Hematologic Disorders
Katherine Schafer, MSN, APRN, PCNS-BC, CNL,
CCRN-K, CHSE
Instructor
Women, Children, & Family Nursing
Anemias
Iron Deficiency
 Aplastic Anemia
 Sickle Cell Disease
 Beta Thalassemia
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Let’s Begin with a Case Study
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M. is a 2 year-old male who was admitted to the inpatient pediatric
unit for pneumonia. He is on Day 3 of antibiotic therapy and has
been afebrile for 24 hours.
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T 98.8 axillary, HR 136, RR 22, BP 100/60, SaO2 95% on 1L O2 per
NC. The night nurse reports he is drinking well, so his IVF were
decreased to a KVO rate. His mother is at the bedside.
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You complete your 8 AM assessment.
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Significant Findings: Generally thin, but well-appearing child.
Lethargic and uninterested in playing. Skin pale & cool to the touch.
Pulses 3+, Cap refill 2 seconds. Lungs with adequate air exchange,
bilateral coarse sounds. S1 & S2 heart sounds with splitting of S2.
No appetite.
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What other information would you like?
Additional Information
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Eats pasta, cheese, crackers, chicken nuggets, &
“McDonalds”
Drinks about 1 quart of cow’s milk per day
Does not take a multivitamin
Favorite activities: “video games” & “watching cartoons”
Sleeps 16 hours per day. Falls asleep easily &
“everywhere”
Lives in a 70 year-old home with parents & 8 year-old
sibling
CBC Results
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WBC: 6.5
RBC: 4.1
HGB: 10.9
HCT: 29.5
MCV: 69
MCHC: 19
Retic: 0.8%
Range: 5.5 – 15.5
Range: 3.1 – 4.5
Range: greater than 11.5
Range: greater than 33.5
Range: greater than 80
Range: 24-30
Range: 0.5% - 2.0%
Iron Related Tests
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Serum Ferritin: 2 ng/ml
 Range: 7 – 142 ng/ml
Total Iron: 30 mcg/dl
Range: 50 – 120 mcg/dl
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Pb Level
Lead level: 4 mg/dl
 Reference Range: greater than 5 mg/dl
requires further investigation
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Iron Deficiency Anemia
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Most common cause of anemia
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Rare before 4 to 6 months of age
Etiology
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AAP recommends screening during infancy, early/late
childhood, and adolescence
Decreased iron intake
Increased iron or blood loss
Increased growth rate (children)
Can be associated with lead poisoning
Family education is critical
Collaborative Management
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Increase iron rich foods & assess milk weaning
Administer iron (elemental iron)
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Hgb should increase within 2 weeks
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Administer iron on empty stomach in an acidic
environment along with vitamin C
Clean teeth after administration (stains—and yuck
taste!)
Black stools
Therapy continued for at least 3 months after Hgb has
normalized
Food insecurity—social service referral
IMPORTANT NOTE: Iron pills are number one
pediatric ingestion – KEEP OUT OF REACH
Aplastic Anemia
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Defined
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Incidence
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A rare condition in which the bone marrow ceases
production of cells resulting in peripheral
pancytopenia
2 cases per million per year in Europe and United
States
As a reference point: leukemia has an incidence of 50
cases per million per year
Etiology
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Congenital
Acquired
 Chemicals, drugs, viruses, radiation, autoimmune
Idiopathic (70%)
Collaborative Management
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Diagnosed through bone marrow aspirate and
biopsy
Discontinue exposure to causative agent, if found!
