ANEMIA

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ANEMIA

• Mrs. Mahdia Samaha Alkony

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Definition

• Anemia, a condition in which the hemoglobin
  concentration is lower than normal, reflects the
  presence of fewer than normal RBCs within the
  circulation.
• As a result, the amount of oxygen delivered to
  body tissues is also diminished.

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Classifications of anemia according to etiology

• Loss of RBCsoccurs with bleeding, potentially
  from any major source, such as the
  gastrointestinal tract, the uterus, the nose, or
  a wound
• Decreased production of RBCscan be caused by a
  deficiency in cofactors (including folic acid,
  vitamin B12, and iron) required for
  erythropoiesis RBC production may also be
  reduced if the bone marrow is suppressed (eg, by
  tumor, medications, toxins) or is inadequately
  stimulated because of a lack of erythropoietin
  (as occurs in chronic renal disease).
• Increased destruction of RBCsmay occur because
  of an overactive RES (including hypersplenism) or
  because the bone marrow produces abnormal RBCs
  that are then destroyed by the RES (eg, sickle
  cell anemia).

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Classification of Anemias

• Hypoproliferative anemia the deficiency in RBCs
  is caused by a defect in their production. The
  RBCs usually survive normally, but the marrow
  cannot produce adequate numbers of these cells.
• Hemolytic anemia by RBCs destruction
• Bleeding by RBCs loss.
• In the hypoproliferative anemias,

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Clinical Manifestations

• Several factors influence the development of
  anemia-associated symptoms
• The speed with which the anemia has developed
• The duration of the anemia (ie, its chronicity)
• The metabolic requirements of the individual
• Other concurrent disorders or disabilities (eg,
  cardiopulmonary disease)
• Special complications or concomitant features
  of the condition that produced the anemia

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Hypoproliferative Anemias

• IRON DEFICIENCY ANEMIA

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Etiology

• Inadequate dietary intake of iron The body can
  store about one fourth to one third of its iron.
• Most common anemia in the world and can affect
  all groups.
• More common in underdeveloped countries, where
  inadequate iron stores or from blood loss (eg,
  from intestinal hookworm).

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Etiology

• In men and postmenopausal women the most common
  cause is bleeding (from ulcers, gastritis,
  inflammatory bowel disease, or gastrointestinal
  tumors).
• In premenopausal women the most common cause is
  menorrhagia (excessive menstrual bleeding) and
  pregnancy with inadequate iron supplementation.
• Chronic alcoholism
• Malabsorption as is seen after gastrectomy or
  with celiac disease.

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Clinical Manifestations

• If the deficiency is severe or prolonged
• smooth, sore tongue
• brittle and ridged nails
• angular cheilosis an ulceration of the corner of
  the mouth.
• These signs subside after iron-replacement
  therapy.

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Assessment and Diagnostic Findings

• Health history
• multiple pregnancies
• gastrointestinal bleeding
• pica (a craving for unusual substances, such as
  ice, clay, or laundry starch).
• Clinical picture Weakness, fatigue, and general
  malaise, pallor of the skin and mucous membranes
  (sclera, oral mucosa).

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Assessment

• A nutritional assessment is important, because it
  may indicate iron deficiency
• Cardiac status should be carefully assessed
• Gastrointestinal Assessment of nausea, vomiting
  (with specific questions as to the appearance of
  any emesis eg, looks like coffee grounds),
  melena, diarrhea, anorexia, and glossitis
• Stools for occult blood.
• Women should be questioned about their menstrual
  periods (eg, excessive menstrual flow, other
  vaginal bleeding) and the use of iron supplements
  during pregnancy.

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Diagnostic Findings

• Bone marrow aspiration. The aspirate is stained
  to detect iron.
• low serum ferritin levels
• low hemoglobin level. The diminished iron stores
  cause small RBCs
• MCV, which measures the size of the RBC, also
  decreases.
• Hematocrit and RBC levels are also low in
  relation to the hemoglobin level.

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Diagnostic Findings

• Low serum iron level and an elevated TIBC, which
  measures the transport protein supplying the
  marrow with iron as needed (also referred to as
  transferrin).
• However, other disease states, such as infection
  and inflammatory conditions, can also cause a low
  serum iron level and TIBC with an elevated
  ferritin level.
• Therefore, the most reliable laboratory findings
  in evaluating iron deficiency anemia are the
  ferritin and hemoglobin values.

