Chapter 47
Care of the Patient with a Blood
or Lymphatic Disorder
- Agranulocytosis
- Leukemia
- Coagulation Disorders
- Platelet Disorders
- Thrombocytopenia
- Clotting Factor Defects
- Hemophilia
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 1
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis

Etiology/pathophysiology
• Severe reduction in the number of granulocytes

Basophils, eosinophils, neutrophils
• WBC extremely low – leukopenia

Neutrophil count is < 200/mm3 – neutropenia
o Normal = 3000 to 7000/mm3
• Causes of agranulocytosis





Medications adverse reaction or toxicity primary cause
Chemotherapy
Radiation
Neoplastic disease
Viral and bacterial infections
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 2
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis

Etiology/pathophysiology
• Suppression of bone marrow reduces the number and
production of WBC
• Leukocytes from bone marrow provide body protection
against microorganisms
• Protection is ineffective when bone marrow suppression
has occured
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 3
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis (continued)

Clinical manifestations/assessment
• Symptoms of infection

Fever, headache, chills, fatigue
• Ulcerations of mucous membranes

Mouth, nose, pahrynx, vagina, rectum
• Bronchial pneumonia
• Urinary tract infection

Assessment
• Subjective data


Note common complaints of fever, extreme fatigue,
prostration
Meds taken, OTC & prescription may cause condition
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 4
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis (continued)

Assessment
• Objective data





Fever over 100.6º F
Erythema and pain from ulcers
Ulcers are cultured
Lung and bronchial auscultations reveals crackles and
ronchi due to trapped exudates
Causative chemical agents:
o Analgesics (Butazolidin), antibiotics
(chloramphenicol, penicillin derivatives,
cephalosporins), antiepileptics (phenytoin),
antihistamines, antineoplastic drugs (vincristine),
antithyroid (PTU), diuretics, phenothiazides
(thorazine, prolixin, sparine, compazine),
Sulfonamides and derivatives
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 5
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis (continued)

Diagnostic Tests
• Leukocytes with neutrophil differential < normal
• Bone marrow study

Medical management/nursing interventions
•
•
•
•
•
•
•
Remove cause of bone marrow depression
Prevent or treat infections
Blood and ulceration cultures
PRBCs for transfusion
G-CSF (filgrastim [Neupogen]) subQ for neutropenia
Meticulous hand washing
Strict asepsis
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 6
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis (continued)

Nursing Interventions
• Directed at protecting the patient from infection
• Meticulous hand washing
• Strict asepsis

Patient Teaching
• Discuss frequent, thorough oral hygiene

Treat or prevent mouth and pharyngeal infection
• Explain need for soft, bland diet high in protein, vitamins
and calories
• Balanced rest and activity to prevent fatigue and malaise
• Avoid crowds, people with infectious diseases, and cold
or hot environments;
• Teach S/Sx of infection and its intervention
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 7
Disorders of the Hematological
and Lymphatic Systems
• Agranulocytosis (continued)

Prognosis
• A potentially fatal condition due to possibility of a lifethreatening bacterial infection
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 8
Disorders of the Hematological
and Lymphatic Systems
• Leukemia

Etiology/pathophysiology
• Malignant disorder of the hematopoietic system
• Excess leukocytes accumulate in the bone marrow and
lymph nodes
• Cause unknown

Attributed to genetic origin, virus, previous radiation
treatment, chemotherapeutic agent toxic to bone marrow
• Marrow is replaced by white cells with abnormal
numbers and forms of immature cells found in
circulation and infiltrated into lymph nodes, spleen, liver
• Increased WBCs lead to infiltration and damage to BM,
lymph nodes, spleen, and organs
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 9
Disorders of the Hematological
and Lymphatic Systems
• Leukemia

Etiology/pathophysiology – cont’d
• Leukemic infiltration leads to:



Hepatomegaly, splenomegaly, lymphadenopathy, bone
pain, meningeal irritation, and oral lesion
Hematopoietic function is disturbed by incompetent BM
o Increased susceptability to infection
Classification
• Acute or chronic
• Proliferating cells (lymphocytic, monocytic, etc.)
• Types of leukocyte involved

Myelogenous or lymphocytic origin
• Four major types of leukemia

ALL, AML, CML, CLL
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 10
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Clinical manifestations/assessment
• BM failures results from:


