The Child with Hematologic Disorders 4.

Red blood cells

- Also called erythrocytes
Contents - Life span of 120 days
1. Review of the hematologic system - Transports oxygen & CO2 to & from the
2. Disorders of the red blood cells tissues
a. Anemia - Hemoglobin
i. Iron deficiency anemia o Molecule responsible for carrying O2
ii. Sickle cell anemia to the tissues
iii. Thalassemia o Somewhat affected by age
iv. Aplastic anemia ▪ 15-20 g/dL in neonates
3. Defects on hemostasis ▪ 12-15 g/dL in >2 mos
a. Hemophilia o Heme = iron
4. Neoplastic disorders o Globin = protein that attracts oxygen
a. Leukemia - Hematocrit
b. Lymphoma o Times 3 the amount of hemoglobin
i. Hodgkin’s disease o Normal 35-45%
ii. Non-Hodgkin’s lymphoma o Affected by hydration
▪ Elevated if px is dehydrated
▪ Decreased if fluid overload
The Hematologic System - Bilirubin is the waste product
- Interrelated w/ immune system in fighting off - Decreased RBC is manifested by fatigue since
infection there is an impairment in the oxygen-carrying
- Rises from the bone marrow capacity of the blood
- Stem cell - Erythropoietin
o Also called as pluripotent stem cells o Hormone produced by the kidney
o Has the ability to transform into any o Stimulates the production of RBCs in
type of cell the bone marrow
- Hematopoiesis - Lead can replace iron in the cells, causing
o The process of blood formation toxicity
o Occurs mainly in the liver and spleen 5. White blood cells
during intrauterine life - Also called leukocytes
o Red bone marrow is the main site - Fights off infection
after birth - Types
▪ As the child approaches o Granulocytes
adolescence, bone growth ▪ Neutrophils
ceases • Fight off acute
▪ Bone marrow in many bones infections or invading
can’t fr microorganisms
1. Bone marrow • Phagocytosis effect
- Red bone marrow is the main site of (engulfing the foreign
hematopoiesis after birth body)
- As the child approaches adolescence, the red • Remove dead cells
bone marrow can’t form blood cells since it has ▪ Eosinophils
transformed into the yellow bone marrow • Acts in allergic
o This is due to the cessation of bone reactions
growth • Defends against
- Creates the primitive form of blood cells called parasites & lung &
the blast cells skin infections
o Blast cells are the most immature form ▪ Basophils
of blood cells • Releases heparin &
o The precursor to RBCs, WBCs, & histamine
platelets • Protects against
2. Yellow bone marrow infections via
- Is made of fat deposits inflammation
3. Blood
• Also known as mast
- Composed of plasma & cells
cells
o Plasma is the fluid portion
o Agranulocytes
o Blood cell types originate from blast
▪ Lymphocytes
cells
• Main cells that fight
▪ RBC
infections
▪ WBC
• “natural killer cells”
▪ Platelets
 • T cells & B cells - Physical examination
o B cells produce o Cardinal S&S
immunoglobulins ▪ Fatigue
▪ Monocytes ▪ Headache
• Help w/ neutrophils ▪ Vertigo
• Form macrophages in ▪ Irritability
the body tissues ▪ Depression
6. Platelets ▪ Anorexia & weight loss
- Also called thrombocytes ▪ Bleeding, bruising
- Coagulation or clotting process ▪ Heavy menstrual flow
o Adhere to one another & plug holes in ▪ Epistaxis
the tissues where there is bleeding ▪ Recurrent infections
- Releases serotonin ▪ Bone & joint pain
o Vasoconstrictor o Vital signs
o Decreases blood flow to the injured ▪ Tachycardia
area ▪ Tachypnea
- Normal: 150 – 450 x10^9 g/dL o Inspection
o Low levels manifest as abnormal ▪ Bruising
bleeding ▪ Pallor or flushing
7. Antibodies ▪ Jaundice (skin & eyes)
- Immunoglobulin A (IgA) ▪ Purpura
o Found in the linings of respi & GI tract • Blood spots or skin
o Also found in breastmilk hemorrhages
o Prevents large foreign bodies from • <4 mm are called
being absorbed in the GI tract petechiae
o Prevents adherence of pathogens to • >1 cm are called
mucosal cells ecchymoses
- Immunoglobulin G (IgG) ▪ Pruritus
o Most frequently occurring antibody in ▪ Cyanosis
plasma ▪ Retinal hemorrhage
o Neutralizes bacterial toxins ▪ Blurred vision
