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NCM112j - Hematology

Module 4: Hematologic System


HEMATOLOGIC SYSTEM
 WHITE BLOOD CELL
 RED BLOOD CELL
 PLATELET
 PLASMA ss
BLOOD: LIVING FLUID

Functions of Blood
1. Provides the Body's Cells with
Oxygen and Removes Carbon
Dioxide
2. Blood Transports Nutrients and
Hormones LYMPHOID STEM CELLS
3. Regulates Body Temperature  Produces lymphocytes - some will
4. Inflammatory and Immune enter in our blood stream, but most
Responses will move through our lymphatic
5. Blood Brings Waste Products to the system.
Kidneys and Liver  25% of new lymphocytes remain s in
the bone marrow and become B -
 Blood is composed of 55% plasma CELLS.
and 45% “formed elements,”  The other 75% travels to our Thymus
including red blood cells, white blood and becomes T- CELLS
cells, and platelets.  T- CELLS include:
a. Effector Cells
b. Memory Cells
 B-Lymphocytes recognize antigens
and become Plasma Cells that
produces antibodies to fight antigens.
 3 Types of T- CELLS
1. Cytotoxic T- Cells or Killer T-Cells
2. Helper T- Cells
3. Regulatory T- Cells
Bone Marrow
 One of the largest organ of the body CELLULAR COMPONENTS
 Consist of islands of cellular Myeloid Stem Cells
components (red marrow), separated • ERYTHROCYTES (RBC)
by fat (yellow marrow) • LEUKOCYTES (WBC)
 Produces 175 billion erythrocytes • THROMBOCYTES (PLATELETS)
 70 billion neutrophils (a mature type
 of WBC). ERYTHROCYTES (RBC)
 175 billion platelets each day. • Red blood cells, or erythrocytes
(erythro- = “red”; -cyte = “cell”),
specialized cells that circulate
through the body delivering oxygen

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Module 4: Hematologic System
to other cells, are formed from stem • Function: clot formation
cells in the • Lifespan: 7-10 days
bone marrow. • Stored in the spleen and destroyed
• Normal erythrocytes is an anucleated there by phagocytosis.
biconcave disk that resembles a
softball compressed between two
fingers.
• Hemoglobin
• Heme - Each heme group bears an
atom of iron
• Globin- Protein molecules made up
of two alpha and two beta chains
• Oxygen binds in to hemoglobin in the
lungs is carried as Oxyhemoglobin in
arterial blood.
• The average life span is 120 days and
is eliminated in the liver and spleen. Plasma and Plasma Proteins
• Iron, B12, Folic acid, Vit. B6 • Plasma, the liquid component of
(Pyridoxine). blood, comprises 55 percent of the
total blood volume.
LEUKOCYTES (WBC) • Plasma is composed of 90 percent
• White blood cells, also called water with antibodies, coagulation
leukocytes (leuko = white) factors, and other substances such as
• Account for only about 1% of the electrolytes, lipids, and proteins
blood. required for maintaining the body.
• 4500-11,000/mm3
• Primarily involved in the immune
response to identify and target
pathogens, such as invading bacteria,
viruses, and other foreign organisms.
• White blood cells contain nuclei; they
are divided into granulocytes (e.g.
neutrophils, eosinophils, and
basophils) and agranulocytes (e.g.
monocytes and lymphocytes).
• White blood cells can become
macrophages at sites of infection or
inflammation.
• Lymphocytes make up the majority of
the cells in the immune system; they
include B cells, T cells, and natural
killer cells.

