Chapter 37: Disorders of Hematopoietic System

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Chapter 37 Disorders of Hematopoietic System

• Medical Surgical 1
• Fall Semester

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Blood Dyscrasias pg 564

• Abnormalities in the numbers and types of blood
  cells and bleeding disorders develop from both
  treatable and chronic pathologic processes.
• Some are life threatening
• Many have similar symptoms and require diagnostic
  test

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Anemia

• Decrease in number of erythrocytes (Red blood
  cells) and a lower than normal Hgb level.
• Causes a decrease in amount of oxygen carried to
  tissues
• Caused by blood loss, inadequate nutrition, and
  genetic or acquired disorders

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Hypovolemic Anemia

• Loss of blood volume results in a decrease of
  blood cells.
• Because erythrocytes are the most abundant type
  of blood cell, the most critical consequence is
  hypovolemic anemia.

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Pathophysiology pg 564

• Caused by sudden loss of a large amount of blood
  or a gradual loss of small amounts over a
  prolonged period

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Signs Symptoms Acute

• S/S of acute hypovolemic anemia from sudden
  severe blood loss--shock extreme pallor,
  tachycardia, hypotension, reduction of urinary
  output and altered consciousness

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Signs Symptoms Chronic

• Symptoms from chronic blood loss pallor,
  fatigue, feeling chilled, postural hypotension,
  and rapid heart and respiratory rate
• CBC, Hgb and Hct levels used to diagnose

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Hemoglobin Levels

• Normal results vary, but in general are
• Male 13.8 to 17.2 gm/dL
• Female 12.1 to 15.1 gm/dL

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Medical Management

• Treatment of sudden blood loss --transfusions
• Treatment of chronic (uterine tumors,
  hemorrhoids) underlying condition is treated
• May need transfusion or IV or IM iron to help
  compensate loss of hgb.
• Oxygen helps if severe

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Nursing Process pg. 565 (Hypovolemia)

• Monitor I O--report urine below 30 to 50
  ml/hour
• Assess vitals q 2 to 4 hours. Report systolic BP
  less than 90 or heart rate above 100
• Monitor oxygen saturation ..report levels less
  than 90
• Apply direct pressure to stop bleeding
• Use modified trendelenburg

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Trendelenburg Position

• Facilitates
• Blood
• Flow
• To
• Brain

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Nursing Care (Hypoxemia)

• Monitor oxygen saturation continually with a
  pulse oximeter. (it measures the percentage of
  oxygen bound to hemoglobin)
• Report a sustained oxygen saturation value below
  90. (normal is 95 100)
• Supplement oxygen as ordered by physician.

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Nursing Care (Activity Intolerance Body Temp)

• Space activity..let him rest
• Provide extra blanket, if chilled warm blanket
• Close door, raise temp of room

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Iron Deficiency Anemia pg 567

• Develops when there is an insufficient amount of
  iron to produce hemoglobin.
• Occurs when
• Heme cannot be recycled d/t blood loss
• Dietary intake of iron is insufficient
• Absorption of iron from food is inadequate
• The need for iron exceeds the reserves

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Pathophysiology

• Even when a healthy diet is consumed, less than
  10 of the iron is absorbed.

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Pathophysiology

• Iron is needed to produce Hgb.
• Need for iron increases if rapid growth,
  pregnancy, reproductive years and during menses.

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Signs Symptoms

• Causes reduced energy, feel cold all the time,
  and experience fatigue and dyspnea with minor
  physical exertion. Heart rate is fast even at
  rest

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Nursing Management 567

• Improve the nutritional intake of iron.
• Use Z tract if iron given IM. Insert the needle
  at a 90 degree angle. Give test dose
• Review clinical procedure 37-1 pg. 568 Z-Track
  Technique on own.

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Stop Think Respond

• Why does the nurse use the Z-track method to
  administer parenteral iron???

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• Z-track method ensures that the medication does
  not seep into SQ tissue.
• Iron can irritate and stain tissue

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Teaching

• Dilute liquid preparations of iron with another
  liquid such as juice drink with a straw to
  avoid staining the teeth.
• Take with food or immediately after a meal to
  avoid gastric distress
• Take with vitamin C to promote its absorption
• Expect that iron colors stool dark green or black

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Sickle Cell Anemia

• Erythrocytes become sickle or crescent shaped
  when there is low oxygen in blood
• Caused by abnormal form of Hgb...Hgb S
• Hereditary, found primarily in African Americans
  but also occurs in people from Mediterranean and
  Middle Eastern countries
• Must inherit gene from each parent to have the
  disease but can have the trait if only one parent
  has the gene..