o Activates phagocytosis ▪ Lymphadenopathy
- Immunoglobulin M (IgM) ▪ Tachypnea, orthopnea, or
o Agglutinates antigens dyspnea
o Lyses cell walls ▪ Impaired thought process
o Discovered early in the course of an ▪ Lethargy
infection in the bloodstream ▪ Bone or joint swelling
- Immunoglobulin E (IgE) ▪ Hematuria
o Involved in immediate hypersensitivity ▪ Heavy menstruation
reactions o Palpation
o Bound to mast cells on tissue surfaces ▪ Decreased CRT
o Associated w/ allergy & parasitic ▪ Tenderness over lymph nodes
infections ▪ Hepatomegaly
- Immunoglobulin D (IgD) ▪ Splenomegaly
o Found in plasma ▪ Decreased muscle mass
o Receptor that binds antigens to ▪ Bone & joint tenderness
lymphocyte surfaces o Auscultation
▪ Heart murmurs
Nursing Process ▪ Abnormal lung sounds
1. Assessment - Diagnostic tests
- Health history o CBC w/ differential count
o History of present illness ▪ One of the most general
o Prenatal & family history screening test for hematologic
▪ Blood type & immune system problems
▪ Prematurity ▪ Differential count determines
▪ Low birth weight percentage of WBC present
▪ Diet & diet behaviors ▪ RBC count is the total # of
▪ Menstrual flow RBCs found in a cm3 of blood
▪ Exposure to infections ▪ WBC count is the total # of
▪ Fam history of bleeding circulating leukocytes
disorders
 ▪ Hemoglobin helps diagnosis o Chronic blood loss
of anemia ▪ Heavy menstrual flow
▪ Hematocrit measurement of ▪ GI bleeding
the state that PRBCs & o OTC drugs
measurement of RBC mass ▪ Long-term use (>3 mos) of
▪ MCV (mean corpuscular aspirin
volume) is the measurement o Celiac disease
of individual RBC size o Surgical removal of a part of the GI
▪ MCH (mean corpuscular tract
hemoglobin) measures the o Parasite infestations
average weight of hemoglobin ▪ Tapeworms
in the RBC ▪ Round worms
▪ MCHC (mean corpuscular - Body’s capacity to transport O2 is impaired
hemoglobin concentration) - IDA during pregnancy is associated w/ low
measures the average fetal BW & preterm birth
concentration of hemoglobin - Sa
in the RBC o Folic acid
o Bone marrow biopsy ▪ Used as supplementation
▪ Aspiration of bone marrow ▪ Synthetic
sample ▪ Can help prevent neural tube
▪ Aids in diagnosis of aplastic defects
anemia, leukemia, etc. o Folate
▪ Sedation is needed ▪ Naturally found in food
(Benadryl) ▪ Best source of vitamin B9
▪ Bleeding & hematoma ▪ Can help prevent neural tube
formation may occur upon defects
aspiration ▪ Do not overcook vegetables to
2. Nursing Diagnosis get the most folate
- Risk for deficient fluid volume - S&S
- Activity intolerance o Fatigue
- Fatigue o Pallor
- Risk for infection o Hypoxia
- Ineffective tissue perfusion o Weakness
- Risk for tissue injury o Activity intolerance
- Delayed growth & development o Poor weight gain
3. Planning o Overweight
4. Implementation o Infections
5. Evaluation o Hemorrhage
o Epistaxis
Disorders of the Red Blood Cells o Bruising
Anemia o Mucosal bleeding (oral, GI, vagina)
- Occurs when there is a low RBC count, - Risk factors
hematocrit, or hemoglobin o Prematurity
- Results in poorly oxygenated cells and - Complications (neural tube defects):
malfunction of the body organs & systems o Anencephaly
- Causes ▪ Parts of the brain & skull are
o Damage in the bone marrow missing
o Suppressed production of o Spina bifida
erythropoietin in the kidney ▪ Spine & spinal column doesn’t
o Nutritional component needed to form develop properly during
blood (iron) is insufficient intrauterine development
o Blood cells produced are poorly ▪ There is a gap in the spin
structured o Encephalocele
▪ Sac-like protrusion of the brain
Iron Deficiency Anemia & its membranes through an
- Hemoglobin synthesis is deficient opening in the skull
- Amount of hemoglobin circulating in the blood - Nursing management
is inadequate o Educate the parents about the diet &
- Caused by insufficient iron in the blood (heme treatment regimen:
component of hemoglobin is primarily iron) ▪ Prescribed iron supplement
o Inadequate iron intake therapy
o Malabsorption of iron in the GI tract
