Transcript Management of Clients with Hematologic Disorders

Management of Clients with
Hematologic Disorders
NRS 108
Majuvy L. Sulse RN, MSN, CCRN
HEMATOLOGIC DISORDERS
Disorders associated with Erythrocytes
 Disorders of bleeding
 Disorders associated with white blood
cells
 Lymphomas

ANEMIA
(decreased in number of RBCs)
Abnormal & deficient production,
blood loss, destruction of RBC
Low Hgb
( Causes)
H/H, Bone marrow
aspiration, Peripheral
smear
( Diagnosis)
Less 02 carrying capacity
Hypoxia
Pallor, Fatigue, Palpitation, Low BP, SOB, DOE, MI, Renal failure
Management of Anemia
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Medical:
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Identify cause
Treat cause
Relieve
symptoms
Prevent
complications
Nursing:
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Assess
Educate
Classification of Anemia
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Based on the size of the RBC
Normocytic
 Microcytic
 Macrocytic
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Iron Deficiency Anemia
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Causes:
 Inadequate iron supply
 Chronic blood loss without iron replacement
 Decreased iron absorption in the intestines
Signs/ Symptoms:
 Hypochromic (low MCH), microcytic ( low MCV)
 Elevated serum binding capacity
 Brittle, spoon-shaped nails with longitudinal ridges
 Cheilosis (painful mouth cracks/ sores)
 Red shiny tongue
 Insidious development of fatigue
Diagnosis/Treatment
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Treatment:
 Identify cause
Diagnosis:
 Laboratory values
 Gastroscopy
 Sigmoidoscopy
 Occult blood in stools
 Radiographic studies
of GI
 Iron supplement
 Nutritional/dietary
Megaloblastic Anemia
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Predominance of megaloblasts & lack of normoblasts
Includes pernicious anemia, Vit. B12 & Folic acid
deficiencies
Related to surgery particularly of terminal ileum where
B12 is absorbed; vegetarian diet; prolonged exposure
to nitrous oxide
Related to aging & long term gastritis
Related to alcohol malnutrition & malabsorption (Folic
acid deficiency)
 Macrocytic, normochromic RBC
Lack of Intrinsic factor
Autoimmune response
Diagnosis & Treatment:
(Megaloblastic Anemia)

Schilling test- definitive dx of pernicious anemia
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Gastric secretion analysis- pH, free HCL, low gastric
secretion
Diagnosis & Treatment :
(Megaloblastic Anemia)
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ManagementLifelong tx with Vit B12 injection
 Iron & Folic acid supplement
 Nutritional/ dietary changes
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Foods that are rich in folic acid and vitamin B12
include the following:
eggs
meat
poultry
milk
shellfish
fortified cereals
Aplastic Anemia
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Low Hb & pancytopenia
Unknown etiology / autoimmune disturbance
Direct injury by Myelotoxins- agents causing bone marrow
damage when received in large doses
 Medication induced:
 Chemo, chloramphenicol, sulfonamides,
mephenytoin, quinine
 Exposure to environmental hazards:
 Benzene, insecticides, radiation & radioactive
materials
 Infections
 Hepatitis, miliary TB, EPBV
Congenital/hereditary causes
Aplastic Anemia-Manifestations & Dx
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Manifestations Exertional dyspnea, fatigue, pallor, infections
 Bleeding (nasal,oral, rectal,vaginal) ecchymosis,
petechiae, pupura
 Low platelets (less 30, 000), RBCs, WBCs
 Dry bone marrow on aspiration
Diagnosis Differential count
 Manifestations
 History of exposure to myelotoxins
Aplastic Anemia
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Medical
 Remove causative
 Idiopathic cause- treat with steroids, hormone
therapy
 Autoimmune cause- bone marrow transplants
(younger than 30 or those who have not received
transfusions yet
 Antithymocyte & cyclosporine therapy- skin testing
& watch for allergic reactions
 Blood transfusions
 Frequent CBCs esp for those on radiation therapy
Nursing management
 Infection control
 Client education
Anemia from Blood Loss
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Types
 Acute-trauma, complications from surgery
 Hypovolemia, hypotension, hypoxemia,
weakness, tachycardia, stupor
 Chronic- bleeding ulcer, hemorrhoids
 Gradual and vague symptoms as fatigue, pallor,
dyspnea
Medical/ Nursing Interventions
 Identify source of bleeding
 Control through medical or surgical interventions
 Blood transfusions
Hemolytic Anemia
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Abnormal destruction of RBCs by
 Intrinsic-defective RBCs, enzyme deficit(G6PD)
 extrinsic factors- toxins, injury as in prosthetic heart