1. BM overcrowding by abnormal cells
2. inadequate production of normal marrow elements
• Anemia
• Thrombocytopenia; leukopenia
• Leukemic cells infiltrate


Organs leading to splenomegaly, hepatomegaly,
lymphadenopathy, bone pain, meningeal irritation, oral
lesions
Enlarged lymph nodes and painless splenomegally first
sign of disease in some
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 11
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Diagnostic Tests
•
•
•
•
•
•
WBC is low, elevated, or excessively elevated
Anemia and thrombocytopenia
BM biopsy shows immature leukocytes
CXR shows mediastinal node and lung involvement
Node biopsy shows excessive blasts (immature cells)
Peripheral blood evaluation and bone marrow
examination are primary methods of diagnosing and
classifying type of leukemia
• Lumbar puncture and CT scan are done to determine
presence of leukemic cells outside of blood and BM
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 12
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Assessment
• Subjective Data



Complaints regarding symptoms that may seem
unrelated at first
Pain in bones or joints is noticed
Fatigue, malaise, decreased activity tolerance, irritability
• Objective Data




Infections are common
Occult blood is detected in urine and stool
Skin abnormalities
o Petechiae and ecchymoses
Mucous membrane abnormalities
o bleeding
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 13
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Medical management
• Goal is achieve remission or control symptoms
• Treatment is aimed at eradicating leukemia with:


Chemotherapy (mainstay for treatment); radiation
Bone marrow transplant
• Three purposes for using multiple drugs



1. decrease drug resistance
2. minimize drug toxicity to patient by using multiple
drugs with varying toxicities
3. interrupt cell growth at multiple points in cell cycle
• BM and stem cell transplantation selected as
treatment of choice in patients with suitable donors if
the initial remission of acute leukemia has been induced
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 14
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Medical management
• Chronic leukemia

Occurs almost exclusively in adults and develops slowly,
the desired objectives depend of kind of cells involved
• Medications: Leukeran, hydroxyurea, corticosteroids,
Cytoxan

Not curative but prolongs life
• Lymph node radiation is used and blood transfusion is
given for anemia
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 15
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Nursing Interventions and Patient Teaching
•
•
•
•
•
Prevention of infection and avoid infectious agents
Leukopenia (< 5000 cells/mm3) can be fatal
Throbocytopenia induced hemorrhage is life threatening
Pain is controlled through analgesia
Support of patient and family promoted through a
positive nurse-patient-family relationship and referral to
community support groups
• Nurses have contact with patient 24 hrs/day

Help reverse feelings of abandonment and loneliness by
balancing the demanding technical needs with a
humanistic, caring approach
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 16
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Nursing Interventions
• Physical care perspective



Nurse are challenged to make astute assessments and
plan care to help patient survive the severe side effects of
chemotherapy
Nurse must be knowledgeable about all drugs
o MOA, purpose, routes of administration, dose, S/E
o Know how to assess laboratory data reflecting drug
effects
Patient survival and comfort during aggressive
chemotherapy are significantly affected by quality of
nursing intervention
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 17
Disorders of the Hematological
and Lymphatic Systems
• Leukemia (continued)

Nursing Interventions
• Procedures, meaning of treatments, and care plans are
discussed by nurse and patient
• Community resources for support and information are
invaluable for education of patient and family
• Continuation of medical regimen is encouraged
• Avoidance of situations in which infection is transmitted
• Medication and diet are discussed
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 18
Disorders of the Hematological
and Lymphatic Systems
• Prognosis

Chemotherapy has improved the prognosis of children
with ALL
• Untreated median survival is 4 to 6 months
• Treatment with vincristine and prednisone and
anthracycline drug (daunorubicin and doxorubicin), the
median survival is 5 years
• 50% of children with ALL can be cured
• AML, remission is achieved in 75%
• 20 to 25% of adults with AML has 5 year remission
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 19
Disorders of the Hematological
and Lymphatic Systems
• Coagulation Disorders

Etiology/Pathophysiology
• Release of blood from vascular system is due to:

Trauma or vessel damage, vessel inadequacy,
disturbance of function of platelets or clotting factors, or
liver disease (impaired clotting mechanism)
• Clotting mechanism is a hemostatic chain reaction




