BLOOD DISORDERS

Minerva A. Cobus
Overview of
Hematological System
Bone Marrow
Contained inside of the bone,
occupies interior of spongy and
center of long bones
4-5% of total body weight
Primary function is Hematopoiesis-
formation of blood cells
Bone Marrow
2 kinds:
a. Red marrow (functioning)
a. Carries out hematopoiesis
b. Found in ribs, vertebral column, other flat
bones
b. Yellow marrow
a. Red marrow that has changed to fat;
found in long bones
b. Does not contribute to hematopoiesis
Blood
Composed of plasma (55%) and
cellular components (45%)
Hematocrit
a. reflects portion of blood composed of
RBC



Plasma
Liquid part of blood; yellow in color
Consist of serum (liquid portion of
plasma) and fibrinogen
Contains plasma proteins such as:
a. Albumin: largest plasma proteins,
involve in regulation of intravascular
plasma volume and maintenance of
osmotic pressure

Plasma
b. Serum globulins:
a. Alpha role in transport of steroids, lipids,
bilirubin
b. Beta: role in transport of iron and copper
c. Gamma: role in immune response,
function of antibodies
c. Fibrinogen, prothrombin, plasminogen-
for coagulation
Cellular Components
Erythrocytes RBCs responsible for
oxygen transport
Normal 12-18 g Hgb/100cc blood;
men- 14-18 g; women 12-14g
Average life span- 120 days
Leukocytes- protection from bacteria
and other foreign substances

Cellular Components
Thrombocytes essential factor in
coagulation via adhesion,
aggregation, and plug formation

Diagnostic Test
Blood
Complete CBC: WBC; Hgb and Hct;
platelet and reticulocyte count
Coagulation studies: PT; PTT; (FSP)
fibrin split products; Lee-white clotting
(whole blood clotting time)
Blood Chemistry: BUN; creatinine; uric
acid
Urine and stool
Urinalysis
Hematest
Bence-Jones protein assay (urine)
Radiologic
Chest x-ray
Radionuclide scans (bone scan)
Bone marrow aspiration and biopsy
Puncture of the iliac crest (preferred
site), vertebrae body, sternum, or tibia
(in infants) to collect tissue from bone
marrow
Purpose: study cells involved in blood
production
Nursing care:
Confirm consent
Allay anxiety
Position client and assist physician to
maintain sterile field
Immediately after aspiration, apply
pressure to the site for at least 5
minutes and longer, if necessary
Send specimen to the laboratory.

Sickle Cell test
Sickledex
No hemoglobin S present
Routine screening test for cell, trait
or disorder; does not distinguish
between them
Hemoglobin Electrophoresis
Separates various hemoglobins and
allows for identification of specific
problem
Differentiates between traitor
disorder in sickle cell anemia
Diagnosis of thalassemia and
hemolytic anemia
Fibrin Split Products
Fibrinolysis occurs in intravascular
coagulation
Abnormally high levels in DIC
Schilling Test
Normal excretion is 10-40% of oral
dose of the radioactive B12
Stage 1-is a 24-hour urine test after
a dose of radio labeled B12
Stage 2-is a second 24-hour urine to
determine response to intrinsic
factor; distinguishes between
pernicious anemia and
malabsorption.
BLOOD COMPONENT
THERAPY
Plasma Components and
Products

Consist of:
Fresh frozen plasma
Cryoprecipitate

Fresh Frozen Plasma
Plasma which is separated from whole
blood and frozen within 6 hours of
phlebotomy.
Content:
-plasma protein
-all coagulation factors ( include labile F V
+ F VIII)
Storage and shelf life:
- storage at -18C
- deep freeze at -40C can be kept for 2
year



FFP
Properties:
-1 unit volume: 200 ml
- pediatric : 50-100 ml
Administration :
- thawed in 37C water bath
- administer within half an hour after
thawing
( activity of labile F V, V III is rapidly
lost)
FFP
Definite indications:
1) Replacement for isolated factor
deficiencies (specific factor not available
eg: F V)
2) Immediate reversal warfarin effect.
3) Replacement of coagulation factors in:
DIVC.
4) Inherited def of inhibitors of coagulation
eg: Anti thrombin III, protein C, protein S.