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Anemia Sickle Cell Anemia
Figure 37-2 A normal spherical red blood cell and
sickled cell
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Sickle Cell Anemia

• Sickle cell disease--100 Hgb affected
• Sickle cell trait--40 Hgb affected, so less
  chance of developing symptoms
• Abnormality in Hgb shortens life span of
  erythrocytes and they become hemolyzed
  (destroyed)
• Spleen becomes obstructed by excess volume of
  destroyed RBCs so risk of infection high

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Sickle Cell

• Bone marrow enlarges to compensate for the
  continuous need to produce more RBCs
• Persistent anemia causes tachycardia, dyspnea,
  cardiomegaly and arrhythmias
• Vascular occlusion causes severe pain
• Sickle cells can lodge in small blood vessels and
  block blood flow
• Stroke is common even in small children

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Signs and Symptoms

• Severe joint pain is a frequent symptom seen
• Will have jaundice
• Chronic leg ulcers as blockage to legs
• Priapism (prolonged erection as delayed emptying
  of thick blood
• Other signs of other types of anemia also present

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Sickle Cell Crisis

• Blockage of small vessels reduces blood flow and
  causes localized ischemia and severe pain
• Fever, pain, and swelling of one or more joints
  are common
• Can lead to CVA, pulmonary infarction, shock and
  renal failure

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Sickle Cell Anemia

• To diagnose sickle cell a sickle cell screening
  is done. Sickle cell anemia is present when the
  sickle cell screening test is positive for
  hemoglobin S.

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Medical Management pg 569

• Treatment is supportive as cant be cured
• Blood transfusion may be given (however,
  transfused blood increases blood viscosity
  (thickness), which potentially does more harm
  than good.
• Every infection, no matter how minor, treated
  with antibiotics

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Management

• A few people have been cured of sickle cell
  disease, using bone marrow transplantation.
• The possibility of curing sickle cell disease
  with gene replacement therapy is undergoing
  research, but practical application of the
  technique is remote at this time.

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Medical management

• Narcotics given for pain, oxygen given
• Complete bedrest, hydrated with IV fluids
• Iron chelating agent (Desferal) given to remove
  excess iron from transfusions and erythrocyte
  destruction

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Stop think, and Respond

• What is the correlation between sickle cell
  anemia and stroke (cerebrovascular accident)?????

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• Sickling causes the abnormally shaped RBCs to
  occlude small blood vessels. Blockage of small
  blood vessels in the brain results in cerebral
  injury from a loss of oxygen to the tissue and
  ultimately cellular death.

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Teaching Box 37-1 pg 571

• Drink extra fluids
• Dress warmly in cold temperatures
• Avoid vigorous physical exercise
• Avoid leg positions or clothing that cause
  vasoconstriction
• Stop smoking or other use of nicotine
• Avoid high altitudes, get flu and pneumonia
  immunizations and see Dr stat if infection

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Hemolytic Anemia pg 571

• Chronic premature destruction of erythrocytes
  (RBCs)
• Causes --cardiopulmonary bypass during surgery,
  arsenic or lead poisoning, malaria, infectious
  agents or toxins and exposure to hazardous
  chemicals and blood transfusion reactions

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Signs Symptoms Treatment

• Similar to other anemias. In severe form, they
  may have jaundice and spleen enlargement
• Steriods given, blood transfusions and if doesnt
  respond, spleenectomy done

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Thalassemia Cooleys Anemia pg 571

• Group of genetic inherited syndromes of abnormal
  Hgb synthesis
• Results in impaired synthesis of either alpha or
  beta chain of adult Hgb.

