Interventions for Clients Interventions for Clients with Hematologic Problemswith Hematologic Problems

Anemia Anemia Reduction in either the number Reduction in either the number

of red blood cells, the amount of of red blood cells, the amount of hemoglobin, or the hematocrithemoglobin, or the hematocrit

Clinical sign (not a specific Clinical sign (not a specific disease); a manifestation of disease); a manifestation of several abnormal conditionsseveral abnormal conditions

ANEMIAANEMIA1.) Anemia1.) Anemia is reduction in either RBCs, is reduction in either RBCs,

amount of hemoglobin, or hematocrit (% of amount of hemoglobin, or hematocrit (% of packed RBC per deciliter of blood)packed RBC per deciliter of blood)

““Anemia” is a symptom of an underlying Anemia” is a symptom of an underlying disease.disease.

Causes and types vary:Causes and types vary: 1.)dietary problems—deficiency in 1.)dietary problems—deficiency in

components necessary to make RBC—iron, components necessary to make RBC—iron, vitamin B12 (cyanocobalamin), folic acid, or vitamin B12 (cyanocobalamin), folic acid, or intrinsic factorintrinsic factor

2.)genetic disorders2.)genetic disorders 3.)bone marrow disease3.)bone marrow disease 4.)excessive bleeding4.)excessive bleeding 5.)Immune reactions5.)Immune reactions 6.)Changes in blood chemistry6.)Changes in blood chemistry 7.)Toxins in the blood7.)Toxins in the blood

Chronic Chronic anemiasanemias  develop gradually, develop gradually, more subtle symptoms-- lethargy, pallor, more subtle symptoms-- lethargy, pallor, and anorexia and anorexia (gastritis, hemorrhoids, menstrual (gastritis, hemorrhoids, menstrual flow) flow)

Acute anemiasAcute anemias do not allow the body do not allow the body sufficient time to make physiologic sufficient time to make physiologic adjustments -- patients symptomatic adjustments -- patients symptomatic with shortness of breath, extreme fatigue, with shortness of breath, extreme fatigue, and cardiac discomfort and cardiac discomfort (trauma, blood vessel (trauma, blood vessel rupture)rupture)

Hematologic ProblemsHematologic Problems AnemiasAnemias Results in:Results in: reduction in oxygen reduction in oxygen

transport due to decrease in transport due to decrease in hemoglobin production, a decrease in hemoglobin production, a decrease in erythrocytes, or a combination of erythrocytes, or a combination of these factors.these factors.

Reduced oxygen leads to less energy Reduced oxygen leads to less energy in all cells, reduced cell metabolism in all cells, reduced cell metabolism and reproduction.and reproduction.

Compensation mechanisms include tachycardia Compensation mechanisms include tachycardia and peripheral vasoconstrictionand peripheral vasoconstriction

Hematologic ProblemsHematologic Problems AnemiasAnemias

General signs of anemia: General signs of anemia: fatigue, pallor, fatigue, pallor, dyspnea, and tachycardiadyspnea, and tachycardia

Severe anemia may lead to angina if Severe anemia may lead to angina if oxygen supply to the heart is insufficientoxygen supply to the heart is insufficient

Chronic severe anemia may cause CHFChronic severe anemia may cause CHF Other affects may include hair and skin Other affects may include hair and skin

changeschanges

Key Features of AnemiaKey Features of AnemiaIntegumentary manifestationsIntegumentary manifestations Pallor, of ears, nail beds, palmar creases, conjunctiva, and Pallor, of ears, nail beds, palmar creases, conjunctiva, and

around moutharound mouth cool to touchcool to touch intolerance of cold temperaturesintolerance of cold temperatures Nails become brittle, overtime become concave and Nails become brittle, overtime become concave and

fingers are club like in appearance.fingers are club like in appearance.Cardiovascular ManifestationsCardiovascular Manifestations Tachycardia, murmurs, gallops when anemia severe Tachycardia, murmurs, gallops when anemia severe

orthostatic hypotensionorthostatic hypotension

Key Features of AnemiaKey Features of AnemiaRespiratory ManifestationsRespiratory Manifestations Dyspnea on exertionDyspnea on exertion Decreased oxygen saturation levelsDecreased oxygen saturation levelsNeurologic ManifestationsNeurologic Manifestations Increased somnolence and fatigueIncreased somnolence and fatigue HeadacheHeadache