FFP
Conditional uses (FFP only indicated in
the presence of bleeding and disturbed
coagulation):
- Massive transfusion
- Liver disease
- Cardiopulmonary bypass surgery
- Special pediatric indications eg: severe
sepsis

FFP
Contraindication:
- estimated as many 73% are used without
justification
- 1) volume expander
- 2) nutritional source of protein +
immunoglobulin
- 3) Urticaria + anaphylactoid reaction or
previous severe reaction to plasma

FFP
Dose + Administration:
- ABO compatible with recipient blood cell
+ without regard to Rh type
- Cross matching not required
- Amount used depend on clinical situation
eg: DIVC case: 2 units for adult
paediatric: 10-20 mls /kg
- efficacy assessed with PT, aPTT
Platelets
Will raise recipient's platelet count by
10,000 u/liter
Pooled from 4-8 units of whole blood

Albumin Preparation
Protein solution containing
predominantly albumin prepared by
fractionation of large pools of
plasma.
Properties:
1) Albumin solution
2) Plasma protein fraction
Albumin solution
Available in 5% and 25% solution.
Contain 96% albumin and 4% globulin +
other plasma protein.
Pooled plasma product and is heat treated
to inactivate viral activity.
5% solution: osmotically + oncotically
equivalent to plasma
25% solution; markedly hyperoncotic.
Shelf life: 5 years at 2-10C.


Plasma Protein Fraction
Prepared the same manner as
albumin.
Differences: albumin content 80 to
85%
Stored at room temperature for 3
years or at 1-6C for 5 years.
Administration:
- albumin + PPF do not contain ABO
antigen or Ab compatibility not
required

Albumin preparation
Indication (both):
Volume expansion and colloid replacement
for patients in hypovolemic shock, burn,
undergoing retroperitoneal surgery in
which large volume of protein-rich fluid
may pool in the bowel.
Indication for PPF: parallel those for 5%
albumin.
Albumin solution not indicated to correct
nutritional def (enteric, parenteral,
hyperalimentation > appropriate)



Factor VIII Concentrate
FVIII concentrate or antihemophilic
Factor (AHF) specific treatment for
F VIII def (hemophilia A)
Prepared fr large donor pools of
plasma
Pooled plasma is fractionated ,
lyophilized + stored at 1-6C
Heat treated to reduce risk of
transfusion transmitted disease.

Factor IX Concentrate
2 heat treated , lyophilized
concentrates of F IX:
1) Factor IX complex
2) Pure Factor IX concentrate
F IX complex (Prothrombin
complex) Contain factor II, VII,
IX and X
F IX concentrate: contain 20-30 %
F IX, trace amount of F II, VII, X.
Factor IX concentrate
Indication:
1) Hemophilia B
2) Haemophilia A with inhibitors
Contraindication:
-Fac IX complex a/w thromboses and
episode of DIC.
- Adverse effect also seen in
Haemophilia A with inhibitors

Autologous Transfusion
Therapeutic benefit:
1) Safe (risk of TTD eliminated)
2) No alloimmunization
Conserves blood banks limited
homologous blood supply
Either be:
1) Perioperative Hemodilution
2) Intraoperative Salvage
3) Postoperative Blood Salvage
Perioperative Hemodilution
Eg: Cardiopulmonary bypass surgery
Withdraw 1/> units of blood
preoperatively + replace with crystalloid /
colloid solution normovolemic,
hemodiluted state
Results in improved blood flow in pt
circulation + loss of < red cell
Labelled properly (pt name, RN,date/time
phlebotomy)
Reinfuse within 8 hr: reliable source of plt,
coag factors.


Intraoperative Salvage
Blood-containing fluid aspirated fr op
site, centrifuge or washed and reinfused
through a filter during op/post op.
Vascular procedure large amount of
blood pool in body cavities.
Collection device washes with 0.9%
saline + transfuse immediately or
stored at room temperature for 6 hrs or
at 1-6C for 24 hrs.
Complication: hemolysis, DIVC, air
embolism
Postoperative Blood Salvage
Within first 24-48 hrs after surgery in pt
actively bleeding in closed site (chest,
joint cavity)
Contraindicated if evidence of infection or
malignant tumor cell at site
Blood collected into sterile container,
labelled and filtered.
Reinfuse within 6 hours, to minimize
proliferation of bacteria

Nursing Care during
Blood Transfusion
1. Assess client for history of previous
blood transfusion and any adverse
reactions.
2. Ensure that pt. has an 19 or 19
gauge IV catheter in place
3. Use .0% sodium chloride
4. At least 2 nurses should verify the
blood component
5. Take baseline v/s
6. Start transfusion at slow rate
7. Stay w/ the client for the 1
st
15
mins; continue monitor v/s
8. Maintain prescribe transfusion rate:
1. Whole blood: approx 3-4 hours
2. RBCs: approx 2-4 hours
3. FFP: as quickly as possible
4. Cryoprecipitate: rapid infusion
9. Monitor for adverse reactions
10. Document the following:
1. Blood component unit number
2. Date infusion starts and ends
3. Type of component and amount
transfused
4. Client reaction and v/s