 ▪ Iron fortified milk, cereal, or
formula
▪ Avoid fresh cow’s milk to
avoid allergies & GI irritation
▪ Food rich in iron
▪ Iron therapy may cause
constipation & dark green or
black stools
▪ Safe storing of iron
▪ Take iron supplement w/
vitamin c sources to aid iron
absorption
o Administer vitamin B12 IM to treat
deficiency due to failure of gastric
mucosa to secrete the intrinsic factor
needed to absorb vitamin B12
▪ Injected into the ventrogluteal
muscle
▪ Using z-track method
o Assist in blood transfusion (PRBC)
o Oxygen supplementation if hypoxia is
presence
o Give liquid iron w/ a straw to avoid
contact w/ & staining the teeth
o Avoid substances that impair iron
absorption
▪ Antacids
▪ Milk
Sickle Cell Disease
- Caused by a mutation in the hemoglobin
- Results in the sickle form of the RBC when it is
deoxygenated
o RBCs have a shorter lifespan (20
days)
o Sickled RBCs are stiff and sticky
which may cause an obstruction in
smaller blood vessels
o Obstruction causes hypoxia and
necrosis of the affected tissue
- Autosomal recessive disorder
o Genes need to be inherited from both
parents
o Only inheriting one gene result in a
Sickle Cell Trait where a person is
asymptomatic and a carrier
▪ Symptoms only present when
the O2 demands of the body
increases
o More common in Africans
- Factors that trigger sickling
o Hypoxia
o Increase in blood viscosity
▪ Due to an increased
concentration of sickle cells
- Clinical manifestations
o Acute pain
o Swollen, tender hands & feet
o Hepatomegaly
o Splenomegaly
o Jaundice
o Fatigue
o Fever
o Hematuria
o Swollen abdomen
o Painful erection
o Organ damage
o Pooling of blood
o Growth retardation
o Crisis episodes
▪ Caused by obstruction of
blood flow due to sickled cells
or vaso-occlusion
o Sequestration
▪ Aplastic crisis due to an
extreme drop of RBCs
o Megaloblastic anemia
▪ Excess need for folic acid or
vitamin B12
o Hyper hemolytic crisis
▪ Rapid RBC destruction
- Nursing diagnoses
o Pain rlt joint swelling
o Knowledge deficit rlt disease,
treatment, & prevention crisis
o Ineffective tissue perfusion rlt vaso-
occlusion
- Nursing management
o Assess for pain q2-4h
▪ Administer prescribed
analgesics
▪ Pain scale <5 is not a priority
▪ Monitor for effectiveness
▪ Apply warm compress to joint
as prescribed
▪ Avoid cold compress bc it may
cause vasoconstriction
o Provide rest periods
▪ Encourage bed rest
o Prevent dehydration
▪ Administer fluids
▪ Increase OFI unless
contraindicated
o Assist in BT of PRBC if prescribed
o Educate parents on the disease
process & the prevention of crises
▪ Sickle cell trait
▪ Sickle anemia
▪ Sickling
o Prevent infection
▪ Administer prescribed
antibiotics
▪ Encourage immunization
o Administer oxygen supplementation
Thalassemia
- Autosomal recessive disorder wherein there is
an impaction of the alpha & beta polypeptide
chains in the hemoglobin A
- Characterized by a deficient synthesis of
specific globulin chains of the hemoglobin
molecule
 o Results in damaged RBCs o Thrombocytopenia
o Alpha thalassemia is a deficiency in - Also characterized by bone marrow hypoplasia
alpha globulin chains - Types
o Beta thalassemia is a deficiency in o Primary
beta globulin chains ▪ Congenital
- Overproduction of RBCs occur to compensate o Secondary
for hemolysis ▪ Acquired due to autoimmune
o Folic acid deficiency may occur due to disease
increased demands of the bone - Clinical manifestations
marrow o Lack of RBCs
- Types acc to presentation of symptoms ▪ Pallor
o Thalassemia major ▪ Lethargy
▪ Moderate to severe anemia ▪ Tachycardia
▪ Clinical manifestations present ▪ Shortness of breath
around 6 months of age ▪ Decreased hemoglobin
o Thalassemia minor • <5-6 g / 100 mL in
▪ Mild to moderate anemia children
▪ May be asymptomatic o Lack of WBCs
▪ Commonly goes undetected ▪ Recurrent infection
- Clinical manifestations o Lack of platelets
o Early signs ▪ Abnormal bleeding
▪ Insidious onset ▪ Bruising
▪ Anemia - Prognosis
▪ Unexplained fever o Depends on the extent & duration of