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Medical Management

• Investigate the cause (gastrointestinal cancer or
  uterine fibroid tumor)
• Stool specimens for occult blood.
• People 50 years of age or older should have a
  colonoscopy, endoscopy, or other examination of
  the gastrointestinal tract to detect ulcerations,
  gastritis, polyps, or
• cancer.

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Iron supplement

• oral iron preparationsferrous sulfate, ferrous
  gluconate, and ferrous fumarate
• intravenous or intramuscular administration of
  iron dextran.
• Before parenteral administration of a full dose,
  test the pt. for allergy
• Antidote for anaphylaxis should be ready (eg,
  epinephrine). If no signs of allergic reaction
  have occurred after 30 minutes, the remaining
  dose of iron may be administered.

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Nursing Management

• Preventive education is important
• Food sources high in iron include organ meats
  (beef or calfs liver, chicken liver), other
  meats
• beans , leafy green vegetables, raisins ??????,
  and molasses ?????.
• Taking iron-rich foods with a source of vitamin C
  enhances the absorption of iron.
• Antacids or dairy products should not be taken
  with iron, because they greatly diminish the
  absorption of iron.

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PATIENT EDUCATION

• Take iron on an empty stomach (1 hour before or 2
  hours after a meal). Iron absorption is reduced
  with food, especially dairy products.
• To prevent gastrointestinal distress, the
  following schedule may work better if more than
  one tablet a day is prescribed
• Start with only one tablet per day for a few
  days, then increase to two tablets per day, then
  three tablets per day. This method permits the
  body to adjust gradually to the iron.
• Increase the intake of vitamin C (citrus fruits
  and juices, strawberries,
• tomatoes, broccoli), to enhance iron absorption.
• Eat foods high in fiber to minimize problems
  with constipation.
• Remember that stools will become dark in color.

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MEGALOBLASTIC ANEMIAS
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• In the anemias caused by deficiencies of vitamin
  B12 or folic acid, identical bone marrow and
  peripheral blood changes occur, because both
  vitamins are essential for normal DNA synthesis.
• In either anemia, the RBCs that are produced are
  abnormally large and are called megaloblastic RBCs

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Path physiologyFOLIC ACID DEFICIENCY

• Folic acid is stored as compounds referred to as
  folates.
• The folate stores in the body are much smaller
  than those of vitamin B12, and they are quickly
  depleted when the dietary intake of folate is
  deficient (within 4 months).
• Folate is found in green vegetables and liver.

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Risk factors

• In people who rarely eat uncooked vegetables.
• Alcoholism.
• In patients with chronic hemolytic anemias
• Pregnant women.
• Patients with malabsorptive diseases of the small
  bowel

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VITAMIN B12 DEFICIENCY

• A deficiency of vitamin B12 can occur in several
  ways.
• Inadequate dietary intake e.g. in strict
  vegetarians
• Faulty absorption from the gastrointestinal
  tract is more common such as Crohns disease, or
  after ileal resection or gastrectomy.
• Absence of intrinsic factor, as in pernicious
  anemia. Without intrinsic factor, orally consumed
  vitamin B12 cannot be absorbed.
• Pernicious anemia, which tends to run in
  families, is primarily a disorder of adults,
  particularly the elderly. The abnormality is in
  the gastric mucosa the stomach wall atrophies
  and fails to secrete intrinsic factor. Therefore,
  the absorption of vitamin B12 is significantly
  impaired.

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Clinical Manifestations

• Patients with pernicious anemia develop a
  smooth, sore, red tongue and mild diarrhea. pale,
  particularly in the mucous membranes.
• Confusion
• Paresthesias in the extremities.
• Difficulty in maintaining balance
• These symptoms are progressive, although the
  course of
• illness may be marked by spontaneous partial
  remissions and exacerbations.
• Without treatment, patients can die after several
  years,
• usually from heart failure secondary to anemia.

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Assessment and Diagnostic Findings

• Schilling test
• Patient receives a small oral dose of radioactive
  vitamin B12, followed in a few hours by a large,
  nonradioactive parenteral dose of vitamin B12.
• If the oral vitamin is absorbed, more than 8
  will be excreted in the urine within 24 hours
  therefore, if no radioactivity is present in the
  urine, the cause is gastrointestinal
  malabsorption of the vitamin B12.
• Conversely, if the urine is radioactive, the
  cause of the deficiency is not ileal disease or
  pernicious anemia.