Transfusion Reaction
1. Hemolytic Reaction with in 15 min
a. S/sx headache, lumbar or sternal
pain, dyspnea, restlessness, fever,
chills, heat along veins, renal
shutdown
b. Intervention stop transfusion, obtain
1
st
voided urine specimen, send blood
unit and client blood sample to lab,
continue saline IV
Transfusion Reaction
2. Allergic Reaction with in 30 min
a. S/sx uticaria [hives], laryngeal edema,
dyspnea, sever-shortness of breath
b. Intervention stop transfusion, administer
antihistamine and/or epinephrine.
3. Febrile Reaction with in 15 90 min
a. S/sx fever, chills, flushing, headache, N &
V, palpitations
b. Intervention treat temperature, keep client
covered and warm during transfusion,
administer antipyretic medication.
ANEMIAS
Decreased availability of oxygen
to the tissues
Kinds:
Iron Deficiency Anemia
Aplastic Anemia
Folate Deficiency Anemia
Pernicious Anemia
Hemolytic Anemia
Sickle Cell Anemia
ANEMIAS

Causes
Acute / chronic blood loss
Inadequate dietary intake of
vitamins / minerals needed for rbc
production
Decreased rbc production by the
bone marrow
Increased destruction of rbc
Increased demands of vitamins /
minerals needed for rbc production
COMMON CLINICAL
MANIFESTATIONS
Pallor
Easy fatigability
Weakness
Anorexia
Weight loss
SOB
Headache /
Dizziness
Tachycardia /
palpitations

Syncope
Brittle hair and
nails
Paresthesia
Cold sensitivity
Amenorrhea
Iron-deficiency Anemia
Chronic microcytic, hypochromic
anemia caused by either
inadequate absorption or excessive
loss of iron.
Risk Factors:
a. Women between 15-45 y/o
b. Common in infants whose primary diet
is milk
c. Heavy flow during menses
Assessment:
a. Mild cases usually asymptomatic
b. Palpitations, dizziness, and cold
sensitivity
c. Dyspnea, weakness
d. Laboratory findings:
a. RBCs small and pale
b. HGB markedly decreased
c. Hct decreased
d. Serum iron decreased
Nursing Interventions
Promote rest
Provide good oral care
Provide good skin care.
Diet: Iron rich foods
Organ meals
Beans
Green, leafy vegetables
Raisins
a. Monitor for s/sx of bleeding
b. Provide adequate rest
c. Explain all diagnostic procedure
d. Administer iron preparation
COLLABORATIVE MANAGEMENT
Medical Management
Iron Supplement
Oral
Ferrous Sulfate
Ferrous Gluconate
Ferrous Fumarate
Parenteral: Iron Dextran (Imferon)
Oral iron should be given p.c.
Oral liquid iron to be administered with straw
Vitamin C increases iron absorption
X administer with milk, antacid
Imferon : Z track (deep IM)
Do not massage site of injection
Iron salts change color of stool to dark green
or black
Oxygen therapy
Blood transfusion as needed
FOLATE DEFICIENCY ANEMIA
(MEGALOBLASTIC ANEMIA)
Causes
Poor dietary intake
Rarely eat uncooked
(raw) fruits and
vegetables
Alcoholism
Chronic malnutrition
Pregnancy
Anorexia nervosa
Malabsorption
Malignancy
Prolonged TPN
Chronic
hemodialysis
Assessment
Cracked lips, sore
tongue
Same as Pernicious
Anemia
Collaborative
Management
Well balanced diet
Folic acid 1mg. / day

PERNICIOUS ANEMIA
Macrocytic, hyperchromic anemia
Causes
Gastric surgery
Crohns disease
Pathophysiology
INTRINSIC FACTOR production

VITAMIN B12 absorption

RBC production
DNA synthesis in maturing RBC
(megaloblastic cells)
Impairment of integrity of cells
(mouth, stomach, anus, vagina, axon of
neurons)
DIAGNOSTIC TESTS
Tubeless Gastric Analysis

blue
HCl is present
(-) Pernicious Anemia
X blue
(-) HCL Achlorhydria
(+) Pernicious Anemia
Collect urine specimen

Diagnex blue / Azuressin tablet

DIAGNOSTIC TEST
Schillings Test
Most definitive diagnostic test
Oral radioactive Vitamin B12
IM nonradioactive Vitamin 12
Collect 24 hour urine specimen
Decreased excretion of Vitamin
B12 in the urine
(+) Pernicious Anemia
COLLABORATIVE
MANAGEMENT
Vitamin B12 IM
Daily: 7 days
Weekly: 10 weeks
Monthly: LIFETIME (100mg/Mo)
Hydrochloric acid p.o. for 1 week
Iron therapy
Blood transfusion as needed
Physical examination every six
months
*At risk for Gastric Cancer