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Thalassemias Pg 571

• Hereditary hemolytic anemias
• Cooleys anemia, a severe form of
  beta-thalassemia, has symptoms of severe anemia
  and a bronzing of the skin caused by hemolysis of
  RBCs
• Iron chelation required because of iron deposits
  in skin
• Must be on bedrest and protected from infection

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Thalassemias
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Pernicious Anemia Pg 571

• Develops when there is a lack of intrinsic
  factor in gastric secretions causes it. The
  intrinsic factor is necessary for absorption of
  vitamin B12
• B12 and the intrinsic factor is necessary for the
  maturation of erythrocytes

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Pathophysology

• Aging and gastric mucosa atrophy reduces
  intrinsic factor.
• Also secondary to the surgical removal of the
  stomach or small bowel resection in which the
  ileum, the site for vitamin B12, erythrocytes
  remain in an immature form.

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Pernicious Anemia

• If not recognized, degeneration of nervous system
  develops and can be permanent if not treated stat.

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Signs Symptoms pg 572

• Stomatitis, glossitis (red, beefy looking
  tongue), digestive disturbances and diarrhea.
• If severe, dyspnea, jaundice, irritability,
  confusion and depression occur

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glossitis (red, beefy looking tongue)
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Signs Symptoms

• Numbness and tingling in arms and legs and ataxia
  (incoordination) are common signs of neurological
  damage. Vibratory and position sense are
  sometimes lost.

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Management pg 572

• B12 is given IM for life

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Folic Acid Anemia Pg 572

• Characterized by immature erythrocytes. Commonly
  caused by insufficient dietary intake of folic
  acid (Vit B9)
• Elderly, alcoholics, ones with intestinal
  disorder that affect food absorption, malignancy
  and chronically ill may have folic acid
  deficiency because of poor nutrition
• Pregnancy and hemolytic anemia can cause

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Signs Symptoms

• Severe fatigue, sore tongue that is beefy red,
  dyspnea, nausea, anorexia, headache, weakness and
  light-headedness occur
• Will have low Hgb and Hct and serum folate is
  decreased
• A schilling test differentiates pernicious anemia
  and one caused by folic acid
• Only one that has neuro symptoms is pernicious
  anemia

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FYI Schilling Test (Not in book)

• Schilling test Give B12 IM to load up patient
  and then give radioactive B12 P.O. to see if it
  is discarded in urine. It should be discarded as
  body has all the B12 it needs. If not discarded
  then it is positive for pernicious anemia

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Medical Management

• Parenteral folic acid given if absorption
  disorder otherwise a high folic acid diet given
• As in any other type of anemia, should space
  activity and plan frequent rest periods
• Vitamin C enhances absorption of folic acid and
  iron

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Erythrocytosis pg 573

• An increase in circulating erythrocytes. One of
  these conditions is called polycythemia vera
• Polycythemia vera has a greater than normal RBC,
  WBC and platelets (also called thrombocytes)
• If living at higher altitudes it is normal to
  have erythrocytosis

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Polycythemia Vera

• Despite the abundance of erythrocytes, their life
  span is shorter.
• The dead RBC release potassium and uric acid
  (causes gout)
• Oxygen combining capacity is lower and since
  blood is thicker, clots form
• Complications include hypertension, CHF, stroke,
  areas of infarction and hemorrhage

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Signs and Symptoms

• Face and lips are reddish purple. Fatigue,
  weakness, headache, pruritis, exertional dyspnea
  and dizziness are common
• Excessive bleeding after minor injury as engorged
  capillaries and veins.
• Spleenomegaly and swollen and painful joints
  because of uric acid levels.

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Medical Management pg 573

• A phlebotomy (opening a vein to withdraw blood)
  is done several times a week 500 ml is removed
  at a time.
• Anticoagulants are given and radiation can be
  given to slow production of RBC in the bone
  marrow. Cancer drugs (Mustargen) given to curb
  excessive bone marrow activity

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Nursing Management

• Drink 3 quarts of fluid daily
• Avoid crossing legs and wearing tight clothing
  that impairs circulation
• Change position frequently and elevate lower
  extremities
• Do isometric exercises --contracting and relaxing
  quadriceps and gluteal--wiggle toes
• Wear TED hose or support hose, rest stat if chest
  pain

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Leukemia Pg 573

• Malignant blood disorder--too many leukocytes
  (WBC) formed but they are immature. Besides
  making too many WBCs, there is a decrease in RBC
  formation. WBC is too immature to fight infection
• Cause unknown but exposure to toxic chemicals and
  radiation, viruses, and certain drugs precipitate