LABORATORY PROFILELABORATORY PROFILETest Significance of abnormal findingTest Significance of abnormal finding Red blood cell count Decreased indicate possible Red blood cell count Decreased indicate possible anemia/hemorrhageanemia/hemorrhage Hemoglobin/Hematocrit Increased indicate possible Hemoglobin/Hematocrit Increased indicate possible

chronic chronic hypoxia, or polycythemia verahypoxia, or polycythemia vera Mean cell hemoglobin (MCV) Increased levels indicate Mean cell hemoglobin (MCV) Increased levels indicate

macrocyticmacrocytic cells, cells, possible anemia. Decreased possible anemia. Decreased

levels levels indicate indicate microcyticmicrocytic cells, cells, possible iron deficiency anemiapossible iron deficiency anemia Reticulocyte count helpful in determining bone marrow Reticulocyte count helpful in determining bone marrow

function function (immature RBC) **(immature RBC) **Increased levels Increased levels

indicate indicate chronic blood loss—desireable in chronic blood loss—desireable in

anemic client anemic client or after hemorrhage.or after hemorrhage.

Hemoglobin electrophoresis detects abnormal forms of hemoglobin, such asHemoglobin electrophoresis detects abnormal forms of hemoglobin, such as hemoglobin S in sickle cell disease,hemoglobin S in sickle cell disease,Prothrombin time /INR assesses extrinsic clotting cascade, Prothrombin time /INR assesses extrinsic clotting cascade, reflects how much clotting reflects how much clotting factors II, V, VII, X is functioning. factors II, V, VII, X is functioning. IncreasedIncreased=deficient in clotting factor =deficient in clotting factor cascade. cascade. DecreasedDecreased=vitamin K excess. =vitamin K excess. (monitors Coumadin tx) 25-38 sec(monitors Coumadin tx) 25-38 sec PTT aPartial thromboplastin time assesses the intrinsic clotting cascade, PTT aPartial thromboplastin time assesses the intrinsic clotting cascade, factors VIII, IX, XI, XII. factors VIII, IX, XI, XII. Prolonged w/hemophilia or disseminatedProlonged w/hemophilia or disseminated intravascular coagulation (DIC). intravascular coagulation (DIC). (monitors Heparin)(monitors Heparin) Level maintained 1.5 to 2.5 times their Level maintained 1.5 to 2.5 times their baseline valuesbaseline values

3.) Hypoproliferative anemia3.) Hypoproliferative anemia. . Hypoproliferative anemia Hypoproliferative anemia can be subdivided into three classes based upon the can be subdivided into three classes based upon the size of size of the RBCs.the RBCs. The cells may be larger than normal (macrocytic), The cells may be larger than normal (macrocytic), normal (normocytic), or smaller than normal (microcytic).normal (normocytic), or smaller than normal (microcytic).

Macrocytic anemia.Macrocytic anemia. Macrocytic anemia can be Macrocytic anemia can be due to several causes. The first is a deficiency due to several causes. The first is a deficiency in vitamin B12 or folate, both important in vitamin B12 or folate, both important ingredients in RBC production.ingredients in RBC production.

Microcytic anemia.Microcytic anemia. Microcytic anemia is due to Microcytic anemia is due to abnormalities in the production of the essential abnormalities in the production of the essential RBC protein, hemoglobin. This is often to due to RBC protein, hemoglobin. This is often to due to underlying disease, such as thalassemia, iron underlying disease, such as thalassemia, iron deficiency anemiadeficiency anemia

Normocytic anemiaNormocytic anemia. Normocytic anemia may be . Normocytic anemia may be due to chronic disease including malnutrition or due to chronic disease including malnutrition or mixed anemia (combined macrocytic and mixed anemia (combined macrocytic and microcytic anemia).microcytic anemia).

Sickle Cell DiseaseSickle Cell Disease Genetic disorder resulting in Genetic disorder resulting in

chronic anemia, pain, disability, chronic anemia, pain, disability, organ damage, increased risk for organ damage, increased risk for infection, and early deathinfection, and early death

Formation of abnormal Formation of abnormal hemoglobin chainshemoglobin chains

(Continued)(Continued)

Sickle Cell Disease Sickle Cell Disease (Continued)(Continued)

Conditions causing sickling: Conditions causing sickling: hypoxia, dehydration, infections, hypoxia, dehydration, infections, venous stasis, low environmental venous stasis, low environmental body temperatures, acidosis, body temperatures, acidosis, strenuous exercise, and strenuous exercise, and anesthesia.anesthesia.

