Hematological & Immunological Dysfunction

Hematologic Review
A 7 year old is receiving a transfusion of packed RBCs. After 45 minutes, he begins to
have chills, fever, tightness in chest, headache. Priority is: stop transfusion, maintain a
patent IV line with NS and new tubing, notify practitioner
(tight in chest is biggest concern because it affects airway)

The nurse is discussing the pattern of inheritance with family. Which statement about
autosomal dominance is correct? 50% chance of inheriting it if parent is affected

Blood has 2 components

1) Plasma
 90% water
 10% solutes

2) Cellular elements
 RBC
 WBC
 Platelets
 Normal Hgb
 1-3 day – 14.5-22.5
 2 mos – 9-14
 6-12 yr – 11.5-15.5
 12-18 yr Male – 13-16/Female – 12-16

Blood-forming organs
 Red bone marrow
 Lymphatic system

Hemostasis
Definition: process that stops bleeding when a blood vessel is injured
Depends on 3 things

1) Vascular influence:
 Local Vasoconstriction
 Compression of blood vessels by extravasated blood,
 Release of von Willebrand factor,
 Presence of collagen in exposed subendothelial cells that acts as site
for platelet adhesion
2) Platelet role: Plts provide a plug at site, release variety of chemicals to
stimulate VC and vessel repair
3) Clotting factors (will not ask details, just know what they respresent)
 Intrinsic pathway: PTT
 Extrinsic pathway: PT

IRED BLOOD CELL DISORDERS

ANEMIA
Definition: reduction of RBC volume or Hgb concentration to levels below normal
  Insidious onset but children adapt readily
 Smaller than individual, smaller the circulating blood will be

Basic causes
1. Excessive blood loss
 Loss of plasma and formed elements
2. Increased destruction of RBCs
 Defect in RBC that shortens life span of it
 Factors cause hemolysis in normal RBCs
3. Impaired or decreased rate of RBC production
 Bone marrow failure
o Replacement of bone marrow by fibrosis or neoplastic cells
o Depression of bone marrow (from radiation, chemicals, drugs)
o Interference with bone marrow activity
 Deficiency of essential nutrients
o Iron
o Amino acids; vitamin B6 and B12, C; folic acid; copper

Patho/ S&S:

All related to decrease in O2 carrying capacity of blood and decreased amount

available to cells—red blood cell regulation controlled by tissue hypoxia
 Muscle weakness
 Fatigue, headache
 Pale
 CNS manifestations
 Growth retardation
Body responds by: increased heart rate/CO, murmur, cardiac failure during stress

Dx
Screening, DIC (disseminated intravascular coagulation—blood clotting mechanisms
are slowed, PTT will be elevated, shows in septic shock episodes, easy to diagnose,
platelets don’t function)
o Give FFP (fresh frozen plasma) to raise PTT
AAFP (all infants screened at least once), AAP (Hgb/HCT measured once between 9-
12 months, 1-5 years, 5-12 years, 14-20 year)

Hx & physical, CBC, look at lips for paleness

Treatment/Nursing

 Treat underlying cause

 Replacement of blood or nutrient [Review blood transfusion care]
 Supportive care
o O2, IVF, bed rest
o Iron supplements
 Good interview
 Prep of child for lab tests
 Observe for complications
o Heart failure, blood incompatibilities, infection
Iron Deficiency Anemia
Need for iron to produce hemoglobin is not met, oxygen carrying ability reduced

Etiology
 Decreased supply
 Impaired absorption
 Blood loss
 Increase need

S/S

Same as anemia plus:

 “Milk baby”—low SES moms give cows milk sooner than they should and kids
become anemic
o Milk is poor source of iron, most formulas are fortified
 Infants may be overweight, HCT can be around 45
 Leak plasma proteins
o Edema, linear growth retardation, delayed sexual maturation,
cognitive deficiencies

Dx: normal or low RBC, low Hgb, iron studies (serum iron concentration, total
iron-binding capacity)
CBC

Treatment/Nursing
 Prevention
o Use only breast milk or formula during first 12 months
o Iron supplements
o Need iron-rich foods
 Medications
o Ferrous sulfate
o *liquid iron preparation: tarry, green stool
 Parent education
o Diet
o Administration of iron
o Follow up
When the hemoglobin level falls sufficiently to produce clinical manifestations of anemia,
the patient experiences: tissue hypoxia