Vasoconstriction inhibits capillary leakage
Hematoma compression provides pressure
Body reaction occurs: arterial BP lowers
Disruption in mechanism leads to hemorrhage
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 20
Disorders of the Hematological
and Lymphatic Systems
• Coagulation Disorders

Clinical Manifestations
• Petichiae and ecchymoses
• Epistaxis and gingival bleeding
• Circulatory hypovolemia is noted through hypotension:

Pallor, cold, clammy skin and tachycardia
• GI bleeding with flank pain
• CNS involvement

Altered response and malaise to loss of consciousness or
affected speech
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 21
Disorders of the Hematological
and Lymphatic Systems
• Coagulation Disorders

Assessment
• Subjective Data




Note history of bleeding after surgical or dental procedure
Exposure to toxic or hazardous agents or to radiation
Headache, extremity pain and numbness
Medications (i.e. aspirin)
• Objective Data



Pain on abdominal pressure
o Reveals spleen or liver tenderness and enlargement
Look for petechiae, ecchymoses, hematoma
Examination of emesis an stool may show signs of bleeding
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 22
Disorders of the Hematological
and Lymphatic Systems
• Coagulation Disorders

Diagnostic Tests
•
•
•
•

Platelet count is low
RBC is low with decreased Hgb level
Altered coagulation time
BM shows abnormal cells
Medical Management
• Underlying cause is assessed and corrected
• Heparin therapy and toxicity is considered as a cause
• Infections and complications are treated and prevented
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 23
Disorders of the Hematological
and Lymphatic Systems
• Coagulation Disorders

Nursing Interventions
• Nurse should monitor VS to note any signs of
hypovolemic shock
• Patient is moved gently to prevent trauma to tissues
• Nurse will monitor IV infusions and transfusions as
ordered
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 24
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Etiology/pathophysiology
• Deficiency of number of circulating platelets or change
in function of platelets alters the coagulation process
• Condition in which the number of platelets is reduced
below 150,000/mm3; may be due to decreased
production or decreased survival

Aplastic anemia, leukemia, tumors, chemotherapy
• Decreased platelet survival occurs in:

Presence of antibody destruction, infection, viral invasion
• Increased platelet destruction occurs in DIC
• Splenomegaly is due to entrapment of blood in spleen
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 25
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Etiology/pathophysiology
• Thrombocytopenic purpura


Most common cause of increased destruction of platelets
Drug induced or immune throbocytopenic purpura (ITP)
• ITP




Most common acquired thrombocytopenia
Syndrome of abnormal destruction of circulating platelets
Autoimmune disease
Platelets are coated with Ab and when they reach the
spleen they are destroyed by macrophages
• Medication induced

Platelet returns to normal 1 to 2 weeks after the
medication is withdrawn
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 26
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Etiology/pathophysiology
• Acute ITP is mostly in children
• Chronic ITP is most common in women 20 to 40 y/o
• It is an autoimmune process caused by production of an
autoantibody (IgG) directed against a platelet antigen

Clinical Manifestations
• Major signs are petichiae and ecchymoses on skin
• Petichiae only occurs in platelet disorder
• < 100,000/mm3 risk of bleeding from mucous
membranes and in cutaneous sites and internal organ
increases
• < 20,000/mm3 is significant risk for serious bleeding
• < 5000/mm3 leads spontaneous, fatal CNS or GI bleed
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 27
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Assessment
• Subjective Data

Ask patient about
o Recent viral infections (produce transient
thrombocytopenia)
o Medications in current use
o Extent of alcohol ingestion
• Objective Data



Observe for petichiae and ecchymoses
Epistaxis and gingival bleeding
Signs of increased ICP due to cerebral hemorrhage
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 28
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Diagnostic Tests
• Complete lab studies


Platelet count, peripheral blood smear, bleeding time
• Bone marrow aspiration to determine presence of immature
platelets
Medical Management
• Primary treatment are corticosteroid therapy and splenectomy

Ability to suppress phagocytic response of splenic
macrophages
• IV Ig or immunosuppressive drugs
• Platelet transfusions

Recommended when count is < 20,000/mm3 unless actively
bleeding
• Plasmapheresis is used to treat ITP by removing Ab
produced y autoimmune process
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 29
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Nursing Interventions and Patient Teaching
• Medication toxicity is the cause then D/C meds
• Infections are prevented by meticulous asepsis and
gentle handling of patient
• Monitor plasma and platelet infusion and whole blood
transfusion for reaction and effects on condition
• Instructions on S/Sx and preventive measures are given:
• Avoid trauma, use stool softeners, high fiber diet, check
for presence of blood, use soft tooth brush, gently blow
nose
• Stress the importance to notify physician for signs and
symptoms of bleeding
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 30
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Prognosis
• ITP