valves
Failure of bone marrow to replace destroyed RBCs
Sites of hemolysis:
 Intravascular-circulation
 Extravascular- macrophages of spleen, liver & bone
marrow
Findings/ Treatment
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Findings
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Normocytic
anemia
Reticulocytosis as
compensatory
mechanism
Increased RBC
fragility
Short lifespan
Hyperbilirubinemia
- blood, urine,
stools
S/S as in Anemia
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Treatment
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Identify/Treat
cause
IV fluids to flush
kidneys
NAHCO3 or Na
Lactate to alter
urine pH
(decrease)
precipitation in
renal tubules
Splenectomy
Sickle Cell Anemia and Sickle Cell
Trait
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Inherited, autosomal, recessive disorders of HB
synthesis resulting in decrease O2 to the tissues
(hypoxia) and obstruction of blood vessels
Substitution of valine for glutamic acid in B-globin
gene
Primarily seen in the black population
Sickle cell trait is a mild form of the disease and it is
the commonest
Sickle cell trait is prevalent in Africa
Resistant to the parasite that causes malaria
Genetically inherited from each parent
Inheritance
Pathophysiology

Sickling of RBC-triggered by
 Hypoxia- low O2 tension in blood
 High altitude
 Emotional/ physical stress
 Surgery
 Blood loss
 Infection-bacterial & viral
 Dehydration
 Acidosis
 Decreased plasma volumeIncreased blood viscosity
 Hypothermia
 Stress
Pathophysiology
Sickled cell-rigid & elongated causes
tissue injury (as cells can’t pass through
small vessels) results in local hypoxia
anemia as more cells are hemolyzed by
spleen
 Initially reversible with re-oxygenation
then becomes irreversible due to cell
membrane damage from recurrent
sickling
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Sickle Cell crisis
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Exacerbation of RBC sickling
Vaso- occlusion (Vaso-occlusive crises) vasospasm
Changes in membrane permeability plasma
loss & hemoconcentration
development of
thrombi, tissue ischemia, infarction, necrosis
Shock is a life threatening consequence
Frequency, extent, & severity of episode is
dependent on percentage of HbS present
Causes of Sickle Cell Crisis
Vaso - Occlusion
 Aplastic crisis
 Hemolytic crisis
 Sequestration crisis
 Mixed crisis
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Types of Sickle Cell Disease
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Sickle cell anemia
Sickle cell Thalassemia
Sickle cell HbC-
Clinical Manifestations
Cardiovascular changes
 Skin changes
 Abdominal changes
 Musculoskeletal changes
 Central nervous system changes
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Clinical Manifestation
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Noticed after 6 months when fetal Hb is no
longer present
Improper growth related to anemia
Retarded growth and delay in sexual maturity
Hand- foot syndrome- edema of hands and
feet
Pain from tissue ischemia-hands, feet , joints
Clinical Manifestation
Weakness and fatigue-exercise
intolerance
 Jaundice- development of gallstones
 Pallor-of mucous membrane-grayish cast
on skin
 Priapism-occlusion of penile veins
 Infarct on the spleen - small and scarred
 Leg ulcers in about 75% of cases
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Complications
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CHF from ischemia &
heart enlargement
Acute Chest syndromefever, chest pains, cough,
dyspnea
Pulmonary infarctpulmonary HTN, MI, Cor
Pulmonale
Blindness from retinal
obstruction-hemmorhagedetachment
Renal failurehemoconcentration
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Autosplenectomyshrinking of spleen from
repetitious scarring
 CVA- thrombus
formation
 Osteoporosis/
Osteosclerosis
 Leg ulcers esp. at
ankles-tissue hypoxia
 Infection- absence of
phagocytic activity by
spleen
Diagnostic findings
Peripheral Smear
 Sickling Test
 DNA
 Elevated serum bilirubin levels
 Skeletal xrays-reveal bone/joint
deformities & flattening
 MRI-check for clots (CVA)
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Medical/ Nursing Management
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No cure
 Supportive interventions: pain relief,
 Hydration, O2, rest, blood transfusions
 Patient Teaching to avoid factors that cause
crisis
 Assessment of family understanding of the
disease & coping mechanism
 Links for support
 Genetic counseling
Get Involved Class!
What nursing diagnosis should receive
the highest priority in a client with sickle
cell crisis?
 A nurse is preparing a teaching plan for a
sickle cell client, what should the nurse
emphasize on to prevent sickle cell
crisis?