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Pathophysiology

• Severe anemia eventually occurs, and the
  reduction of platelets leads to bleeding.
• The excessive numbers of leukocytes infiltrate
  the spleen, liver, lymph nodes, and brain if
  unchecked

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Types of Leukemia (Table 37-3 pg 574)

• Review on own

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Signs Symptoms

• Infections, fatigue from anemia, and easy
  bruising are hallmarks of leukemia.
• Fever is present, spleen and lymph nodes enlarge
  and internal and external bleeding occurs.
  Nosebleeds, mouth and GI tract are common sites
  of bleeding

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Medical Management

• Drug therapy is the primary weapon for arresting
  leukemia
• The type of drug or combination of drugs depends
  on the form of leukemia.
• Erythrocyte platelet transfusions are necessary
  to treat the anemia and decrease in platelets.

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Treatment

• Antibiotics to prevent infection, and bone marrow
  transplant and stem cell transplantation have all
  increased the survival rate. Can be from oneself
  (autologous) or from another (allogenic)

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Nursing management

• Drugs used to fight leukemia are highly toxic and
  can impair formation of all blood cells
• Antineoplastic drugs cause alopecia, nausea,
  vomiting, diarrhea, excessive bleeding, anorexia,
  stomatitis and oral ulcerations
• Give small frequent feedings of bland foods and
  frequent sips of cool water to maintain fluid and
  nutrition

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Nursing Management

• Initial assessment includes a history of current
  symptoms and past symptoms
• Joint pain and other symptoms associated with
  leukocyte infiltration of the central nervous
  system ( headache, confusion) can occur.
• Observe for symptoms of kidney, ureteral, or
  bladder stones as high uric acid form
  chemo--increase fluids

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Nursing for Leukemia (Care plan pg 575)

• Assess for signs of infection (swelling and
  tenderness)
• Protective isolation
• Neutropenic precautions--box 37-2 page 576--no
  raw fruits or vegetables or flowers and etc.
• Stay away from people who are ill, including
  nurses

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Nursing for Leukemia

• Monitor platelet count--inspect skin for bruises
  and petechiae
• Handle gently to avoid bruises
• Report melena (black tarry stools), hematuria,
  epistaxis
• Apply prolonged pressure after injections or IV
  sites
• Spongy toothettes for oral hygiene, use electric
  razor

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Teaching for Leukemia Box 37-2 pg 577

• See Dr stat is excessive bleeding or bruising or
  symptoms of illness or infection
• If sores in mouth so not self treat see Dr. stat.
  Call Dr. stat if severe nausea with prolonged
  vomiting, severe diarrhea, fever and chills,
  excessive bruises or bleeding, cough, chest pain,
  cloudy urine, rash, blood in stool or urine,
  severe headache, extreme fatigue, increased resp
  rate or dyspnea

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Multiple Myeloma pg 576

• Malignancy involving plasma cells,
  lymphocyte-like cells in the bone marrow.
  Prognosis is poor, with estimated survival of 1
  to 5 years
• Associated with aging and rarely occurs before
  40. Immature plasma cells proliferate (multiply
  rapidly) in the bone marrow forming single or
  multiple osteolytic (bone destroying) tumors

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Multiple Myeloma

• Malignant plasma cells release an abnormal
  protein called M-type globulin. Excess production
  of plasma cells reduces production of
  erythrocytes, leukocytes and platelets. Later the
  liver, spleen, soft tissues and kidneys are
  affected

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Multiple Myeloma

• First symptom is usually vague pain in pelvis,
  spine or ribs. As it progresses the pain is more
  severe and localized
• When bone marrow is replaced by tumors,
  pathologic fractures occur.
• Resistance to infection is decreased as
  lymphocytes make antibodies
• Bone marrow causes anemia and renal calculi and
  renal failure occur

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Multiple Myeloma

• Skeletal X-ray shows punched out bone lesions in
  bone marrow. Serum calcium levels are elevated
  due to bone destruction and uric acid levels are
  high

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Nursing for Multiple Myeloma

• Assess for signs of infection, excessive fatigue
  and pain in new areas
• Assist with ambulation because immobility can
  worsen loss of calcium from bone
• Push fluids up to 4000 cc daily to prevent renal
  damage
• Safety 1st as any injury, no matter how slight
  can cause fractures. Give analgesics before care
  delay bath until med helps