THROMBOCYTES (PLATELETS) Reticuloendothelial System


150,000 – 450,000 /mm3
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Module 4: Hematologic System
• Composed of special tissue three types of cells suspended in fluid
macrophages called plasma: white blood cells
• Specialized cells capable of (WBCs), red blood cells (RBCs), and
Phagocytosis platelets (PLTs).
• Component of many other organs • They are produced and mature
within the body (spleen, lymph primarily in the bone marrow and,
nodes, lungs, kidneys and liver). under normal circumstances, are
SPLEEN released into the bloodstream as
• Function: as blood filtration system needed
and reservoir • When to Get Tested? As part of a
• Vascular, bean shaped; beneath routine medical exam; when you
diaphragm, behind and to the left of have signs and symptoms that may
stomach ▪ 75% made of red pulp be related to a condition that affects
• White pulp consisting of B and T blood cells; at regular intervals to
lymphocytes monitor treatment or when you are
• Sequesters newly released receiving treatment known to affect
reticulocytes from the marrow. blood cells

HEMOSTASIS
• The balance between clot formation
(thrombus) and clot dissolution or
fibrinolysis.
• The process of wound being closed
by clotting, starts when blood leaks
out of the body.

LABORATORY TESTS FOR BLOOD


DISORDERS
Hematologic Studies
Complete Blood Count (CBC)
• measures the number of red blood
cells, white blood cells, and platelets
in the blood.
• Complete blood count (CBC) is a
test that evaluates the cells that
circulate in blood. Blood consists of
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Module 4: Hematologic System
• surgical excision of an active lymph
node or needle aspiration for
histological examination.
• cervical, supraclavicular, axillary, or
inguinal
NURSING RESPONSIBILITY
• consent
• local anesthetic agent
• For excisional biopsy: instruct the
patient to restrict food from midnight
and to drink clear liquids only.
• For a needle biopsy, no restriction of
fluid or foods is necessary.
ERYTHROCYTE INDEX • monitor for bleeding & infection
MCV - MEAN CORPUSCULAR
VOLUME
• done to evaluate the size of the RBC.
• 80-94 Cu. microns. Microcytic,
Macrocytic
• ANISOCYTOSIS-ABNORMAL SIZE
MCH - MEAN CORPUSCULAR
HEMOGLOBIN
• done to evaluate the Hgb content of
the RBC.
• 22-28 micro micrograms,
hypochromic, hyperchromic
MCHC - MEAN CORPUSCULAR
HEMOGLOBIN CONCENTRATION
• done to evaluate the Hgb content in
grams per 100 ml of packed RBC
• 30-36 grams/100 ml packed RBC

Diagnostic Procedures
BM Aspiration and Biopsy
• Site: Posterior iliac crest
• Nsg Care:
⁃ Consent
⁃ Position, sterile field – Local
anesthetic
⁃ Pressure 5 min
⁃ Check site:
⁃ Send to lab
⁃ Teaching:

LYMPH NODE BIOPSY


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• Provision of platelets (platelet conc,
FWB)

NURSING RESPONSIBILITIES
• Assess client for history of previous
BT, adverse rx
• IV cannula : G 18- 19, 0.9% Plain NSS
• 2 nurses: ABO group, Rh type, Blood
no, Expiration date
• Baseline VS
• Start slow: 2ml/min
• Stay with client first 15 min, take VS
Blood Transfusion and Component • Maintain prescribed transfusion rate
Therapy • Monitor for adverse reactions
Purposes: • Document:
• Improve O2 transport (RBCs) • Blood component unit number (apply
• Volume expansion (WB, Plasma, sticker)
albumin) • Date infusion starts and ends
• Provision of proteins (FFP, albumin, • Type of component and amount
plasma protein fraction) transfused.
• Provision of coagulation factors • Client reaction and VS
(cryoprecipitate, FFP, fresh WB) • Signature of transfusionist