Supportive Care
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Treatment
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Transfusions
 Platelet and RBCs
Infection control
Bleeding prevention
Bone marrow/stem cell transplant
Medication regimen of immune suppressants
Psychosocial support
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Referral to transplant center
http://www.aamds.org/aplastic/
Sickle Cell Disease
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Defined
 A group of hemoglobinopathies where normal Hb is
partially or totally replaced by abnormal HbS
 Several varieties, though HgbSS (SCA) most “classic”
Prevalence
 Particularly common among (but not exclusive to)
those whose ancestors came from sub-Saharan
Africa; Spanish-speaking regions in the Western
Hemisphere (South America, the Caribbean, and
Central America); Saudi Arabia; India; and
Mediterranean countries such as Turkey, Greece, and
Italy
Etiology
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Inherited, autosomal recessive
Genetic Transmission
Father:
Mother:
HbS
Hb
Hb
Hb
(sickle cell trait)
(unaffected)
Offspring:
HbSHb HbHb HbSHb HbHb
50% chance of child inheriting the trait
Father:
Mother:
HbS
HbS
Hb
Hb
(sickle cell trait)
(sickle cell trait)
Offspring:
HbSHbS HbSHb HbHbS HbHb
25% unaffected, 50% carry the trait, 25% affected
Pathophysiology
HbS
 Increased RBC destruction plus
decreased RBC lifespan=
chronic anemia
 High reticulocyte counts
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As new RBCs being made
Microcirculation obstructions
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Brain, Heart, Kidney, Eye, Spleen
Clinical Manifestations
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Often detected on
newborn screen, but
patient not symptomatic
for 4-6 months due to
fetal hemoglobin
Stressful event results in
sickling crisis
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Deoxygenation, acidosis,
dehydration, infection
Vaso-occlusive crisis
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Pallor, jaundice, fatigue
Delayed growth/puberty
Avascular necrosis or
hips and shoulders
Renal dysfunction
Retinopathy
Priapism
Cerebral infarct
Cardiomyopathy
Acute chest syndrome
(respiratory failure)
Acute sequestration
(hypovolemic shock)
Collaborative Management
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Folate
Prophylactic penicillin therapy by 2 months
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Invasive pneumococcal disease (10-fold risk over tha
of general population/15-35% fatality rate)
Hydroxyurea (if experiencing frequent VOC)
Vaccines: influenza, pneumococcal, Hib,
meningococcal
PRBC transfusions (routine)
Pain management (action plan)
Can be “cured” by bone marrow or stem cell
transplant (BMT/SCT)
VOC Management
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Treat cause (often infection)
Hydration
Oxygen if hypoxemic
Possible PRBC transfusions
Pain management—opioids, NSAIDS, heat
 Be self-aware of bias/presumptions about how pain
presents
 Challenges with vascular access
 Previous experiences with poorly managed pain can
affect future healthcare encounters
Monitor for signs of life-threatening complications (such
as?)
Patient/Family Education
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Fluid intake essential
 Expect frequent urination
Caution against extremes in temperature
Discuss risk of and monitoring for infection (primary
cause of death under 5 years)
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Adequate rest
Pain management (action plan)
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Fever greater than 101 requires evaluation
Mental health assessment related to chronic pain
Concerns for substance use disorder
Baseline hemoglobin (action plan)
Importance of primary care, especially dental
Genetic counseling
Pain Action Plan
Beta-Thalassemia
Thalassemia major or Cooley’s anemia
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Defined
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Prevalence
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A group of inherited disorders characterized by a Hb
beta-polypeptide chain abnormality
RBC destruction leads to compensatory
overproduction
Traits for thalassemia are more common (but not
exclusive) among those whose ancestors came from
Mediterranean countries, like Greece and Turkey, and
from Asia, Africa, and the Middle East.
Etiology
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Autosomal recessive
Carrier state = “thalassemia minor”
Clinical Manifestations
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Severe anemia
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Think of this as “worse” anemia than SCA
Fatigue
Frontal bossing
Maxillary prominence
Wide-set eyes
Flat nose
Hepatosplenomegaly
Jaundice, pallor
Growth retardation
Increase risk of fractures
Therapeutic Management
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RBC transfusions – a lot!