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• Later, the same procedure is repeated, but this
  time intrinsic factor is added to the oral
  radioactive vitamin B12.
• If radioactivity is now detected in the urine
  (ie, the B12 was absorbed from the
  gastrointestinal tract in the presence of
  intrinsic factor), the diagnosis of pernicious
  anemia can be made.
• The Schilling test is useful only if the urine
  collections are complete.

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• Another useful, easier test is the intrinsic
  factor antibody test. A positive test indicates
  the presence of antibodies that bind the vitamin
  B12intrinsic factor complex and prevent it from
  binding to receptors in the ileum, thus
  preventing its absorption.

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Medical Management

• In folic acid deficiency
• Increase the amount of folic acid in the diet and
  administering 1 mg of folic acid daily.
• Most proprietary vitamin preparations do not
  contain folic acid, so it must be administered as
  a separate tablet.

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Medical Management

• In Vitamin B12 deficiency
• Vitamin B12 replacement.
• Vegetarians can prevent or treat deficiency with
  oral supplements
• through vitamins or fortified soy milk.
• When the deficiency is due to defective
  absorption or absence of intrinsic factor,
  replacement is by monthly intramuscular
  injections of vitamin B12, usually at a dose of
  1000 µg.

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Hemolytic Anemias

• SICKLE CELL ANEMIA

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Path physiology

• Sickle cell anemia is a severe hemolytic anemia
  that results from inheritance of the sickle
  hemoglobin gene.
• The sickle hemoglobin (HbS) acquires a
  crystal-like formation when exposed to low oxygen
  tension. consequently, the RBC containing (HbS)
  loses its round, very pliable, biconcave disk
  shape and becomes deformed, rigid, and
  sickle-shaped
• These long, rigid RBCs can adhere to the
  endothelium of small vessels when they pile up
  against each other, blood flow to a region or an
  organ may be reduced.

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Path physiology

• If ischemia or infarction results, the patient
  may have pain,
• swelling, and fever.
• The sickling process takes time if the RBC is
  again exposed to adequate amounts of oxygen (eg,
  when it travels through the pulmonary
  circulation) before the membrane becomes too
  rigid, it can revert to a normal shape. For this
  reason, the sickling crises are intermittent.

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Factors that aggravate sickling process

1. Cold, because vasoconstriction slows the blood
   flow.
2. In an increased blood viscosity, with or without
   occlusion due to adhesion of sickled cells.

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Clinical Manifestations

• Symptoms and complications result from chronic
  hemolysis or thrombosis
• Patients are always anemic, usually with
  hemoglobin values of 7 to 10 g/dL.
• Jaundice is characteristic and is usually obvious
  in the sclerae.
• Enlargement of the bones of the face and skull
  due to expansion of the bone marrow to compensate
  anemia .
• Tachycardia, cardiac murmurs, and an enlargement
  of the heart (cardiomegaly) associated with
  chronic anemia .
• Dysrhythmias and heart failure may occur in
  adults.

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Assessment and Diagnostic Findings

• The patient with sickle cell anemia has a low
  hematocrit and sickled cells on the smear.
• The diagnosis is confirmed by hemoglobin
  electrophoresis.

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Prognosis

• Diagnoses is in the childhood.
• Some children die in the first years of life,
  typically from infection.
• The average life expectancy is at 42 years.
• In some patients, the symptoms and complications
  diminish by 30 years of age these patients live
  into the sixth decade or longer.

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Medical Management

• Currently there are only three primary treatment
  modalities for sickle cell diseases BMT,
  hydroxyurea, and RBC transfusion.
• BMT offers the potential for cure for this
  disease.
• Problem with either the lack of a compatible
  donor or the severe organ (eg, renal, liver,
  lung) damage already present in the patient.

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Hydroxyurea (Hydrea),

• a chemotherapy agent, has been shown to be
  effective in increasing hemoglobin F levels in
  patients with sickle cell anemia, thereby
  decreasing the permanent formation of sickled
  cells.
• Patients who receive hydroxyurea appear to have
  fewer painful episodes of sickle cell crisis, a
  lower incidence of acute chest syndrome, and less
  need for transfusions
• Side effects of hydroxyurea include
• chronic suppression of WBC formation
• Teratogenesis
• Potential for later development of a malignancy.