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COMPLICATIONS OF BT • Iron
 Hemolytic: headache, lumbar pain, • Folic Acid
N/V, diarrhea, jaundice, signs of • Vitamin C
shock/renal shutdown, dyspnea • Vitamin B12
⁃ Stop, ON saline, send (lab) • Absence of these elements will cause
• Allergic: urticaria, wheezing, anemia
dyspnea, headache
⁃ Stop, antihistamine, epinephri ANEMIA
• PYROGENIC: FEVER, CHILLS, • Reduction below normal in the
FLUSHING, TACHYCARDIA number of of erythrocytes, quality of
- STOP, TREAT TEMP, ANTIBIOTICS Hgb, and the volume of erythrocytes.
• CIRCULATORY OVERLOAD: • Causes:
DYSPNEA, TACYCARDIA, INC BP • Decreased RBC production
- STOP, VS, NOTIFY • Increased RBC destruction
• AIR EMBOLISM: DYSPNEA, CHEST • Acute or chronic blood loss
PAIN, DEC BP ANEMIAS
- CLAMP, LEFT HEAD DOWN, O2, • Iron deficiency Anemia
NOTIFY • Pernicious Anemia
• Folic Acid Deficiency Anemia
• G6PD Deficiency
• Sickle Cell disease
• Thalassemia
• Aplastic Anemia
Diagnosis
• History collection
• Physical examination
• Laboratory investigations
Common Blood Disorders • Bone marrow studies
Hematologic (Blood) Diseases Classification According to WHO
• Bleeding Disorders Grading
- Platelet Function 1.Mild Anemia - The level of hb
- Coagulation Factor between 10mg/dl and cut off point
• Platelet Deficiency 2.Moderate Anemia – Hb level
-Thrombocytopenia/ Thrombocytopathia between 7mg/dl and 10mg/dl
• Red Blood Cell Disorders 3.Severe Anemia – Hb level below
- Anemia 7mg/dl
• White Blood Cell (WBC)
Disorders
- Leukopenia
- Leukemia
- (Lymphoma)

ELEMENTS ESSENTIAL FOR


ERYTHROCYTES
• Protein
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3. Hemolytic – Resulting from RBC
destruction
• Altered erythropoiesis (sickle cell,
thalassemia)

Hypoproliferative Anemia
Iron deficiency Anemia
 Inadequate intake of dietary iron for
hemoglobin sysnthesis
 Most common type of anemia in all
age groups.
Classification of Anemia According  Prevalent in developing countries-
to Morphology inadequate iron stores result from
• Normocytic - occurs when the inadequate intake or from blood
overall hemoglobin levels are loss.
decreased, but the red blood cell size  Common type of anemia in all groups
(mean corpuscular volume) remains Anemia owing to blood loss - Iron
normal. Causes include Acute blood Deficiency Anemia
loss, Anemia of chronic disease • Iron deficiency is defined as a
• Microcytic - result of hemoglobin reduction in total body iron to an
synthesis failure/insufficiency. Iron extent that iron stores are fully
deficiency anemia, thalassemia. exhausted and some degree of tissue
• Macrocytic - Megaloblastic anemia, iron deficiency is present.
the most common cause of macrocytic • Females are mostly affected.
anemia, is due to a deficiency of
either vitamin Bi, folic acid, or both. Etiology
Also seen in hypothyroidism, • Chronic blood loss
alcoholism. • Inadequate dietary intake
• Hypochromic microcytic - iron • Faulty iron absorption
deficiency anemia. • Increased requirements for iron-
infancy, childhood, pregnancy.
ANEMIAS Causes
3 Broad Etiologic Classification • Decreased iron stores
1. Hypoproliferative Anemia • Decreased intake
 Resulting from defective RBC • Increased losses
production • Increased demands
• Iron deficiency
• Vit. B12 deficiency
• Folate deficiency
• Decreased erypoietin production ( eg.
From renal dysfunction)
2. Bleeding - Resulting from RBC Loss
• Acute posthemorrhagic anemia PATHOPHYSIOLOGY
• Chronic posthemorrhagic anemia Supply of inadequate iron