Sickle Cell Anemia

Disease versus Anemia

 Sickle Cell Disease (SCD) – group of hereditary disorders in which there is
abnormal sickle hemoglobin, either S, C, glutamic acid substituted for lysine,
sickle cell trait and beta-thalassemia trait.
o Most common
o Found primarily in black and whites of Mediterranean descent
o Autosomal recessive
 Sickle Cell Anemia (SCA) – only one of those diseases in which normal adult
Hgb is partly or completely replaced by HgS
 Patho/S&S
 Problems d/t obstruction by sickled RBCs and destruction of RBCs, leads
to ischemia (pain)
 Destruction of RBC leads to hypoxia
o Vaso-occlusion due to obstruction/destruction
o Spleen enlarges, impaired function
o Liver same
o Kidney same
o Bone changes
o CNS changes
o Heart problems
o Exercise intolerance
o Chronic leg ulcers
o Joint pain from micro-infarcts, risk for stroke

Sickle cell crisis

o Acute exacerbation of symptoms
o Vaso-occlusive crisis most common, not life threatening. Results
from sickled cells obstructing blood vessels—very painful

Dx
o Family history
o CBC (anemia)
o Sickle turbidity test
o Hgb electrophoresis

Treatment/Nursing
Aims of treatment
1. Prevent sickling phenomenon
2. Treat medical emergency of sickle cell crisis
Most care is symptomatic due to lack of cure
1. Pain relief
o Severe—opioids (morphine, large dose)
o Mild-moderate: Tylenol or ibuprofen, may add codeine
o Heat to site
o Psych support
2. Adequate hydration
o Prevent sickling
o Diluting blood
o Enuresis
3. Improve oxygen utilization
o Bedrest, then breaks
o No oxygen unless severely hypoxic, since it doesn’t prevent sickling
and can aggravate anemia by depressing bone marrow
4. Other care
o Electrolyte replacement (treat metabolic acidosis)
o Blood replacement
o Keep infection free (prophylactic antibiotics like penicillin, evaluate if
febrile)
 Prevents pneumococcal infection from 2 months to 5 years
 o Support/education

Prognosis:
 Under 5 years—higher risk
o Death due to overwhelming infection
 Crisis tends to become less severe as children get older
Aplastic anemia: RBC, WBC, Hct all are reduced

Defects in Hemostasis
Hemophilia

 Refers to group of bleeding disorders where there’s a deficiency of a factor

necessary for coagulation of the blood
 Hemophilia A or classic hemophilia is most common—no cure
 Factor VIII - classic
 Most cases are X-linked recessive

 3 classifications
 Severe – have 1% factor VIII activity: spontaneous bleeding without trauma
 Moderate – have 1-5% activity: bleeding with trauma
 Mild – 5-50%: with severe trauma or surgery
Symptoms:
 Spontaneous bleeding (upper airway, intracranial, GI tract)
 Hemarthrosis (blood in joints)
 Seriousness depends on site

Diagnosis:
History
X-linked inheritance
Lab tests

Treatment/Nursing

 Replacement missing clotting factor

 Steroids
 Avoid NSAIDS and aspirin
 Regular diet and exercise
 Education
o Administering factor
o Signs of internal hemorrhage
o Prevention of bleeding
 Family support

Idiopathic Thrombocytopenic Purpura (ITP)

 Acquired hemorrhagic disorder char. by

 Excessive destruction of platelets (thrombocytopenia)
 Purpura (discoloration caused by petechiae beneath the skin)
 S/S
 Easy bruising
 Bleeding
 Internal hemorrhage
 Hematomas over lower extremities

Dx
CBC – platelet count

Treatment/Nursing
 Self limiting, supportive care
 Corticosteroids
 IV gamma globulin or anti-D antibody
 Splenectomy

→ Review Disseminated Intravascular Coagulation (DIC)

Immunologic Review

 Function of immune system is to recognize “self” from “non-self” and initiate

responses to eliminate non-self or foreign substances (antigens).
 Skin is first line of protection
 Primary lymphoid organs
 Secondary lymphoid organs
 Have specific and nonspecific immune defenses (see chart)

Non - activated with any exposure to a foreign substance

Specific - can recognize antigen
Human Immunodeficiency Virus

Etiology
 Human immunodeficiency virus (HIV) that attacks immune system by destroying T
lymphocytes.