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TRANSFUSION THERAPY

• Its highly effective in several situations
• in an acute exacerbation of anemia (eg, aplastic
  crisis)
• in the prevention of severe complications from
  anesthesia and surgery
• in improving the response to infection (when it
  results in exacerbated anemia)
• sickle cell crisis in pregnant women
• Transfusion therapy may be effective in
  preventing complications from sickle cell
  disease.
• Patients with cerebral ischemic injury to
  prevent more severe injury (eg, CVA).
• severe cases of acute chest syndrome

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SUPPORTIVE THERAPY

• Pain management. Acute pain episodes tend to be
  self-limited, lasting hours to days e.g. Aspirin,
  NSAIDs, opioid analgesics, .
• Adequate hydration is important during a painful
  sickling episode.
• Oral hydration or intravenous hydration with
• dextrose 5 in water (D5W) or dextrose 5 in 0.25
  normal saline solution (3 L/m2/24 hours) is
  usually required for sickle crisis.
• Oxygen may also be needed.

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SICKLE CELL CRISIS

• Sickle crisis the very painful situations,
  which result from tissue hypoxia and necrosis due
  to inadequate blood flow to a specific region of
  tissue or organ.
• Aplastic crisis results from infection with the
  human parvovirus. The hemoglobin level falls
  rapidly and the marrow cannot compensate, as
  evidenced by an absence of reticulocytes.
• Sequestration crisis results when other organs
  pool the sickled cells.
• Although the spleen is the most common organ
  responsible for sequestration in children, by 10
  years of age most children with sickle cell
  anemia have had a splenic infarction and the
  spleen is then no longer functional
  (autosplenectomy).
• In adults, the common organs involved in
  sequestration are the liver and, more seriously,
  the lungs.

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THALASSEMIA
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• The thalassemias are a group of hereditary
  disorders associated with defective
  hemoglobin-chain synthesis.
• Thalassemias are characterized by hypochromia (an
  abnormal decrease in the hemoglobin content of
  RBCs), extreme
• microcytosis (smaller-than-normal RBCs)
• destruction of blood elements (hemolysis)
• variable degrees of anemia.

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Classifications

• The alphathalassemias occur mainly in people
  from Asia and the Middle East, It is milder than
  the beta forms and often occur without symptoms.
  The RBCs are extremely microcytic, but the
  anemia, if present, is mild.
• The beta-thalassemias are most prevalent in
  Mediterranean populations but also occur in
  people from the Middle East or Asia.
• The severity of beta-thalassemia varies depending
  on the extent to which the hemoglobin chains are
  affected.

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Prognosis

• Patients with mild forms have a microcytosis and
  mild anemia.
• If left untreated, severe beta-thalassemia can be
  fatal within the first few years of life.
• If it is treated with regular transfusion of
  RBCs, patients may survive into their 20s and
  30s.
• Patient teaching during the reproductive years
• should include pre-conception counseling about
  the risk of congenital thalassemia major.

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Thalassemia Major

• Thalassemia major (Cooleys anemia) is
  characterized by
• severe anemia
• marked hemolysis
• ineffective erythropoiesis (production of RBCs).
• With early regular transfusion therapy, growth
  and development through childhood are
  facilitated. Organ dysfunction due to iron
  overload results from the excessive amounts of
  iron obtained
• through the RBC transfusions.
• Regular chelation therapy (eg, via subcutaneous
  deferoxamine) has reduced the complications of
  iron overload and prolonged the life of these
  patients. This disease is potentially curable by
  BMT if the procedure can be performed before
  damage to the liver occurs (ie, during childhood).

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The Polycythemias
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• Polycythemia refers to an increased volume of
  RBCs when the hematocrit is elevated (to more
  than 55 in males, more than 50 in females.
• Dehydration can cause an elevated hematocrit, but
  not typically to the level to be considered
  polycythemia.

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POLYCYTHEMIA VERA

• Polycythemia vera, or primary polycythemia, is a
  proliferative disorder in which the myeloid stem
  cells seem to have escaped normal control
  mechanisms. The bone marrow is hypercellular, and
  the RBC, WBC, and platelet counts in the
  peripheral blood are elevated.
• However, the RBC elevation is predominant the
  hematocrit can exceed 60. This phase can last
  for an extended period (10 years or longer). The
  spleen resumes its embryonic function of
  hematopoiesis and enlarges. Over time, the bone
  marrow may become
• fibrotic, with a resultant inability to produce
  as many cells

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Clinical Manifestations

• splenomegaly
• The symptoms result from the increased blood
• volume (headache, dizziness, tinnitus, fatigue,
  paresthesias, and
• blurred vision) or from increased blood viscosity
  (angina, claudication, dyspnea, and
  thrombophlebitis.
• Generalized pruritus, which may be caused by
  histamine release due to the increased number of
  basophils.
• Erythromelalgia, a burning sensation in the
  fingers and toes, may be reported and is only
  partially relieved by cooling.