Clinical Manifestations Clinical Manifestations Cardiovascular changesCardiovascular changes Skin changesSkin changes Abdominal changesAbdominal changes Musculoskeletal changesMusculoskeletal changes Central nervous system changesCentral nervous system changes

Interventions Interventions Pain is the most common Pain is the most common

problem.problem.• Drug therapy: 48 hours of Drug therapy: 48 hours of

intravenous analgesicsintravenous analgesics• Oral hydrationOral hydration• Complementary and alternative Complementary and alternative

therapiestherapies

Potential for SepsisPotential for Sepsis Interventions include:Interventions include:

• Protection of the client from Protection of the client from infection in sickle cell crisisinfection in sickle cell crisis

• Drug therapyDrug therapy

Potential for Multiple Organ Potential for Multiple Organ DysfunctionDysfunction

Interventions include:Interventions include:• HydrationHydration• Oxygen therapyOxygen therapy• Transfusion therapy Transfusion therapy

Glucose-6-Phosphate Glucose-6-Phosphate Dehydrogenase (G6PD) Dehydrogenase (G6PD)

Deficiency AnemiaDeficiency Anemia Most common type of congenital Most common type of congenital

hemolytic anemiahemolytic anemia HydrationHydration Screening for this deficiency Screening for this deficiency

necessary before donating necessary before donating blood, because cells deficient in blood, because cells deficient in G6PD can be hazardousG6PD can be hazardous

Iron Deficiency AnemiaIron Deficiency Anemia This common type of anemia can This common type of anemia can

result from blood loss, poor result from blood loss, poor intestinal absorption, or intestinal absorption, or inadequate diet.inadequate diet.

Evaluate adult clients for Evaluate adult clients for abnormal bleeding.abnormal bleeding.

Supplemental iron is the Supplemental iron is the treatment.treatment.

Vitamin BVitamin B1212 Deficiency Anemia Deficiency Anemia Anemia is caused by inhibiting Anemia is caused by inhibiting

folic acid transport and reducing folic acid transport and reducing DNA synthesis in precursor cells.DNA synthesis in precursor cells.

Vitamin BVitamin B1212 deficiency is a result deficiency is a result of poor intake of foods of poor intake of foods containing vitamin Bcontaining vitamin B12.12.

(Continued)(Continued)

Vitamin BVitamin B1212 Deficiency Anemia Deficiency Anemia (Continued)(Continued)

Pernicious anemia is anemia Pernicious anemia is anemia caused by failure to absorb caused by failure to absorb vitamin Bvitamin B1212 and lack of intrinsic and lack of intrinsic factor; clients often exhibit factor; clients often exhibit paresthesia.paresthesia.

Folic Acid Deficiency AnemiaFolic Acid Deficiency Anemia Can cause megaloblastic anemiaCan cause megaloblastic anemia Manifestations similar to those Manifestations similar to those

of vitamin Bof vitamin B1212 deficiency, but deficiency, but nervous system functions remain nervous system functions remain normalnormal

(Continued)(Continued)

Folic Acid Deficiency AnemiaFolic Acid Deficiency Anemia

(Continued)(Continued) Caused by:Caused by:

• Poor nutrition and chronic alcohol Poor nutrition and chronic alcohol abuse abuse

• Malabsorption syndromes, such as Malabsorption syndromes, such as Crohn’s diseaseCrohn’s disease

• Drugs, including anticonvulsants Drugs, including anticonvulsants and oral contraceptives, that slow and oral contraceptives, that slow or prevent absorption of folic acidor prevent absorption of folic acid

Polycythemia VeraPolycythemia Vera Disease with a sustained increase in Disease with a sustained increase in

blood hemoglobinblood hemoglobin Massive production of red blood cellsMassive production of red blood cells Excessive leukocyte productionExcessive leukocyte production Excessive production of plateletsExcessive production of platelets PhlebotomyPhlebotomy Increased hydrationIncreased hydration Anticoagulants are part of therapyAnticoagulants are part of therapy