APLASTIC ANEMIA
Hypoplasia of the bone
marrow
Fat replaces bone
marrow
PANCYTOPENIA
Anemia
Leukopenia
Thrombocytopenia
Causes
Congenital
Acquired
Idiopathic
Infections
Medications
Heavy metals
Hepatitis

Diagnostic tests
Bone marrow
aspiration /
biopsy
Adult
Posterior Iliac
Crest
Prone Position
Brief , sharp
pain
Child
Tibia
Side lying
position

COLLABORATIVE
MANAGEMENT
Medical Management
Bone marrow transplantation
Immunosuppressive therapy
(Antithymocyte Globulin ATG)
Central line 7 10 days
Blood transfusion as necessary
Stool softeners; high fiber diet
NURSING INTERVENTIONS
Assess for signs and symptoms of
infection, tissue hypoxia and
bleeding
Frequent rest periods
Good oral care
Use soft bristled toothbrush /
soft swabs
Male clients
Use electric razor


NURSING INTERVENTIONS
X ASA administration
Reverse / Protective Isolation
Practice asepsis
X fresh fruits / fresh flowers in the
patients unit
Limit visitors
X allow persons with signs and
symptoms of infection to visit
Hemolytic Anemia
Cause often unknown
Characterize by increased rate of
RBC destruction
Assessment:
Pallor, slight jaundice
Hematuria
Melena
Medical Mngt:
Folic acid supplements
BT
Corticosteroids in
autoimmune types of
anema
Intervention
Monitor for s/sx of
hypoxia
If jaundice and
associated pruritus are
present , avoid soap.
Frequent turning

Sickle Cell Anemia
Characterized by the sickling effect
of the erythrocytes
Predominantly a problem of children.
Asymptomatic until 4-6 mons of age.
Autosomal Recessive
Pathologic changes results from:
Increased blood
viscosity
Increased red cell
destruction 6 20
days life span

Types of Crisis
1. Vaso-occlusive severe pain,
thrombotic; caused by stasis of
blood w/ clumping of cells in the
microcirculation
2. Splenic sequestration life
threatening crisis, death can occur
w/in hours; pooling of large amount
of blood in the spleen and liver
3. Aplastic diminished production
and increased destruction of RBC.
Intervention
Pain control
Promote hydration 4 6 liters/day, folic
acid supplement
Control anxiety
Bedrest
BT
Genetic counseling
Prevent infection
Discharge planning and home care.
DISORDERS AFFECTING THE
RBCS
Polycythemia
rbc and hgb production
Compensatory response to chronic hypoxia
Hypoxia

Erythropoietin production by the kidneys

Stimulation of the bone marrow to rbc
production
DISORDERS AFFECTING THE
RBCS
Polycythemia Vera
Hyperplasia of the bone marrow
rbc (erythrocytosis); wbc (leukocytosis);
platelets (thrombocytosis)
Cause is unknown; associated with genetics
Pathophysiology






rbc, wbc, platelets
blood viscosity
thromboembolism
organ
infiltration
Hepatomegaly
Splenomegaly
Arthralgia
Cerebral hypoxia
capillary
overdistention
Rupture
Hemorrhage
Hypovolemia
POLYCYTHEMIA VERA
Signs and Symptoms
Headache
Dizziness
Blurred vision
Fatigue
Hepato-splenomegaly
COLLABORATIVE MANAGEMENT
fluid intake
Monitor for signs and symptoms of bleeding
Monitor for signs and symptoms of
thromboembolism
Angina
Claudication
Thrombophlebitis
Pruritus
Administer antihistamine to decrease pruritus
Administer analgesic as ordered
Therapeutic phlebotomy
Chemotherapy
Radiation therapy (Na Phosphate / IV)
Patient teaching : Avoid high altitude
Disseminated Intravascular
Coagulation
Hallmarks:
Excessive clotting, followed by
dissolution
Presentation:
Cutaneous oozing, petechiae, cyanosis,
N/V, oliguria, bleeding from GI tract
Causes:
Damage to endothelium
Release of tissue thromboplastin
Activation of Factor X

Disseminated Intravascular Coagulation
Tests/Therapies
TESTS:
Platlets
Prothrombin
Time
Fibrin

THERAPIES
Supportive
Fresh-frozen
plasma,
platelets, or
packed red
cells
Heparin
Intervention:
Watch out for bleeding problems
Provide emotional support