relatively mild
  manifestations of anemia, including weakness and
  pallor. In iron deficiency anemia, serum ferritin
  values are less than 12 g/L.
• Iron deficiency anemia is the most common type of
  anemia and can result from blood loss, increased
  energy demands, gastrointestinal malabsorption,
  and dietary inadequacy.
• The basic problem of iron deficiency anemia is a
  decreased supply of iron for the developing RBC.
  Iron deficiency anemia can occur at any age but
  is more frequent in women, older adults, and
  people with poor diets

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSIron Deficiency Anemia

• Collaborative management
• The primary treatment of clients with iron
  deficiency anemia is to increase the oral intake
  of iron from common food sources.
• An adequate diet supplies a person with about 10
  to 15 mg of iron per day, of which only 5 to 10
  is absorbed in the stomach, duodenum, and upper
  jejunum. This amount is sufficient to meet the
  needs of healthy men and healthy women after
  childbearing age but is not sufficient to supply
  the greater needs of menstruating women and
  adolescents during growth spurts.
• Fortunately, if iron intake is inadequate, or if
  bleeding or pregnancy occurs, the
  gastrointestinal tract is capable of increasing
  the absorption of iron to about 20 to 30 of the
  total daily intake.
• When iron deficiency anemia is severe, iron
  preparations can be administered intramuscularly.
  Such preparations are administered using the
  Z-track best practice method

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Common food sources of iron, vitamin b12, and
folic acid
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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSVitamin B12 Deficiency Anemia

• Overview
• Proper production of RBCs depends on adequate
  desoxyribonucleic acid (DNA) synthesis in the
  precursor cells so that cell division and
  maturation into functional RBCs can occur.
• All DNA synthesis requires adequate amounts of
  folik acid to ensure the availability of the
  nucleotide thymidine, which stimulates DNA
  synthesis. One function of vitamin B12 is to
  serve as a cofactor to activate the enzyme system
  responsible for transporting folic acid into the
  cell, where DNA synthesis occurs.
• Thus a deficiency of vitamin B12 indirectly
  causes anemia by inhibiting folic acid
  transportation and limiting DNA synthesis in RBC
  precursor cells.
• These precursor cells then undergo improper DNA
  synthesis and increase in size. Only a few are
  released from the bone marrow. This type of
  anemia is called megaloblastic (macrocytic)
  because of the large size of these abnormal
  cells.

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSVitamin B12 Deficiency Anemia

• Vitamin B12 deficiency can result from inadequate
  intake (dietary deficiency). This can occur with
  strict vegetarian diets or diets lacking
  sufficient dairy products.
• Conditions such as small bowel resection,
  diverticula, tapeworm, or overgrowth of
  intestinal bacteria can lead to poor absorption
  of vitamin B12 from the intestinal tract.
• Anemia caused by failure to absorb vitamin B12
  (pernicious anemia) can also result from a
  deficiency of intrinsic factor (a substance
  normally secreted by the gastric mucosa), which
  is necessary for intestinal absorption of vitamin
  B12.
• Vitamin B12 deficiency anemia may be mild or
  severe, usually develops slowly, and produces few
  symptoms. Clients usually have pallor and
  jaundice, as well as glossitis (a smooth,
  beefy-red tongue), fatigue, and weight loss.
• Because vitamin B12 also is necessary for normal
  nervous system functioning, especially of the
  peripheral nerves, clients with pernicious anemia
  may also have neurologic abnormalities, such as
  paresthesias (abnormal sensations) in the feet
  and hands and disturbances of balance and gait.

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSVitamin B12 Deficiency Anemia

• Collaborative management
• When anemia is caused by a dietary deficiency,
  the client must increase the intake of foods rich
  in vitamin B12 (animal proteins, eggs, dairy
  products).
• Vitamin supplements may be prescribed when anemia
  is severe. For clients who have anemia as a
  result of a deficiency of intrinsic factor,
  vitamin B12 must be administered parenterally on
  a regular schedule (usually weekly for initial
  treatment, then monthly for maintenance).