Polycythemia VeraPolycythemia VeraDue to hyperviscous (thicker than normal blood) the Due to hyperviscous (thicker than normal blood) the

following may occur:following may occur: Key features:Key features: Client’s facial skin and mucous membranes have a Client’s facial skin and mucous membranes have a

dark, flushed (plethoric) appearance dark, flushed (plethoric) appearance Distention of superficial veinsDistention of superficial veins Weight lossWeight loss Intense itchingIntense itching Hypertension Hypertension Fatigue, enlarged hemorrhoidsFatigue, enlarged hemorrhoids Swollen painful jointsSwollen painful joints Enlarged firm spleenEnlarged firm spleen Infarctions of the heart (chest pain, heart failure), Infarctions of the heart (chest pain, heart failure),

kidneyskidneys StrokesStrokes Bleeding tendencyBleeding tendency

Polycythemia VeraPolycythemia Vera Diagnostic TestsDiagnostic Tests Blood cell counts and hematocrit Blood cell counts and hematocrit

markedly elevatedmarkedly elevated Hyperuricemia due to high cell Hyperuricemia due to high cell

destructiondestruction Bone marrow hypercellularBone marrow hypercellular Hgb levels to 18 g/dl Hct of 55% or Hgb levels to 18 g/dl Hct of 55% or

>> RBC count of 6 mil/mm3RBC count of 6 mil/mm3

Polycythemia vera Polycythemia vera Collaborative management:Collaborative management: Phlebotomy (treatment) blood drawing Phlebotomy (treatment) blood drawing Increase hydrationIncrease hydration Anticoagulants are part of therapy to Anticoagulants are part of therapy to

prevent clot formationprevent clot formation Chemotherapy to suppress bone Chemotherapy to suppress bone

marrow activitymarrow activity Radiation therapyRadiation therapy Bone marrow transplantationBone marrow transplantation Significant number of individuals with PV go Significant number of individuals with PV go

on to develop acute leukemiaon to develop acute leukemia

Polycythemia veraPolycythemia vera Client education guideClient education guide Drink at least 3 L dayDrink at least 3 L day Avoid tight or constrictive clothing, Avoid tight or constrictive clothing,

especially garters or girdlesespecially garters or girdles Wear gloves when outdoors in Wear gloves when outdoors in

temperature lower than 50 degreestemperature lower than 50 degrees Contact physician first sign of infectionContact physician first sign of infection Use soft-bristled toothbrushUse soft-bristled toothbrush Do not floss teethDo not floss teeth

Polycythemia veraPolycythemia vera Take anticoagulants as prescribedTake anticoagulants as prescribed Wear support hose while awake and upWear support hose while awake and up Elevate feet when you are seatedElevate feet when you are seated Exercise slowly and only on the advice Exercise slowly and only on the advice

of your physicianof your physician Stop activity at the first sign of chest Stop activity at the first sign of chest

painpain Use electric shaverUse electric shaver

PolycythemiaPolycythemia Production and presence of increased RBCsProduction and presence of increased RBCs 2 types: 2 types: Primary polycythemiaPrimary polycythemia = Polycythemia = Polycythemia

VeraVera Secondary PolycythemiaSecondary Polycythemia = a.) hypoxia = a.) hypoxia

driven = high altitude, cardiopulmonary driven = high altitude, cardiopulmonary disease, defection O2 transportdisease, defection O2 transport

b.)Hypoxia independent= renal cysts or b.)Hypoxia independent= renal cysts or tumorstumors

PolycythemiaPolycythemia Polcycythemia Vera=(PV) is a Polcycythemia Vera=(PV) is a

rare disease with a sustained rare disease with a sustained increase in blood cells (primarily increase in blood cells (primarily red blood cells) produced by the red blood cells) produced by the bone marrowbone marrow

With unknown origin, With unknown origin, chromosomal defect in chromosomal defect in pluripotent stem cellspluripotent stem cells

It is a cancer of the RBCs with 3 major It is a cancer of the RBCs with 3 major hallmarks:hallmarks:

1.) Massive production of 1.) Massive production of red blood red blood cellscells

2.) Excessive 2.) Excessive leukocyteleukocyte production production 3.) Excessive production 3.) Excessive production of plateletsof platelets

Hgb levels to 18 g/dl Hct of 55% Hgb levels to 18 g/dl Hct of 55% or >or >

RBC count of 6 mil/mm3RBC count of 6 mil/mm3

Aplastic Anemia (macrocytic)Aplastic Anemia (macrocytic) Cause:Cause: deficiency of circulation RBCdeficiency of circulation RBC due to due to

failure of bone marrow to produce these failure of bone marrow to produce these RBC cells, may occur alone or with RBC cells, may occur alone or with

LeukopeniaLeukopenia (decreased WBC) and (decreased WBC) and thrombocytopeniathrombocytopenia (decreased (decreased

platelets). When ALL three occur platelets). When ALL three occur together it is called together it is called “Pancytopenia”“Pancytopenia”

CauseCause:: 1.) Congenital in origin – chromosomal 1.) Congenital in origin – chromosomal

abnormalityabnormality 2.)2.) Acquired--Long term exposure to toxic agents, Acquired--Long term exposure to toxic agents,

ionizing radiation or infection, (viral, bacterial), ionizing radiation or infection, (viral, bacterial), medications, antiseizure, antimicrobials) may medications, antiseizure, antimicrobials) may cause Aplastic anemia.cause Aplastic anemia.