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• Assessment and Diagnostic Findings
• Diagnosis is made by finding an elevated RBC mass
  (a nuclear
• medicine procedure), a normal oxygen saturation
  level, and an
• enlarged spleen. Other factors useful in
  establishing the diagnosis
• include elevated WBC and platelet counts. The
  erythropoietin
• level is not as low as would be expected with an
  elevated hematocrit
• it is normal or only slightly low. Causes of
  secondary erythrocytosis
• should not be present (see later discussion).

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• Complications
• Patients with polycythemia vera are at increased
  risk for thromboses
• resulting in a CVA (brain attack, stroke) or
  heart attack
• (MI) thrombotic complications are the most
  frequent cause of
• death. Bleeding is also a complication, possibly
  due to the fact
• that the platelets (often very large) are
  somewhat dysfunctional.
• The bleeding can be significant and can occur in
  the form of
• nosebleeds, ulcers, and frank gastrointestinal
  bleeding.

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• Medical Management
• The objective of management is to reduce the high
  blood cell
• mass. Phlebotomy is an important part of therapy
  and can be performed
• repeatedly to keep the hematocrit within normal
  range.
• This is achieved by removing enough blood
  (initially 500 mL
• once or twice weekly) to deplete the patients
  iron stores, thereby
• rendering the patient iron deficient and
  consequently unable to
• continue to manufacture RBCs excessively.
  Patients need to be
• instructed to avoid iron supplements, including
  those within
• multivitamin supplements. If the patient has an
  elevated uric acid
• concentration, allopurinol (Zyloprim) is used to
  prevent gouty
• attacks. Antihistamines are not particularly
  effective in controlling
• itching.

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• If the patient develops ischemic symptoms,
  dipyridamole
• (eg, Persantine) is sometimes used. Radioactive
• phosphorus (32P) or chemotherapeutic agents (eg,
  hydroxyurea
• Hydrea) can be used to suppress marrow
  function, but they may
• increase the risk for leukemia. Patients
  receiving hydroxyurea appear
• to have a lower incidence of thrombotic
  complications this
• may result from a more controlled platelet count.
  The use of aspirin
• to prevent thrombotic complications is
  controversial. Lowdose
• aspirin is frequently used in patients with
  cardiovascular
• disease, but even this dose is often avoided in
  patients with prior
• bleeding, especially bleeding from the
  gastrointestinal tract. Aspirin

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• is also useful in diminishing pain associated
  with erythromelalgia.
• Anagrelide (Agrylin) inhibits platelet
  aggregation and can also be
• useful in controlling the thrombocytosis
  associated with polycythemia
• vera. Interferon alfa-2b (Intron-A) has also been
  studied,
• but it may be difficult for patients to tolerate
  due to the
• frequent side effects experienced

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• Nursing Management
• The nurses role is primarily that of educator.
  Risk factors for
• thrombotic complications should be assessed, and
  patients should
• be instructed regarding the signs and symptoms of
  thrombosis. Patients
• with a history of bleeding are usually advised to
  avoid aspirin
• and aspirin-containing medications, because these
  medications
• alter platelet function. Minimizing alcohol
  intake should also be
• emphasized to further diminish any risk for
  bleeding. For pruritus,
• the nurse may recommend bathing in tepid or cool
  water, along
• with applications of cocoa butterbased lotions
  and bath products.

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• SECONDARY POLYCYTHEMIA
• Secondary polycythemia is caused by excessive
  production of erythropoietin.
• This may occur in response to a reduced amount of
• oxygen, which acts as a hypoxic stimulus, as in
  cigarette smoking,
• chronic obstructive pulmonary disease, or
  cyanotic heart disease,
• or in nonpathologic conditions such as high
  altitude. It can also
• result from certain hemoglobinopathies in which
  the hemoglobin
• has an abnormally high affinity for oxygen (eg,
  hemoglobin
• Chesapeake). Secondary polycythemia can also
  occur from neoplasms
• (eg, renal cell carcinoma) that stimulate
  erythropoietin
• production.

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• Medical Management
• Management of secondary polycythemia may not be
  necessary
• when it is, it involves treating the primary
  problem. If the cause
• cannot be corrected (eg, by treating the renal
  cell carcinoma or
• improving pulmonary function), therapeutic
  phlebotomy may be
• necessary in symptomatic patients to reduce blood
  viscosity and
• volume.

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