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Depletion of iron stores • 20mg elemental iron + 100 mcg folic
acid
Depletion of serum transferrin • Adolescent :100 mg elemental iron
concentration +500 g of folic acid +25-50 mg vit.c
• Mild Anemia: Once Daily
Subnormal Hb concentration • Moderately And Severe Anemia:
Twice Daily
Subnormal oxygen carrying capacity • Result: Peak increase of reticwlocyte
Clinical Manifestations count in 5th-10th day of
• Chronic fatigue administration
• Pallor of the conjunctiva, lips, and oral Diet therapy
mucosa; Good sources of iron:
• Brittle nails with spooning, cracking, • legumes
• Splitting of nail beds, koilonychia • nuts
• Palmar creases • Green leafy veg.
• Palpitations • dates
• Shortness of breath, numbness • yolk of egg, fish, meat, liver
• Bone pain • ripe banana, mango
Oral Manifestations • Cooking in iron pots
• Angular cheilitis, Poor source of iron:
• Glossitis with different degrees of • Milk
atrophy of fungiform and filliform • Administer iron with fruit juices
papillae • iron with milk or antacids
• Pale oral mucosa
• Oral candidiasis Folic Acid Deficiency Anemia
• Recurrent aphthous stomatitis • Associated with decreased dietary
• Erythematous mucositis intake of folic acid
• Burning mouth
Laboratory findings Causative Factors:
• Microcytic hypochromic anemia due to • Alcoholism
inadequate supply of iron for normal • Malabsorption
hemoglobin synthesis. • Diet deficient in uncooked vegetables
• RBC-3,000,000-4,000,000/cubic mm • Use of oral contraceptives
• Low hemoglobin
• Low serum iron and ferritin with an Dx tests:
elevated total iron binding capacity • CBC: decreased RBC, Hgb, hematocrit
(TIBC) • RBC are large with fragile membranes
MANAGEMENT that rupture easily
• Oral iron therapy • Low Serum folate
• Parenteral iron therapy
• Diet therapy

Oral iron therapy


• Recommended Dosage
• Infant and children: 3 mg/kg/day
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• Sore mouth, smooth beefy red
tongue, constipation or diarrhea
Pathophysiology of Folic Acid • Loss of sense of balance,
Deficiency paresthesias, tingling, paralysis,
Decrease Folic Acid mental confusion
Diagnostic tests:
impaired DNA synthesis in the bone • CBC: macrocytic RBC’s and,
marrow abnormally shaped
• Decreased erythrocyte
impaired RBC development • BM: increased megaloblasts
impaired nuclear maturation but • Bilirubin: elevated
Cytoplasmic maturation continues • Schilling test:

large size
Nursing Interventions:
• Replacement: 1-5 mg oral folate/day
• Prenatal vitamin
• Diet: leafy greens, liver, citrus fruit,
nuts, grains
• Assess client’s tolerance to activity
• Provide adequate rest

Pernicious Anemia
Aplastic Anemia

Pernicious Anemia or Vit. B12


Deficiency
Inability to absorb Vit B12.
• Lack of Intrinsic factor (needed for Signs and Symptoms
the absorption of Vit. B12)
 Anemia (low Hb, Hct) - fatigue,
Causative Factors:
lassitude, dyspnea
• Strict Vegetarian
 Thrombocytopenia (low platelets) -
• GI Malabsorption
bruises, petechiae, serious bleeding
• Gastritis
 Neutropenia (low neutrophils, a type
• Gastrectomy
of white cell) - infections
Assessment:
Causative Factors
• weakness, pallor, fatigue

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1. Environmental Toxins - pesticides, Glucose 6 Phosphate
benzene Dehydrogenase (G6PD) Deficiency
2. Certain Drugs  RBC burst in contact with certain
• Chemotherapeutic agents substances
• Chlorampenicol ⁃ sulfas
• Phenothiazines ⁃ antimalarial
• Sulfonamides ⁃ naphthalene
3. Radiation ⁃ fava beans
4. Chemical; Assessment:
5. Viral, Infection, bacterial. idiopathic  Jaundice
Pathophysiology  Headache, dizziness, easy fatigability
Toxins cause a direct bone marrow Dx tests:
depression  G6PD level during nonhemolytic
phase
acellular bone marrow  Newborn screening test
Nursing Interventions:
decreased production of blood elements  Provide rest periods
LABORATORY FINDINGS  Hydration
1. CBC - decreased blood cell numbers  Antibiotics: to minimize complications
2. Bone marrow aspiration confirms
the anemia - hypoplastic or acellular Sickle Cell Anemia
marrow replaced by fats
Medical Management
1. Bone marrow fransplantation
2. immunosupressant drugs
3. Rarely, steroids
4. Blood transfusion
Nursing management
1. Assess for signs of bleeding and
infection
2. Pancytopenia plan of care to prevent
complications like infection and
bleeding
1. Private room
2. Strict hand washing
3. Minimizing invasive procedures
3. Provide client and family with support
for lengthy hospitalization and tx