Chelation therapy (deferoxamine)
 Alloimmunization
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Splenectomy
 Can be “cured” by BMT/SCT
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Nursing Interventions
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Monitor Hgb level, iron level
Chelation therapy education
Psychosocial support
Vigilant primary care due to immunocompromise
(immunizations, prompt treatment of infections)
Genetic counseling
Vascular access management
Coagulation Disorders
Hemophilia
 Von Willebrand’s Disease
 Immune Thrombocytopenia
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Hemophilia
Factor VIII deficiency
(hemophilia A)
Factor IX deficiency
(hemophilia B)
Etiology
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X-linked recessive
Clinical Manifestations
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Presents with bleeding
Epistaxis
 Bleeding gums
 Hematuria
 Frequent bruising
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Muscle/joint bleeding (hemearthrosis--See
next photo)
 Circumcision bleeding (excessive)
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Diagnostic Evaluation
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Quantitative immunoelectrophorectic assay
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Factors VIII and IX assays
Platelet count
Fibrinogen level
PT
PTT
Management
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Administer intravenous factor (prophylactically)
Administer clot stabilizing medication
Avoid rectal temperature measurement or suppositories
(but constipation increases bleeding risk…)
Apply gentle pressure to superficial wounds
Assess LOC – know why! Think about other
signs/symptoms of bleeding.
Measure the affected joint/extremity, immobilization
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Active range of motion after acute phase as patient can limit for
pain as opposed to passive range of motion
RICE
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NSAIDS with caution—acetaminophen preferred
Family Teaching
Safe environment/injury prevention (think
growth and development)
 Appropriate activities (sports, etc.)
 Soft toothbrush/electric shaver
 Signs/symptoms of bleeding
 Administration of factor (may need a
permanent vascular access device)
prophylactically or emergently
 Genetic counseling
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Von Willebrand’s Disease
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Defined
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An underproduction or dysfunction of vonWillebrand’s protein
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Prevalence/Etiology
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Most common inherited bleeding disorder
Autosomal dominant inherited
Manifestations
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Carrier protein for coagulation factor VIII
Co-factor for the binding of platelets to damaged endothelial tissue
Prolonged bleeding
Variable severity – often undiagnosed until menorrhagia,
childbirth or after a surgical procedure (prolonged bleeding)
Management
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DDAVP
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Releases endogenous stores of vonWillebrand’s protein
Prophylactic factor infusions (HumateP)
Family education: communication, bleeding prevention
Immune Thrombocytopenia (ITP)
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Defined
 An acquired hemorrhagic disorder characterized by
thrombocytopenia (platelet count less than 150,000, a
purpuric rash, normal bone marrow, & the absence of
other identifiable caused of thrombocytopenia)
 Acute = less than 6 months
 Chronic = more than 6 months
Prevalence/Etiology
 Most common acquired bleeding disorder in children
 Unknown, often follows a viral illness, considered an
autoimmune process
Manifestations
 Sudden onset of bruising & petechiae
 Mucous membrane bleeding
Nursing Interventions
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Management
 Watchful waiting
Hmmm..maybe we need to do something?
 IVIG
Platelet count below 10,000 or
20,000 with spontaneous bleeding
 Steroids, anti-D antibody
 Splenectomy
 Platelet Transfusion
Soft toothbrush/electric shaver
Gentle pressure to superficial wounds
Vigilant follow up
 Monitor labs
Discuss home environment/safety
 Appropriate play activities
Steroid use education
 Risk for infection & tapering
Avoid medications that affect platelet function
Blood Product Transfusions
“Whole blood” rarely used
 Packed Red Blood Cells (PRBC)*
 Fresh Frozen Plasma (FFP)*
 Platelets*
 Cryoprecipitate
 Granulocytes, AT3, Albumin, factor VIII,
thrombin, etc. etc.
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PRBC Transfusions
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Intravenous access
Consent: purpose, risks/benefits, alternatives
Type/screen vs. type/crossmatch
Identification of recipient and donor
 2 LICENSED providers (MD, RN, transfusionist)
 This is the NUMBER 1 ERROR SOURCE
Assess patient for S/S of reaction (RN!)
 VS at beginning, 15 minutes in, and end of infusion—
most reactions are at start
Review Harding
TABLE 30-33 ACUTE TRANSFUSION REACTIONS
Transfusion Reactions
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Acute reactions
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Hemolytic
Febrile
Allergic
Transfusion-associated circulatory overload (TACO)
Transfusion-related acute lung injury (TRALI)
Temperature and electrolyte disturbances
Delayed reactions
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Hemolytic
Alloimmunization/antibody formation
Questions?