3.) 70% of acquired is idiopathic (unknown cause)3.) 70% of acquired is idiopathic (unknown cause)

Signs and Symptoms:Signs and Symptoms:

Onset insidius: Onset insidius: manifestations include those of anemiamanifestations include those of anemia Those of leukopenia (recurrent multiple Those of leukopenia (recurrent multiple

infections)infections) Those related to thrombocytopenia (petechia, Those related to thrombocytopenia (petechia,

tendency to bleed excessively, tendency to bleed excessively, especially in the mouth.especially in the mouth.

Aplastic Anemia (macrocytic)Aplastic Anemia (macrocytic) Diagnosis:Diagnosis: definitive bone marrow aspiration definitive bone marrow aspiration

red bone marrow is replaced by fatty red red bone marrow is replaced by fatty red bone marrowbone marrow

Treatment:Treatment: blood transfusions, blood transfusions, immunosuppressive therapy (antilymphocyte immunosuppressive therapy (antilymphocyte globulin (ALG), cyclosporine (Sandiuumne), globulin (ALG), cyclosporine (Sandiuumne), prednisone, cyclophosphamide (Cytoxan) can prednisone, cyclophosphamide (Cytoxan) can bring about partial or complete remissions.bring about partial or complete remissions.

Splenectomy on clients with enlarged spleen Splenectomy on clients with enlarged spleen that is either destroying normal RBCs or that is either destroying normal RBCs or suppressing their developmentsuppressing their development

Bone marrow transplant.Bone marrow transplant.

Hemolytic AnemiaHemolytic Anemia Destruction or hemolysis of RBCs at a Destruction or hemolysis of RBCs at a

rate that exceeds productionrate that exceeds production Third major cause of anemiaThird major cause of anemia Intrinsic hemolytic anemiaIntrinsic hemolytic anemia

• Abnormal hemoglobin Abnormal hemoglobin (sickle cell) (sickle cell)

• Enzyme deficienciesEnzyme deficiencies• RBC membrane abnormalities RBC membrane abnormalities

Hemolytic AnemiaHemolytic Anemia Extrinsic hemolytic anemiaExtrinsic hemolytic anemia

• Acquired Acquired (mechanical injury heart bypass, toxins)(mechanical injury heart bypass, toxins)

Sites of hemolysisSites of hemolysis• IntravascularIntravascular• ExtravascularExtravascular

Hemolytic AnemiaHemolytic Anemia JaundiceJaundice

• Destroyed RBCs cause Destroyed RBCs cause increased bilirubinincreased bilirubin Enlarged spleen and liverEnlarged spleen and liver

• Hyperactive with macrophage phagocytosis Hyperactive with macrophage phagocytosis of the defective RBCsof the defective RBCs

Accumulation of hemoglobin molecules Accumulation of hemoglobin molecules can obstruct renal tubules can obstruct renal tubules • Tubular necrosisTubular necrosis

2.) Hemolytic Anemias resulting 2.) Hemolytic Anemias resulting from increased destruction from increased destruction

(hemolysis) of RBCs(hemolysis) of RBCs a.) Sickle cell diseasea.) Sickle cell disease — — b.)b.) Glucose-6-Phosphate Glucose-6-Phosphate

Dehydrogenase Deficiency anemiaDehydrogenase Deficiency anemia c.)c.) Thrombotic Thrombocytopenia Thrombotic Thrombocytopenia

Purpura (TTP)Purpura (TTP) is a rare autoimmune is a rare autoimmune reaction in blood vessels disorder in reaction in blood vessels disorder in which which plateletsplatelets clump together clump together abnormally in the capillaries and few abnormally in the capillaries and few remain in circulation.remain in circulation.

HemochromatosisHemochromatosis

Primary hemochromatosisPrimary hemochromatosis is an is an inherited disorder characterized by inherited disorder characterized by excessive iron accumulation due to excessive iron accumulation due to increaxed intestinal iron absorption increaxed intestinal iron absorption causing tissue damage. causing tissue damage.