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Agranulocytosis pg 579

• Reduction in production of granulocytes
  (neutrophils, basophils and eosinophils. Causes
  higher risk for infection. Number one defense
  against bacterial infection are granulocytes.
  Most common cause is toxicity from drugs

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Signs Symptoms

• Fatigue, fever, chills, headache, and
  opportunistic infections in mouth, throat, nose,
  rectum or vagina
• If cause found and treatment started early
  usually recover

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Nursing management

• Determine names of all drugs taken in last 6
  months to a year. Protective isolation
• Protective isolation is necessary if the
  leukocyte count is extremely low.
• Visitors or staff with any type of an infection
  are restricted from close client contact until
  the infection has cleared.

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Pancytopenia pg 579

• Refers to conditions such as aplastic anemia in
  which numbers of all marrow-produced blood cells
  are reduced.
• Aplastic anemia is more than just a deficiency of
  erythrocytes---failure to develop

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Aplastic Anemia pg 579

• Consequence of inadequate stem production in bone
  marrow so insufficient numbers of erythrocytes,
  leukocytes, and platelets, collectively described
  as pancytopenia.
• Exposure to toxic chemicals, radiation and drug
  therapy with anticancer drugs and some
  antibiotics.
• Death rate is high

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Signs Symptoms

• Typical symptoms of anemia, frequent
  opportunistic infections, plus coagulation
  abnormalities, unusual bleeding, small skin
  hemorrhages (petechiae and ecchymosis. spleen is
  enlarged
• Bone marrow confirms diagnosis
• In some cases the bone marrow will regenerate
  and have normal function if cause found and
  corrected

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Medical Management

• Transfusions given
• Antibiotics given to prevent infection
• Steroids given in cases of an autoimmune
  connection
• Bone marrow transplantation if donor found
• Stem cell transplantation is an alternative

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Nursing Management

• Assess s/s severe anemia, infection, and bleeding
• Soft foods and oral hygiene techniques to avoid
  bleeding gums. Avoid chewing as much as possible
• Give good oral hygiene
• Protective isolation, restrict visitors if WBCs
  very low
• Apply pressure if injections must be given

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Coagulopathies pg. 580

• The term coagulopathy refers to conditions in
  which a component that is necessary to control
  bleeding is missing or inadequate.
• Two common examples are thrombocytopenia and
  hemophilia.

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Thrombocytopenia pg 580

• Lower than normal number of platelets or
  thrombocytes
• Accompanies leukemia and other malignant blood
  disease and caused by severe infections, and
  certain drugs
• Idiopathic thrombocytopenia purpura is
  thrombocytopenia without a known cause

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Thrombocytopenia

• Purpura, small hemorrhages in the skin, mucous
  membranes, or subQ tissue.
• Bleeding from other parts of body also occur
  (nose, oral mucous membranes, GI. Internal
  hemorrhage can be fatal
• Spleenectomy may be needed to raise platelet
  count and relieve symptoms

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Hemophilia pg 581

• Disorder involving clotting factors. Three types
  but most common types A, B, C. Type A is most
  common results from a deficiency of factor IIIV
• Genetic-Inherited from mom to son as a sex-linked
  recessive characteristic. Daughters inherit the
  trait and can pass on disease to their sons but
  seldom develop the disease. Bleeding noted in
  infancy and childhood. Milder may go
  unrecognized for years

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Hemophelia

• Persistent oozing and sometimes severe bleeding
  that occurs spontaneously after injury. Bleeding
  in joints will damage the joints and lead to
  deformity and limitation of movement.
• Deficiency of factor 8 or 9 (Christmas disease)
  Treatment is transfusions , direct pressure, cold
  compresses and measures to control bleeding

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Nursing management

• Assess joints and mobility
• Inspect skin for purpura or hemorrhagic areas
• Ask if BP cuff causes bleeding
• Temp taken using tympanic to avoid oral or rectal
  injury
• Prevent trauma, reduce pain, conserve energy

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CONSIDERATIONS

• Review Nutritional, Pharmacologic,
• Gerontologic Considerations !!!!!
• There are a lot of similarities so focus on what
  makes it different