▪ Poor feeding the decreased blood cell production
▪ Poor weight gain o Also depends on the client
▪ Enlarged spleen - Diagnostic evaluation
o Later signs o Routine CBC
▪ Chronic hypoxia o Peripheral blood smear
▪ Damage to liver, spleen, ▪ Reveals pancytopenia
heart, pancreas, & lymph o Bone marrow aspiration & biopsy
glands from - Nursing Management
hemochromatosis o Prevent infection
▪ Slight jaundice or bronze skin o Assess for abnormal bleeding
color o Administer prescribed medication &
▪ Thick cranial bones w/ blood products
prominent cheeks & flat nose ▪ Antilymphocyte globulin
▪ Growth retardation (ALG) or Anti-thymocyte
▪ Delayed sexual development globulin (ATG)
- Treatment • Suppresses T cell-
o Bone marrow transplant dependent
o High-iron diet autoimmune
- Nursing management responses w/o
o Prevent infection causing bone marrow
o Avoid activities that may increase the suppression
risk for fractures & injury ▪ Cyclosporine
o Promote rest • Given if ALT or ATG
o Adhere to prescribed diet therapy is ineffective
o Blood transfusion
o Diet as tolerated
Aplastic Anemia ▪ Avoid forcing food if N/V is
- Caused by damage to the bone marrow present
- Bone marrow may be damaged due to: ▪ Liquid or soft diet may be
o Infections (HPV, hepatitis) ordered if N/V is present
o Radiation o Meticulous oral care
o Medications
▪ Immunosuppression
medications used to eliminate Defects on Hemostasis
cancer cells Hemophilia
- Characterized by pancytopenia
o Anemia
o Granulocytopenia
- X-linked recessive congenital bleeding ▪ Spontaneous prolonged
disorders characterized by a deficiency in bleeding
specific coagulation protein • Gums
- Found dominantly in males • Circumcision
- Common types: • Umbilical cord
o Hemophilia A - Clinical manifestations
▪ Classic hemophilia o Epistaxis
▪ Caused by a deficiency of o Hematoma
clotting factor VIII, which is o Bleeding
necessary for the formation of o
thromboplastin - Nursing Management
▪ Caused by deficiency of o Assess for acute or chronic bleeding
antihemophilic globulin C, ▪ Assess skin, joint, muscles,
which is necessary for blood gums, rectum, mouth
clotting o Administer the missing clothing factor
o Hemophilia B o Administer Desmopressin to children
▪ Christmas disease w/ mild hemophilia A
▪ Less common type o Prevent or minimize bleeding
▪ Factor IX deficiency o Provide support
o Hemophilia C o Provide child & family teaching
▪ Deficiency of factor XI
o von Willebrand Disease
▪ Deficiency in von Willebrand Neoplastic Disorders
factor, a protein necessary for Leukemia
platelet adhesion - Most common cancer in children
- Common in Africans - Uncontrolled production of deformed or
o May be a genetic adaptation for immature WBCs
protection against malaria - Rapid increase in lymphocytes causes
- Etiology crowding
o Results in decrease of RBC & platelet
production
- Fatigue, susceptibility to infection, &
increased bleeding results from the decreased
normal WBCs, RBCs, & platelets
- Peak onset of 3-5 y/o
- Types
o Acute lymphoid leukemia (ALL)
▪ Most common type in children
• Greatest in 2-6 y/o
▪ More common in males
▪ Involves WBCs from lymphoid
▪ Most curable type
o Acute myelogenous leukemia (AML)
▪ Most common form of
leukemia
▪ More common in adults
- Assessment findings ▪ Curable rate is only 40%
o Mild ▪ Involves WBCs from myeloid
▪ Hemophilia (factor level of 5- • Granulocytes
50%) • Monocytes
▪ Prolonged bleeding only if ▪ Curable rate is only 40%
injury is present - Clinical manifestation
o Moderate o Weight loss
▪ Hemophilia (1-5%) o Anorexia
▪ Prolonged bleeding during o Vomiting
trauma or surgery o Abdominal pain
▪ Episodes of spontaneous o Bone pain
bleeding o Petechiae
o Severe o Purpura
▪ Factor level of <1% o Hematuria
o Epistaxis
 o Tarry stools o Nerve irritation in the extremities
o Easy bruising o Non-productive cough
- Diagnostic evaluation ▪ Occurs in 50% of patients
o CBC o Pruritus
o Bone marrow aspiration ▪ Systemic manifestation
o Lumbar puncture o Engorgement of neck veins