SymptomsSymptoms do not develop until organ do not develop until organ damage, often irreversible, develops.damage, often irreversible, develops.

Symptoms includeSymptoms include fatigue, fatigue, hepatomegaly, bronze skin pigmentation, hepatomegaly, bronze skin pigmentation, loss of libido, arthalgias, and loss of libido, arthalgias, and manifestations of cirrhosis, diabetes, or manifestations of cirrhosis, diabetes, or cardiomyopathy. cardiomyopathy.

DiagnosisDiagnosis is based on serum iron studies is based on serum iron studies and gene assay. and gene assay.

TreatmentTreatment serial phlebotomies serial phlebotomies

Leukemia Leukemia Type of cancer with uncontrolled Type of cancer with uncontrolled

production of immature white production of immature white blood cells in the bone marrowblood cells in the bone marrow

Acute or chronicAcute or chronic Classified by cell typeClassified by cell type Risk factors: ionizing radiation, Risk factors: ionizing radiation,

exposure to certain chemicals and exposure to certain chemicals and drugs, bone marrow hypoplasia, drugs, bone marrow hypoplasia, genetic factors, immunologic genetic factors, immunologic factors, environmental factorsfactors, environmental factors

Leukemias are grouped into 4 types---Leukemias are grouped into 4 types---according to how quickly they progress according to how quickly they progress

and the type of cell involved.and the type of cell involved.1.) Acute Lymphocytic Leukemia (ALL)** 1.) Acute Lymphocytic Leukemia (ALL)**

((malignant cells are mainlyB malignant cells are mainlyB lymphocytes)lymphocytes)

----most common in childrenmost common in children --Signs and symptoms may appear --Signs and symptoms may appear

abruptlyabruptly• FeverFever• BleedingBleeding• CNS manifestations, commonCNS manifestations, common

22.) Acute Myeloid.) Acute Myeloid(myelogenous)(myelogenous) Leukemia (AML) Leukemia (AML) Myeloblasts affected = precursor to Myeloblasts affected = precursor to

granulocytesgranulocytes ((malignant cells malignant cells granulocytes granulocytes (neutrophils, eosinophils, (neutrophils, eosinophils,

basophils) basophils)

Also called acute nonlymphoblastic Also called acute nonlymphoblastic leukemia (ANLL)leukemia (ANLL)

--stem cell of WBC proliferates, decreasing --stem cell of WBC proliferates, decreasing stem cells availability for RBC and plateletsstem cells availability for RBC and platelets

--result hyperplasia of bone marrow--result hyperplasia of bone marrow --25% of all leukemias --25% of all leukemias

• 85% of the acute leukemias in adults85% of the acute leukemias in adults --Abrupt, dramatic onset--Abrupt, dramatic onset

• Serious infections or abnormal bleedingSerious infections or abnormal bleeding

3.) 3.) Chronic Lymphocytic Leukemia (CLL)Chronic Lymphocytic Leukemia (CLL) --most common form of adult --most common form of adult

leukemialeukemia ( malignant cells are B lymphocytes)( malignant cells are B lymphocytes) * Lymph node enlargement is present * Lymph node enlargement is present

throughout bodythroughout body• Increased incidence of infection Increased incidence of infection Pain, paralysis from pressure caused by Pain, paralysis from pressure caused by

enlarged lymph nodes occurs in later stage enlarged lymph nodes occurs in later stage of diseaseof disease

4.) 4.) Chronic Myeloid Leukemia (CML)---Chronic Myeloid Leukemia (CML)--- (malignant cells are granulocytes= (malignant cells are granulocytes= eosinophis, basophils, neutrophils)eosinophis, basophils, neutrophils)

**Philadelphia chromosomePhiladelphia chromosome• Genetic markerGenetic marker* Move into peripheral blood in massive * Move into peripheral blood in massive

numbersnumbers• Ultimately infiltrate liver and spleenUltimately infiltrate liver and spleen

Clinical ManifestationsClinical Manifestations Cardiovascular: heart rate is Cardiovascular: heart rate is

increased; blood pressure is increased; blood pressure is decreased.decreased.

Respiratory rate increases.Respiratory rate increases. Skin grows pale and cool to the touch.Skin grows pale and cool to the touch. Intestinal manifestations include Intestinal manifestations include

weight loss, nausea, and anorexia.weight loss, nausea, and anorexia. Central nervous system disturbances Central nervous system disturbances

include headache.include headache.