Hemolytic Anemia
 There is an increased rate of RBC Hemolytic Anemia: Sickle Cell
destruction.  A severe chronic incurable hemolytic
⁃ G6PD deficiency anemia that results from heritance of
⁃ Sickle cell anemia the sickle hemoglobin gene.
⁃ Thalassemia
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 Produces specific mutant form of 3. tachycardia, murmurs and
beta-globin cardiomegaly
 Hypoxia-induced change in RBCS ⁃ Primary sites of thrombotic
 Associated with vascular occlusion occlusion: spleen, lungs and CNS
and tissue infarction ⁃ Chest pain, dyspnea
 Anemia is caused by accelerated 1. Sickle cell crises - Results from
breakdown of abnormal BC tissue hypoxia and necrosis
2. Acute chest syndrome -
Manifested by a rapidly falling
Causative Factor hemoglobin level, tachycardia, fever and
 Genetic inheritance of the sickle chest infiltrates in the CXR
gene- Hbs gene  During the sickling crisis, the sickling
cells clog small capillaries and the
resulting hemotasis promotes a self-
prepetuating cycle of local hypoxia,
deoxygenation of more erythrocytes
and more sickling.
MEDICAL MGT:
 Administration of large doses of
narcotic analgesics
 Since pain last 4-6 days
Pathophysiology
1. Bone marrow transplant
Factors
2. Hydroxyurea
3. Long term RBC transfusion
cause defective hemoglobin to acquire a
Nursing Management
rigid, crystal-like C-shaped configuration
1. manage the pain
⁃ Support and elevate acutely inflamed
Sickled RBCs will adhere to endothelium
joint
pile up and plug the vessels
⁃ Relaxation techniques to reduce
metabolic needs
ischemia results
⁃ analgesics
2. Prevent and manage infection
pain, swelling and fever
⁃ Monitor status of patient
Assessment:
⁃ Initiate prompt antibiotic therapy
• Infants: colic (firstsign)
3. Promote coping skills
• Dactylitis
⁃ Provide accurate information
• Splenomegaly, weak bones or spinal
⁃ Allow patient to verbalize her
defects
concerns about medication, prognosis
• Frequent infections, leg ulcers
and future pregnancy
• CVA: CNS infarct
4. Monitor and prevent potential
• Priapism
complications
• Pain: wherever vaso-occlusive crisis
⁃ Provide always adequate hydration
occurs
⁃ Avoid cold, temperature that may
Assessment Findings
cause vasoconstriction
1. jaundice
⁃ Priapism:
2. enlarged skull and facial bones
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⁃ Sudden painful erection – Splenomegaly and hepatomegaly
⁃ Instruct patient to empty bladder, – BM hyperplasia: Thick cranium,
then take a warm bath maxillary sinus space
5. Maintain F&E balance to reduce blood DX TESTS:
viscosity  CBC: microcytic,
 hypochromic RBC
 Hemoglobin electrophoresis
TREATMENT
1. Blood Transfusions. Transfusions
of red blood cells are the main
Thalassemia treatment for people who have
moderate or severe thalassemias.
2. Iron Chelation Therapy. Doctors
use iron chelation therapy to remove
excess iron from the body. Two
medicines are used for iron chelation
therapy: Deferoxamine and
Deferasirox.
3. Folic Acid Supplements.
4. Blood and Marrow Stem Cell
Transplant.
 Thalassemia is an inherited (i.e., NURSING INTERVENTION
– Regular BT, monitor reactions
passed from parents to children
– Folic acid
through genes) blood disorder
– Hemosiderosis:
caused when the body doesn't make
Chelation therapy
enough hemoglobin.
Deferoxamine (Desferal, Exjade 250
 It is an inherited autosomal recessive
mg) – Splenectomy
blood disorder, which is caused by
– BM transplant
missing or variant genes, that
– Genetic counseling
interferes with process of formation
of normal haemoglobin.
POLYCYTHEMIA VERA
 Abnormal haemoglobin results
 Increased blood cell levels
inappropriate oxygen transport and
 Overproduction by bone marrow
leads to destruction of RBCs.
 Red blood cells are responsible for
Types:
 Thalassemia Minor - One carrying oxygen into blood cells to all
body parts. Polycythemia Vera may
thalassemia and one normal gene
also result in an overproduction of
- Mild manifestations
other blood cells, platelets and white
 Thalassemia Major - Two
blood cells as well. Due to an
thalassemia genes causing severe
overproduction of blood cells, the
manifestations
blood will get thicker leading to
 Alpha Thalassemia
various complications.
 Beta Thalassemia
PRIMARY POLYCYTHEMIA VERA
Assessment:
– Skin: pale, jaundiced, fatigue, SOB
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 Is a proliferative disorder of the
myeloid stem cells
 The bone marrow is hypercellular
 Elevated erythrocytes, leukocytes,
and platelet counts in the peripheral
blood.
 This phase can last for 10 years or
more
 Estimated incidence is 2/ 100,000
people.
 Median age of onset is 65 years
SIGNS AND SYMPTOMS:
Disseminated Intravascular
• Headache, dizziness, blurring of vision
Coagulation (DIC)
• Reddening of the lips and mucous
• is a rare but serious condition that
membrane
causes abnormal blood clotting
• Increase peripheral resistance
throughout the body's blood vessels
• Thrombus formation
• DIC is not a disease but a sign of
• Hepatomegally
underlying conditions,which can be
• Spleenomegally
triggered by sepsis, trauma, cancer,
• Bleeding Anemia
shock,abruptio placenta, toxins or
• Inrease body temp.
allergic reactions
• pruritus
• INFECTIOUS:
• dec. body weight
• Meningococcemia - purpura fulminans
Lab tests:
• Bacterial sepsis - staphylococcal,
• CBC: Increased Hct, indirect Bilirubin
streptococcal, E coli
– BM-increased immature cells
• Rickettsia - Rocky Mountain spotted
MANAGEMENT:
fever
• Increase fluid intake
• Viral - CMV, varicella, arboviruses
• Promote activity and exercises
• Malaria, Candida, Aspergillus
• Methotrexate
• TISSUE INJURY:
• Phlebotomy
• Multiple fractures with fat emboli,
crush injury, head injury
Disorders of Platelets and Clotting
• MALIGNANCY:
Mechanism
• Acute promyelocytic leukemia, acute
HEMOSTASIS
myeloid leukemia, neuroblastoma
• The balance between clot formation
• VENOM OR TOXIN:
(thrombus) and clot dissolution or
• Snake bites, insect bites
fibrinolysis.
Assessment:
• The process of wound being closed by
• Petechiae, Ecchymosis
clotting, starts when blood leaks out
• Prolonged bleeding, severe bleed
of the body.
(childbirth, surgery)
• Convulsions
• Coma
DX TESTS:

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• PT, PTT prolonged  Idiopathic thrombocytopenic purpura
• Fibrinogen depressed is an immune disorder in which the
• Platelet depressed blood doesn't clot normally.
Pathophysiology  This condition is now more commonly
referred to as immune
thrombocytopenia (ITP)
Cause:
 Unknown (autoimmune)
 Sudden onset often preceded by a
viral illness
Assessment:
 Petechiae, Ecchymosis
 Bleeding from mucous membrane,
nosebleed – Very low platelet count
CLASSIFICATION
DX TESTS:
Acute DIC
 Decreased platelet count
 Bleeding from vein puncture site,
 Bleeding time is prolonged
surgical wound.
Nursing Interventions:
 Grayish discoloration of tips of
 Control bleed: Platelet transfusion
fingers, toes & ears symmetrical
 Prevent bruising, Protect infection
distribution.
 Administer meds oral, rectal, IV (not
 Meningococcemia (PURPURA
IM)
FUMINANS) bleeding from Gl tract,
 Acetaminophen, avoid aspirin
gingival bleeding, epistaxis,
 IV immunoglobulin, glucocorticoids
pulmonary hemorrhage, hematuria.
 Pad crib, soft toys, knee/elbow pads,
Chronic DIC
headgears
 Superficial and extensive ecchymosis
 Soft toothettes, stool softener, avoid
of extremities without petechia which
contact sports
may be intermittent or can persist.
 Recurrent episodes of epistaxis or
HEMOPHILIA
internal mucosal bleeding.
 Hereditary bleeding disorder (sex-
 Trousseau sign- Recurrent migratory
linked) caused by lack of coagulation
thrombophlebitis in association with
factor
cancer.
 Hemophilia is a genetic condition
 Impairment of renal function,
where the blood cannot clot.
confusion, repeated episodes of
 Blood clotting involves proteins called
cerebral thrombosis.
coagulation factors.
Nursing Interventions:
 Hemophilia is caused by a lack of
 Monitor blood loss
factor VIII or IX.
 BT, medications, avoid aspirin
 Prevent further injury