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Polycythemia Vera
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Excessive production of erythrocytes,
leukocytes, platelets due to excessive
activation of pluripotent stem cells
 Manifestations
 HTN- headache, vertigo, tinnitus, dizziness,
visual disturbances
 CHF- angina- hypervolemia & viscosity
 Intermittent claudication- thrombophlebitis
Polycythemia Vera
Stroke- thrombi formation
 Pruritus- histamine release from basophils
 Hemmorrhage- vessel rupture from tissue
distention
 Hepatomegaly & Slenomegaly from organ
engorgement
 Plethora- Ruddy complexion
 Gout—hyper uric acid production from RBC
destruction

Diagnosis
RBC=8-12 million
 Hgb=18-25 gm
 Increased platelet count
 HCT= >54% in men; 49% in women
 Elevated WBCs with basophilia
 Increased serum uric acid, B12
 Splenomegaly
 Hyperplastic bone marrow
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Medical/Nursing Management
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Goal of treatment is
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Reduce blood volume & viscosity
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Phlebotomy to Hct levels of 45-48% (about 300500ml/day)
Reduce bone marrow activitymyelosuppressive agents & radioactive
phosphorous
Hydration therapy with I & O
DISORDERS OF BLEEDINGTHROMBOCYTOPENIA
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Decreased production of platelets below
150,000/uL
 Manifests as bleeding- skin bruises easily
 Maybe
 acquired –food, drugs, infections, aplastic
anemia
 inherited- pancytopenia, hereditary
thrombocytopenia
Diagnosis/ Management
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CBC shows low platelet & Hb count
Assess for hx of NSAIDS
Avoid injury
Good oral hygiene & skin care
Rectal enemas & suppository-avoid constipation
Avoid IM, SC injections, rectal temperatures
Apply pressure on any bleeding source
Monitor signs of bleeding
Immune Thrombocytopenia Purpura( ITP)
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Autoimmune bleeding disorder
Platelets coated with antibodies
Destroyed by macrophages in liver & spleen
Survival is 1-3 days instead of 8-10
Gradual onset & transient remissions occur
Clinical Manifestations
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Petecchiae- small, flat, pinpoint red
Purpura-numerous petecchiae
Ecchymosis-large purplish lesions
EpistaxisBleeding gums
Heavy menses
Complication=hemorrhage
Diagnosis
Platelet Ct<100, 000
 Prolonged bleeding time with normal
coagulation time
 Increased capillary fragility
 Positive platelet antibody test
 Bone marrow aspirate contains normal
or increased megakaryocytes
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Management
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Cortecosteroids (prednisone)-suppreses the
phagocytic response of Spleenic macrophages,
depress antibody formation, reduce capillary
permeability & bleeding time
IV immunoglobulin (IVIG)
Immunosuppressive therapy
SplenectomyPlatelet transfusions
Hemophilia
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Characterized by prolonged bleeding after surgical/
dental or small trauma or cuts
Types
 Hemophilia A- factor Vlll-most common-80% cases
 Hemophilia B- factor lX deficiency-inherited gene
 Von Willebrand’s disease- deficient Vlll & platelet
dysfunction
Etiology
Sex linked genetic disorder
 Transmitted by females but males
express the disorder
 Carriers transmit the gene to half their
daughters and the disorder to half their
sons
 Males transmit the gene to all their
daughters but none to their sons
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Clinical Manifestations
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Slow persistent bleeding from cuts/scratches
Delayed hemorrhage-hours/days after injury
Severe bleeding after dental surgery
GI bleed
Nosebleed
Hematoma
Prolonged APTT
Treatment
Goal is to stop bleeding
 Increase anti-hemophilic factor-give
factor Vlll, lX
 Prevent complications
 Support therapy
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LEUKEMIA
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DESCRIPTION
 Malignant exacerbation in the number of
leukocytes, usually at an immature stage, in the
bone marrow
 May be acute, with a sudden onset and short
duration, or chronic, with a slow onset and
persistent symptoms over a period of years
 Affects the bone marrow, causing anemia,
leukopenia, the production of immature cells,
thrombocytopenia, and a decline in immunity
LEUKEMIA
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DESCRIPTION
The cause is unknown and appears to
involve gene damage of cells leading to the
transformation of cells from a normal state
to a malignant state
 Risk factors include genetic, viral,
immunological, and environmental factors
and exposure to radiation, chemicals, and
medications
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CLASSIFICATION OF LEUKEMIA
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ACUTE LYMPHOCYTIC LEUKEMIA (ALL)
 Mostly lymphoblasts present in bone marrow
 Age of onset is less than 15 years
 CNS manifestation common-leukemic
meningitis
 Normally, the lymphocytes fight infection by
making antibodies that attack harmful
elements. But, in ALL, the cells are immature
and overabundant. They crowd out other blood
cells, and may collect in the blood, bone
marrow, and lymph tissue.