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSFolic Acid Deficiency Anemia

• Overview
• Primary folic acid deficiency can also cause
  megaloblastic anemia. Clinical manifestations are
  similar to those of vitamin B12 deficiency
  without the accompanying nervous system
  manifestations, because folic acid does not
  appear to affect nerve function.
• The absence of neurologic problems is an
  important diagnostic finding to differentiate
  folic acid deficiency from vitamin B12
  deficiency. The disease develops slowly, and
  symptoms may be attributed to other problems or
  diseases.
• The three common causes of folic acid deficiency
  are poor nutrition, malabsorption, and drugs.
  Poor nutrition, especially a diet lacking green
  leafy vegetables, liver, yeast, citrus fruits,
  dried beans, and nuts, is the most common cause.
  Chronic alcohol abuse and parenteral alimentation
  without folic acid supplementation are other
  dietary causes.
• Malabsorption syndromes, such as Crohn's disease,
  are the second most common cause.
• Specific drugs impede the absorption and
  conversion of folic acid to its active form and
  can also lead to folic acid deficiency and
  anemia. Such drugs include methotrexate, some
  anticonvulsants, and oral contraceptives

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSFolic Acid Deficiency Anemia

• Collaborative management
• Prevention of folic acid deficiency anemia is
  aimed at identifying high-risk clients, such as
  older, debilitated clients with alcoholism
  clients prone to malnutrition and those with
  increased folic acid requirements.
• A diet high in folic acid and vitamin B12
  prevents a deficiency.
• By routinely including assessment of dietary
  habits in a health history, the nurse can
  determine which clients are at risk for
  diet-induced anemias and provide appropriate
  follow-up.
• For the client diagnosed with this type of
  anemia, management includes oral folic acid 1 mg
  daily or intramuscular administration of folic
  acid for clients with absorption problems.

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSAplastic Anemia

• Overview
• Aplastic anemia is a deficiency of circulating
  erythrocytes resulting from arrested development
  of RBCs within the bone marrow. It is caused by
  an injury to the hematopoietic precursor cell,
  the pluripotent stem cell.
• Although aplastic anemia sometimes occurs alone,
  it is usually accompanied by agranulocytopenia (a
  reduction in leukocytes) and thrombocytopenia (a
  reduction in platelets).
• These three problems occur at the same time
  because the bone marrow produces not only RBCs
  but also white blood cells (WBCs) and platelets.
• Consequently, if the bone marrow is abnormal for
  any reason or if it has been exposed to a toxic
  substance that can damage bone marrow cells,
  production of erythrocytes, leukocytes, and
  thrombocytes slows greatly. Pancytopenia (a
  deficiency of all three cell types) is common in
  aplastic anemia.

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSAplastic Anemia

• The onset of aplastic anemia may be insidious or
  rapid.
• The development of aplastic anemia, although
  relatively rare, is associated with chronic
  exposure to several toxic agents. In about 50 of
  cases, the cause of aplastic anemia is unknown.
  Aplastic anemia may occur as an aftermath of
  viral infection, but the mechanism of bone marrow
  damage is unknown

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSAplastic Anemia

• Collaborative management
• Blood transfusions are the mainstay of treatment
  for clients with aplastic anemia.
• Transfusion is indicated only when the anemia
  causes real disability or when bleeding is life
  threatening because of thrombocytopenia.
• Unnecessary transfusion, however, increases the
  opportunity for the development of immune
  reactions to platelets, shortens the life span of
  the transfused cell, and may increase the rate of
  rejection of transplanted marrow cells. Thus
  transfusions are discontinued as soon as the bone
  marrow begins to produce RBCs.