Laboratory AssessmentLaboratory Assessment Decreased hemoglobin and Decreased hemoglobin and

hematocrit levelshematocrit levels Low platelet countLow platelet count Abnormal white blood cell count, Abnormal white blood cell count,

may be low, normal or elevated, may be low, normal or elevated, but is usually quite highbut is usually quite high

Poorer prognosis: client with Poorer prognosis: client with high white blood cell count at high white blood cell count at diagnosisdiagnosis

(Continued)(Continued)

Laboratory AssessmentLaboratory Assessment

(Continued)(Continued) Definitive test: examination of Definitive test: examination of

cells obtained from bone marrow cells obtained from bone marrow aspiration and biopsyaspiration and biopsy

Risk for InfectionsRisk for Infections Infection is a major cause of Infection is a major cause of

death in the client with death in the client with leukemia, and sepsis is a leukemia, and sepsis is a common complication.common complication.

AutocontaminationAutocontamination Cross-contaminationCross-contamination

Drug Therapy for Acute Drug Therapy for Acute LeukemiaLeukemia

Induction therapyInduction therapy Consolidation therapyConsolidation therapy Maintenance therapyMaintenance therapy New drug therapiesNew drug therapies Drug therapy for infectionDrug therapy for infection

Infection ProtectionInfection Protection Frequent handwashingFrequent handwashing Private roomPrivate room HEPA filtration or laminar airflow HEPA filtration or laminar airflow

systemsystem Mask for visitor with upper Mask for visitor with upper

respiratory infectionrespiratory infection(Continued)(Continued)

Infection Protection Infection Protection (Continued)(Continued)

““Minimal bacteria diet” without Minimal bacteria diet” without uncooked foodsuncooked foods

Monitoring of daily laboratory Monitoring of daily laboratory resultsresults

Assessment of vital signsAssessment of vital signs Skin care, respiratory careSkin care, respiratory care

Bone Marrow TransplantationBone Marrow Transplantation Standard treatment for leukemiaStandard treatment for leukemia Purges present marrow of the Purges present marrow of the

leukemic cellsleukemic cells After conditioning, new, healthy After conditioning, new, healthy

marrow given to the client toward a marrow given to the client toward a curecure

Sources of stem cellsSources of stem cells Conditioning regimenConditioning regimen TransplantationTransplantation

Risk for InjuryRisk for Injury Nadir: period of greatest bone Nadir: period of greatest bone

marrow suppressionmarrow suppression Bleeding precautionsBleeding precautions FatigueFatigue Interventions: Interventions:

• Diet therapyDiet therapy• Blood replacement therapyBlood replacement therapy• Drug therapyDrug therapy• Energy conservationEnergy conservation

Hodgkin’s LymphomaHodgkin’s Lymphoma Cancer that starts in a single lymph Cancer that starts in a single lymph

node or a single chain of nodesnode or a single chain of nodes Marker: Reed-Sternberg cellMarker: Reed-Sternberg cell Large, painless lymph node usually Large, painless lymph node usually

in the neck; fever, malaise, night in the neck; fever, malaise, night sweatssweats

One of the most curable cancersOne of the most curable cancers Treatment: external radiation alone Treatment: external radiation alone

or with combination chemotherapyor with combination chemotherapy

Non-Hodgkin’s LymphomaNon-Hodgkin’s Lymphoma All lymphoid cancers that do not have All lymphoid cancers that do not have

the Reed-Sternberg cellthe Reed-Sternberg cell More than 12 types of non-Hodgkin’s More than 12 types of non-Hodgkin’s

lymphomalymphoma Low-grade lymphomas less responsive Low-grade lymphomas less responsive

to treatment; cures are rareto treatment; cures are rare Treatment: radiation therapy and Treatment: radiation therapy and

multiagent chemotherapy, or single-multiagent chemotherapy, or single-agent therapy with fludarabineagent therapy with fludarabine

Multiple MyelomaMultiple Myeloma White blood cell cancer that White blood cell cancer that

involves a more mature lymphocyte involves a more mature lymphocyte than either leukemia or lymphomathan either leukemia or lymphoma

Uncommon cancerUncommon cancer Manifestations: fatigue, easy Manifestations: fatigue, easy

bruising, bone pain, fractures, bruising, bone pain, fractures, hypertension, increased infection, hypertension, increased infection, hypercalcemia, and fluid imbalancehypercalcemia, and fluid imbalance