Modes of transmission
 Maternal-infant (occurs in 1 out of every 4 births to HIV positive women)
o Intrauterine
o Intrapartum (during L&D)
o Postpartum
 Maternal factors that determine transmission:
o Low CD4 count
o High viral load

 Blood transfusions - rare in kids

  Hemophilia
 Sexual contact
 IV exposure (i.e. drugs)

Review patho & S/S.

Diagnosis
 ELISA
 Polymerase chain reaction (PCR)
 Antigen p24

Treatment/Nursing

 Medication regimes

 Prophylactic meds

 Immunizations….

 NO breastfeeding

 Nutrition

Complications

Opportunistic infections
 PCP
 CMV
 Herpes simplex
 MAI (mycobacterium avium
 Candidiases

Prevention
In HIV positive moms - AZT during pregnancy, labor & delivery, and to the infant for first 6 weeks of
life has greatly reduced vertical transmission

Education

Autoimmune Disease Processes

Systemic Lupus Erythematosus (SLE)

 Chronic, inflammatory disease of collagen or supporting tissues of body

 Course of remissions and exacerbations

Etiology
Unknown
 Autoimmune response to some event such as stress, infection, extreme
fatigue, drugs
 Runs in families

S/S

 Insidious onset: low grade fever, arthritis, generalized aching, rash

(characteristic butterfly rash on face)
 Most children have skin disease (rash, itchy, like sunburn)
 Raynaud phenomenon: sensitivity to cold, esp. in feet and hands
 Cyanosis
 Ulcers in cracked, dry skin
 May have alopecia
 Renal involvement
 Neuropsychiatric

Dx
 Established by 4 of 11 criteria

Treatment/Nursing
 Goals of treatment
 Reverse autoimmune and inflammatory processes
 Prevent exacerbations and complications

 Medications
o Corticosteroids
o Antimalarials
o NSAIDs
o Others

 Education about disease, consequences, and meds.

 American Lupus Society and Lupus Foundation of America both good
resources
 Compliance with treatment
 Body image
 Need excellent skin care
 Frequent eye exams
  Family support

Juvenile Idiopathic Arthritis (JIA)

 Inflammatory disease with unknown inciting agent

Patho
 Chronic inflammation of synovium with joint effusion and eventual erosion,
destruction, and fibrosis of articular cartilage. Adhesions in joint surfaces
occur.

S/S

 Stiffness, swelling, and loss of motion of affected joints

 May be tender to touch, or not
 Limited motion d/t muscle spasm and joint inflamm. early on, then by soft
tissue contracture.
 Morning stiffness characteristic

Dx

Treatment/Nursing
 No cure
 Major goals of therapy
 Control pain
 Preserve joint function
 Minimize effects of joint deformity
 Promote G&D
 Medications
o NSAIDS
o Antirheumatics

 Physical therapy
 Nonpharmacological management
 Health promotion
 Normal G&D
 Family support
Rheumatic Fever

 Autoimmune reaction to group A, beta-hemolytic strept pharyngitis

 Self-limiting disease that involves joints, skin, brain, and heart**

Patho & S/S

 Lesions formed called Aschoff bodies

 Cardiac
 Involves endocardium, pericardium and myocardium
 Murmur
 Tachycardia
 Signs of CHF
 Cardiomegaly

 Polyarthritis
 Edema, inflammation, and effusions in joint tissue

 Erythema marginatum
 red macule

 Subcutaneous nodules
 Small and non-tender

 Chorea
 sudden, aimless, irregular movements of extremities, involuntary facial
grimaces

Dx
 Based on guidelines set by American Heart Association

Treatment/Nursing
Goals of treatment
 Eradicate hemolytic strept
 Prevent permanent cardiac damage
 Treat symptoms
 Prevent recurrences

 Medications -
o Pencillin
o Salicylates

Goals of Nursing
 Encourage compliance with medications
 Facilitate recovery from illness
 Provide emotional support