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ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF
RED BLOOD CELLSAplastic Anemia

• Because clients with some types of aplastic
  anemia have a disease course similar to that of
  autoimmune problems, immunosuppressive therapy
  may be helpful. Agents that selectively suppress
  lymphocyte activity, such as antilymphocyte
  globulin (ALG), antithymocyte globulin (ATG), and
  cyclosporine (Sandimmune), have brought about
  partial or complete remissions. In more severe
  cases, general immunosuppressive agents, such as
  prednisone and cyclophosphamide (Cytoxan,
  Procytox), have been effective.
• Splenectomy (removal of the spleen) is considered
  in clients with an enlarged spleen that is either
  destroying normal RBCs or suppressing their
  development.
• Bone marrow transplantation, which replaces
  defective stem cells, has also resulted in a cure
  for some clients. Cost, availability, and
  complications limit this technique for treatment
  of aplastic anemia, however

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POLYCYTHEMIA

• In polycythemia, the number of red blood cells
  (RBCs) in whole blood is greater than normal.
• The blood of a client with polycythemia is
  hyperviscous (thicker than normal blood).
• The problem may be temporary (occurring as a
  result of other conditions) or chronic. One type
  of polycythemia, polycythemia vera, is fatal if
  left untreated.

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Polycythemia Vera

• Overview
• Polycythemia vera (PV) is characterized by a
  sustained increase in blood hemoglobin
  concentration to 18 g/dL, an RBC count of 6
  million/mm3, or a hematocrit increase to 55 or
  greater.
• PV is a cancer of the RBCs with three major hall
  marks continuous production of massive numbers
  of RBCs, excessive leukocyte production, and
  overproduction of thrombocytes. Extreme
  hypercellularity (cell excess) of the peripheral
  blood occurs in people with PV
• The skin, especially facial, and mucous membranes
  have a dark, flushed (plethoric) appearance.
  These areas may appear purplish or cyanotic
  because the blood in these tissues is
  incompletely oxygenated. Most clients experience
  intense itching related to vasodilation and
  variation in tissue oxygenation.

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Polycythemia Vera

• Blood viscosity is also greatly increased,
  causing a corresponding increase in peripheral
  resistance.
• Superficial veins are visibly distended. Blood
  moves more slowly through all tissues and thus
  places increased demands on the pumping action of
  the heart, resulting in hypertension. In some
  highly vascular areas, blood flow may become so
  slow that vascular stasis occurs. Vascular stasis
  causes thrombosis (clot formation) within the
  smaller vessels to the extent that the vessels
  are occluded and the surrounding tissues
  experience hypoxia, progressing to anoxia and
  further to infarction and necrosis. Tissues most
  prone to this complication are the heart, spleen,
  and kidneys, although infarction with loss of
  tissue and organ function can occur in any organ
  or tissue

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Polycythemia Vera

• Because the actual number of cells in the blood
  is greatly increased and the cells are not
  completely normal, individual cell life spans are
  shorter. The shorter life spans, coupled with
  increased cell production, result in a rapid
  turnover of peripheral blood cells. This rapid
  turnover increases the amount of intracellular
  products (released when cells die) in the blood,
  adding to the general "sludging" of the blood.
  These products include uric acid and potassium,
  which cause the symptoms of gout and hyperkalemia
  (elevated serum potassium level).
• Later clinical manifestations of PV are related
  to abnormal blood cells. Even though the number
  of circulating erythrocytes is greatly increased,
  their oxygencarrying capacity is impaired, and
  clients experience severe generalized hypoxia. In
  spite of the RBC excess, most clients with PV are
  susceptible to bleeding problems because of an
  associated platelet dysfunction

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Polycythemia Vera

• Collaborative management
• Polycythemia vera is a malignant disease that
  progresses in severity over time. If left
  untreated, few people with PV live longer than 2
  years.
• Conservative management with repeated
  phlebotomies (two to five times per week) can
  prolong life for 5 to 10 years. (Phlebotomy is
  the collection of the client's RBCs to decrease
  the number of RBCs and diminish blood viscosity.)
  
• Maintaining adequate hydration and promoting
  venous return are essential to prevent thrombus
  formation. Therapy aims to prevent clot formation
  and includes the use of anticoagulants
• As the disease progresses, clients need more
  intensive therapies that suppress bone marrow
  activity, including oral alkylating agents and/or
  irradiation with injections of radioactive
  phosphorus.
• Bone marrow transplantation, an experimental
  treatment, is promising, but the results are too
  limited to determine its application to PV