CLASSIFICATION OF LEUKEMIA
 ACUTE
MYELOGENOUS LEUKEMIA
(AML)
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affects the young blood cells (called blasts) that
develop into a type of white blood cell (called
granulocytes). The main function of
granulocytes is to destroy bacteria. The blasts,
which do not mature and become too
numerous, remain in the bone marrow and
blood.
Age of onset is between 15 and 39 years
Classification
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CHRONIC MYELOGENOUS LEUKEMIA (CML)
Mostly granulocytes present in bone marrow
 Age of onset is after 50 years of age
 CML occurs mainly in adults and is rare in
children
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CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
Mostly lymphocytes (B cells) present in
bone marrow
 Age of onset is after 50 years of age,
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Diagnosis
CBC-WBC may be normal, decreased or
elevated
 Decreased platelet
 Decreased Hb
 Bone marrow aspiration- increase in
marrow cells
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LEUKEMIA
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ASSESSMENT
Anorexia, fatigue, weakness, weight loss
 Anemia
 Bleeding (nosebleeds, gum bleeding, rectal
bleeding, hematuria, increased menstrual
flow)
 Petechiae
 Prolonged bleeding after minor abrasions or
lacerations
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Assessment
Elevated temperature
 Lymphadenopathy and splenomegaly
 Palpitations, tachycardia, orthostatic
hypotension
 Pallor and dyspnea on exertion
 Headache
 Bone pain and joint swelling
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Leukemia
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ASSESSMENT
Normal, elevated, or reduced white blood
cell (WBC) count
 Decreased hemoglobin and hematocrit
levels
 Decreased platelet count
 Positive bone marrow biopsy identifying
leukemic blast phase cells
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Leukemia
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INFECTION
A major cause of death in the
immunosuppressed client
 Can occur through autocontamination or
cross-contamination
 Common sites of infection are the skin,
respiratory tract, and gastrointestinal (GI)
tract
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Leukemia
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IMPLEMENTATION: INFECTION
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Initiate protective isolation procedure-also called
Neutropenic Precautions or Reverse isolation
Ensure frequent and thorough hand washing
Reduce exposure to environmental organisms by
eliminating raw fruits and vegetables (low-bacteria)
from the diet, fresh flowers from the client’s room,
and by not leaving standing water in the client’s
room
Avoid invasive procedures such as injections, rectal
temperatures, and urinary catheterization
Leukemia
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CLIENT EDUCATION: INFECTION
 Avoid crowds and those with infections
 Consume a low-bacteria diet and to avoid drinking
water that has been standing for longer than 15
minutes
 Avoid activities that expose the client to infection
such as changing a pet’s litterbox or working with
houseplants or in the garden
 Neither the client nor their household contacts
should receive immunization with a live virus
Leukemia
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BLEEDING
 During the period of greatest bone marrow
suppression (the nadir), the platelet count may be
extremely low, less than 10,000/mm3 (bleeding risk)
 Examine the client for signs and symptoms of
bleeding; examine all body fluids and excrement for
the presence of blood
 Handle the client gently; use caution when taking
blood pressures to prevent skin injury
 Measure abdominal girth, which can provide an
indication of internal hemorrhage
 Provide safety-pad side rails and sharp corners of
the bed and furniture
Leukemia
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IMPLEMENTATION: BLEEDING
 Provide soft foods that are cool to warm in
temperature
 Avoid injections if possible to prevent trauma to the
skin and bleeding; apply firm, gentle pressure to a
needle stick site for at least 10 minutes
 Avoid rectal suppositories, enemas, and
thermometers
 If the female client is menstruating, count the
number of pads or tampons used
 Administer blood products as prescribed
Leukemia
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FATIGUE AND NUTRITION
 Assist the client in selecting a well-balanced diet
 Provide small, frequent meals (high calorie, highprotein, high-carbohydrate) that require little
chewing
 Assist the client in self-care and mobility activities
 Allow adequate rest periods during care; do not
perform activities unless they are essential
 Administer blood products for anemia as prescribed
Leukemia
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CHEMOTHERAPY
Induction therapy: Aimed at achieving a
rapid, complete remission of all
manifestations of the disease
 Consolidation therapy: Administered early in
remission with the aim of cure
 Maintenance therapy: May be prescribed for