▪ Insertion of a needle in the o Edema of face, neck, & right arm
lower spine o Urinary retention
▪ Headache & back pain may o Constipation
occur after the procedure o Compression of the cord
- Nursing Management - Stages
o Prevent infection o Stage I
▪ Pseudomonas is the most ▪ Involves single lymph node
common infectious agent region or lymphoid structure
o Prevent bleeding & injury • Spleen
▪ Use toothbrushes w/ soft • Thymus
bristles • Waldeyer’s ring
o Promote energy conservation o Tonsils
▪ Uninterrupted sleep & rest o Adenoids
o Relieve anxiety o Other
o Promote normal growth & lymphoid
development tissues in
o Promote positive body image URT
o Promote family coping o Stage II
o Prednisone therapy may be prescribed ▪ Involves 2 or more lymph
▪ May cause a moon-shaped node regions on the same
appearance side of the diaphragm
▪ Assure px that side effects ▪ E.g. mediastinum is a single
only temporary site, hilar lymph nodes are
lateralized
o Stage III
Lymphoma ▪ Involves lymph node
- Tumors in the lymphoid system regions/structures on both
o Thymus sides of the diaphragm
o Bone marrow ▪ III1
o Lymph nodes • w/ or w/o involvement
o Spleen of splenic, hilar,
o Tonsils celiac, or portal nodes
o Intestinal lymphoid tissues ▪ III2
- Occur more frequently in males • w/ involvement of
1. Hodgkin’s Disease para-aortic, iliac, or
- Known as cancer of the lymph or lymphoma mesenteric nodes
- Incidence o Stage IV
o Most common in adults ▪ Involvement of extranodial
o Most common in 15-19 y/o sites
- Believed to be caused by Epstein-Barr virus - Treatment
(EBV) o Surgical removal of the tumor
o Associated w/ immunosuppression & ▪ Usually indicated for stage 1
organ transplantation o Chemotherapy
- Mechanism of growth & spread is unknown o Radiotherapy
- Risk factors ▪ May be combined w/
o Family history (1st degree) chemotherapy
- Pathophysiology 2. Non-Hodgkin’s Lymphoma
o Cancerous transformation occurs from - Group of malignancies w/ a common origin in
a particular site in the lymph node the lymphoid cells
o As growth continues, the entire node - 7x more common than HD
becomes replaced w/ zones of - Incidence
necrosis obscuring the normal nodular o Most common in childhood (< 15y/o)
pattern - Risk factors
- Clinical manifestation o No dietary, hereditary, or ethnic risk
o Painless lymphadenopathy factors involved
▪ Clavicles, cervical
 o Immunosuppression Review Questions
▪ Autoimmune disorders 1. Why should a client with SCA be concerned if
▪ Infectious, physical, & they marry a person w/ the sickle cell trait?
chemical agents 2. What defect is most commonly caused by
- Major histopathologic patterns anemia?
o Nodular a. Increased RBC count & blood
▪ Well-differentiated viscosity
lymphocytes b. Decreased hematopoietic system &
▪ Nodal & extra nodal sites hyperactivity
o Diffuse c. Increased presence of abnormal
▪ Cell aggregate is not evident, hemoglobin
unlike the nodular pattern d. Decreased capacity of blood to carry
- Pathophysiology O2
o Abnormal proliferation of neoplastic 3. Common symptoms of hemophilia in children
lymphocytes include _________, _________, __________,
o Cells remain fixed at 1 phase of _________.
development & continue to proliferate
o Both T & B lymphocytes mature in the
lymph nodes Answers
o Mechanical obstruction of enlarged 1. Since there is a possibility that their offspring
lymph nodes occur may be a carrier of the sickle cell trait.
- Clinical manifestations 2. D.
o Localized or generalized painless 3. Bleeding
lymphadenopathy Hemarthrosis
▪ Common sites: Joint swelling, pain, & limited ROM
• Cervical Altered neurologic status (caused by
• Axillary intracranial bleeding)
• Inguinal
• Femoral
- Nursing diagnoses
o Altered nutrition rlt anorexia in
chemotherapy
o Risk for ineffective management of
therapeutic regimen
- Treatment
o Combination chemotherapy for
shrinking the tumor & remission
o Cyclophosphamide
o Doxorubicin
- Complications
o Metastasis of the tumor to other parts
of the body
o Thrombocytopenia due to
chemotherapy