Treatment: chemotherapyTreatment: chemotherapy

Autoimmune Autoimmune Thrombocytopenic PurpuraThrombocytopenic Purpura Large ecchymosis or petechial Large ecchymosis or petechial

rash on arms, legs, upper rash on arms, legs, upper chest, and neckchest, and neck

Diagnosed by decreased Diagnosed by decreased platelet count and large platelet count and large numbers of megakaryocytes in numbers of megakaryocytes in the bone marrowthe bone marrow

(Continued)(Continued)

Autoimmune Autoimmune Thrombocytopenic PurpuraThrombocytopenic Purpura

(Continued)(Continued) Interventions include:Interventions include:

• Therapy to prevent bleedingTherapy to prevent bleeding• Drug therapy to suppress immune Drug therapy to suppress immune

functionfunction• Blood replacement therapyBlood replacement therapy• Splenectomy Splenectomy

Thrombotic Thrombotic Thrombocytopenic PurpuraThrombocytopenic Purpura

Rare disorder; platelets clump Rare disorder; platelets clump together abnormally in the capillaries together abnormally in the capillaries and too few platelets remain in and too few platelets remain in circulationcirculation

Inappropriate clotting, yet blood fails Inappropriate clotting, yet blood fails to clot properly when trauma occursto clot properly when trauma occurs

Plasma pheresis, infusion of FFPPlasma pheresis, infusion of FFP Aspirin, alprostadil, plicamycinAspirin, alprostadil, plicamycin Immunosuppressive therapyImmunosuppressive therapy

Hemophilia Hemophilia Hemophilia A is deficiency of factor Hemophilia A is deficiency of factor

VIII and accounts for 80% of cases.VIII and accounts for 80% of cases. Hemophilia B (Christmas disease) is Hemophilia B (Christmas disease) is

deficiency of factor IX and accounts deficiency of factor IX and accounts for 20% of cases.for 20% of cases.

For hemophilia A with blood For hemophilia A with blood transfusion and factor VIII therapy, transfusion and factor VIII therapy, survival time has increased greatly.survival time has increased greatly.

Transfusion Therapy Transfusion Therapy Pretransfusion responsibilities to Pretransfusion responsibilities to

prevent adverse transfusion prevent adverse transfusion reactions:reactions:• Verify prescription.Verify prescription.• Test donor’s and recipient’s blood Test donor’s and recipient’s blood

for compatibility.for compatibility.• Examine blood bag for identification.Examine blood bag for identification.• Check expiration date.Check expiration date.• Inspect blood for discoloration, gas Inspect blood for discoloration, gas

bubbles, or cloudiness.bubbles, or cloudiness.

Transfusion Responsibilities Transfusion Responsibilities Provide client education.Provide client education. Assess vital signs.Assess vital signs. Begin transfusion slowly and stay Begin transfusion slowly and stay

with client first 15 to 30 minutes.with client first 15 to 30 minutes. Ask client to report unusual Ask client to report unusual

sensations such as chills, shortness sensations such as chills, shortness of breath, hives, or itching.of breath, hives, or itching.

Administer blood product per Administer blood product per protocol.protocol.

Types of TransfusionsTypes of Transfusions Red blood cellRed blood cell Platelet transfusionsPlatelet transfusions Plasma transfusions: fresh Plasma transfusions: fresh

frozen plasmafrozen plasma CryoprecipitateCryoprecipitate Granulocyte (white cell) Granulocyte (white cell)

transfusionstransfusions

Transfusion Reactions Transfusion Reactions Clients can develop any of the Clients can develop any of the

following transfusion reactions:following transfusion reactions:• Hemolytic Hemolytic • AllergicAllergic• Febrile Febrile • BacterialBacterial• Circulatory overloadCirculatory overload

Autologous Blood Autologous Blood TransfusionTransfusion

Collection and infusion of client’s Collection and infusion of client’s own bloodown blood

Eliminates compatibility Eliminates compatibility problems; reduces risk for problems; reduces risk for transmission of bloodborne transmission of bloodborne diseasedisease

Preoperative autologous blood Preoperative autologous blood donationdonation

(Continued)(Continued)

Autologous Blood Autologous Blood Transfusion Transfusion (Continued)(Continued)

Acute normovolemic Acute normovolemic hemodilutionhemodilution

Intraoperative autologous Intraoperative autologous transfusiontransfusion

Postoperative blood salvagePostoperative blood salvage