Treatment is administered 3 to 4 weeks before a
complete response is seen
• Chronic ITP

Transient remissions occur
o 80% benefit from splenectomy resulting in complete
or partial remission
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 31
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia

Clinical manifestations/assessment
•
•
•
•
Petechiae
Ecchymoses
Platelets below 100,000/mm3
Bleeding from mucous membranes
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 32
Disorders of the Hematological
and Lymphatic Systems
• Thrombocytopenia (continued)

Medical management/nursing interventions
•
•
•
•
•
•
Corticosteriod therapy
Splenectomy
Gamma globulin
Immunosuppressive drugs
Platelet transfusions
Avoid trauma
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 33
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia

Etiology/pathophysiology
• Hereditary coagulation disorder, characterized by a
disturbance of clotting factor
• Hemophilia A

85% of total incidence, antihemophillic factor VIII is
absent which is essential in converting prothrombin to
thrombin
• Hemophilia B (Christmas disease

Deficiency in Factor IX, absence of plasma
thromboplastin component leading to non formation of
thromboplastin
• X-linked hereditary trait affects mainly males, females
are carriers
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 34
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia

Etiology/pathophysiology – cont’d
• Decrease in formation of prothrombin activators occurs
as a result of decrease in clotting factors
• In the past, they were high risk for HIV infection
• Hemophilia A people developed AIDS due to
transfussion of factor VIII concentrate
• Heat treatment of Factor VIII destroys the virus
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 35
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia

Clinical manifestations/assessment
• Internal and external bleeding
• Hemarthrosis


Bleeding into joints is a hallmark of a severe disease
o Occurs in knees, ankles and elbow
Pain, edema, erythema, and fever occurs
• Excessive blood loss from small cuts and dental
procedures
• Pain from hemorrhage damage
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 36
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia

Assessment
• Subjective Data


Incidents of ecchymoses and hemorrhage from slightest
trauma
Pain with joint motion
• Objective Data



Note blood in subcutaneous tissues, urine, or stool
Note edematous or immobile joints
Diagnostic Tests
• Factors VIII and IX are absent/deficient
• Normal platelet count, BT, PT, INR
• PTT is prolonged
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 37
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia (continued)

Medical management
• Prevent and treat bleeding and relive pain are the focus
• Transfusion and administration of Factor VIII and IX



For prophylaxis and to stop hemorrhage
Cryoprecipitate
o Clotting factor concentrate rich in factor VIII
o Risk of viral disease transmission
Factor VIII concentrate
o Typically used
• Genetic engineering

Recombinant factor VIII is advatageous because of:
o Viral safety, unlimited supply, lower cost
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 38
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia (continued)

Medical management
• Minimize bleeding—avoid trauma
• Relieve pain—no aspirin
• Transfusions



Factor VIII or IX concentrate
Cryoprecipitate (rich in factor VIII)
Manufactured factor VIII or IX
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 39
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia (continued)

Nursing Interventions and Patient Teaching
• Nurse will control bleeding in emergency situation by
applying pressure and cold to the site
• Monitor transfusion of factor VIII
• Supportive care measures

Pain management and genetic counseling
• Not given aspirin
• Nurse and patient should discuss avoiding injury and
controlling bleeding
• Avoid trauma
• Supervision of young patient and inform playmates,
teachers and others
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 40
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia (continued)

Nursing Interventions and Patient Teaching
• Emergency care treatment

Immobilizing affected part, applying ice, notify physician
• Discuss diet to prevent obesity, which put excess
pressure on joints
• Regular dental care and preventive dental and medical
measures
• Discuss overprotection
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 41
Disorders of the Hematological
and Lymphatic Systems
• Hemophilia (continued)

Prognosis
• Hemophiliacs who received clotting factor concentrates
before 1984 became seropositive for HIV
• Now, risk of contracting HIV from clotting factor
concentrate is almost nil
• Average life span is near normal
• END!!!
Copyright © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. All rights reserved.
Slide 42