Idiopathic Thrombocytopenic
Purpura (ITP)

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Nursing Interventions:
• Control acute bleeding: RICE, manual
pressure of 15 mins.
• Avoid sutures, cauterization, aspirin –
Hemostatic agents: Fibrin foam
• Provide care for hemarthrosis:
• Immobilize, elevate slightly flexed
• Administer analgesic (no aspirin)
• Avoid weight bearing: 48 hrs
• Active/Passive exercise
• Factor Vlll
• Factor IX
• Teaching: Prevention of trauma
• Genetic counselling

Ty
pes:
• Hemophilia A - def Factor Vlll
• Hemophilia B (Christmas disease) -
def of Factor lX
According to Severity
• Severe (factor levels less than 1%) LEUKEMIA
represent approximately 60% of cases
• Moderate (factor levels of 1-5%)
represent approximately 15% of cases
• Mild (factor levels of 6%-30%)
represent approximately 25% of cases
Assessment:
 Prolonged bleeding after minor injury
 Bruising and hematomas but no
petechiae
 Peripheral neuropathies: pain, MAIN PATHOPHYSIOLOGY
paresthesia, muscle atrophy  there is uncontrolled, abnormal,
 Hemarthrosis: knees, ankles, elbows, proliferation, multiplication of
wrist immature wbc known as blast cells.
DX TESTS: PATHOPHYSIOLOGY
 Platelet count normal  Immature blood cells
 PTT increased  Multiplying cells
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 Proliferating cells
 Cells congesting inside the bm
 Advanced stage multiplying cells can
invade the vital organs
ASSESSMENT:
 Anemia:
 Bleeding, petechiae, ecchymosis
 Infection:
 Enlarged lymph nodes
 Hepatomegaly, splenomegaly
 Abdominal pain – Bone pain
How to Classify LEUKEMIA
ACUTE LEUKEMIA
 Sudden
 Short course/short duration
 Less than 6 mos.
 Predominant cells are immature blast
cells
CHRONIC LEUKEMIA
 Gradual
 Long course/long duration , 2-8 years
 Predominant cells are matured wbc

MANAGEMENT
 RADIATION: Administered through
a high energy x-ray beam.
 CHEMOTHERAPY: Chemotherapy is
a drug treatment that uses powerful
chemicals to kill fast-growing cells in
your body
-Maintenance: chemo (2-3 yrs)
 STEM CELL TRANSPLANT

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