months or years following successful
induction and consolidation therapy; the aim
is to maintain remission
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Leukemia
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IMPLEMENTATION
 Administer antibiotic, antibacterial, antiviral,
and antifungal medications
 Blood replacements as needed
 Prepare the client for transplantation
 Administer colony-stimulating factors as
prescribed
 Maintain infection and bleeding precautions
Leukemia
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IMPLEMENTATION
Provide an adequate diet
 Provide an activity schedule that will
conserve energy
 Instruct the client in appropriate home care
measures
 Provide psychosocial support and support
services for home care

Leukemias
Acute
Combination Chemo
Induction
Rest- 3 weeks
Consolidation
Maintenace follows
remission- lasts 2-3 years
Bone marrow transplant
Chronic
Treatment based on
symptoms
Radiation to spleen, nodes
Interferon
Autologous stem cell
transplant
Blood transfusions: RBCs,
platelets, WBCs
HODGKIN’S DISEASE
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DESCRIPTION
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A malignancy of the lymph nodes that originates in
a single lymph node or a single chain of nodes;
metastasis occurs to other adjacent lymph
structures and eventually invades nonlymphoid
tissue
Usually involves lymph nodes, tonsils, spleen, and
bone marrow and is characterized by the presence
of the Reed-Sternberg cell in the nodes
Possible causes include viral infections and
previous exposure to alkalyting chemical agents
Prognosis is dependent on the stage of the disease
Etiology & Pathophysiology
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Unknown cause
Linked to Epstein-Barr Virus
Genetics- high among Jews
Mechanism of growth & spread- unknown
Cancerous cells transforms in lymph and
progresses to other nodes or direct infiltration
of blood vessels
Clinical Manifestations
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Enlargement of cervical, axillary, inguinal
lymph nodes may be painless
B symptoms-Weight loss, Fever, night sweats
Cough, stridor, dyspnea
Hepatomegaly
Splenomegaly
SVC syndrome from intra thoracic involvement
Diagnosis & Staging
Peripheral blood analysis-microcytic,
hypochromic anemia, leukocytosis
 Lymph node biopsy-definitive dx
 Bone marrow examination-staging( l-lV)
 CXR, CT Scan, radioisotope studiesdefine sites
 Lymphangiography-assess nodes &
vessels

Hodgkins Disease
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IMPLEMENTATION
For stage 1 and 2 without mediastinal node
involvement, the treatment of choice is
extensive external radiation of the involved
lymph node regions
 With more extensive disease, radiation
along with multi agent chemotherapy is
utilized
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HODGKIN’S DISEASE
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Implementation
 Monitor for side effects related to chemo or
radiation
 Monitor for signs of infection & bleeding
 Maintain infection and bleeding precautions
 Discuss possibility of sterility with male client
receiving radiation & inform of options related to
sperm banks
 Pain management
 Client & family support
 If terminal- ensure client has dignified death
NHL
NON- HODGKIN’S LYPHOMA
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Group of malignancies with origins in lymphoid
cells affecting all ages
Most common occurring cancer & 5th leading
cancer causing death
Men are more affected than women; survival
better for women than in men
Incidence higher in whites than other races
Painless lymph node enlargement is primary
manifestation
Symptoms will present on where disease has
spread
Diagnosis & Treatment
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Diagnostic tests are same as for Hodgkin’s
disease-Lymph node biopsy done
Treatment-radiation & chemotherapy
Nursing assessment for nodes characteristics,
B symptoms appearance
Support for client & family
Manage risk for infection, bleeding, sexual &
reproductive dysfunction
Nutritional management
Multiple Myeloma
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Cancer of the plasma cell, an important part of
the immune system that produces
immunoglobulins (antibodies) to help fight
infection and disease.
 Hypercalcemia, anemia, renal damage,
increased susceptibility to bacterial infection,
and impaired production of normal
immunoglobulin are common clinical
manifestations of multiple myeloma. It is often
also characterized by diffuse osteoporosis,
usually in the pelvis, spine, ribs, and skull.
Diagnostics/ Management
Diagnostics
Radiologic studies- lesions in
bones, demineralization,
osteoporosis
Bone marrow biopsy- immature
plasma cells
Abnormal immunoglobulin
Monoclonal immunoglobulin chains
in blood & urine exam
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Multiple Myeloma
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Management
Chemotherapy, radiation tx
 Autologous bone marrow transplant
 Plasmapheresis
 Hydration, diuretics, phosphate for treatment
of Hypercalcemia
 Pain management
 Supportive for anemia, leukopenia,
thrombocytopenia
