Hematology Lecture notes
Blood
Blood is the fluid of life
Blood is composed of:
Plasma
RBC
WBC
Platelets
Plasma
Plasma consists of:
90% water.
10 % solutes: albumin, electrolytes and proteins.
Proteins consist of clotting factors, globulins, circulating antibodies and
fibrinogen.
Red Blood Cells
RBC’s travel through the body delivering oxygen and removing waste.
RBC’s are red because they contain a protein chemical called hemoglobin
which is bright red in color.
Hemoglobin contains iron, making it an excellent vehicle for transporting
oxygen and carbon dioxide.
RBC’s
Average life cycle is 120 days.
The bones are continually producing new cells.
White Blood Cells
The battling blood cells.
The white blood cells are continually on the look out for signs of disease.
When a germ appears the WBC will:
Produce protective antibodies.
Surround it and devour the bacteria.
WBC’s
WBC life span is from a few days to a few weeks.
WBC’s will increase when fighting infection.
Platelets
Platelets are irregularly-shaped, colorless bodies that are present in
blood.
Their sticky surface lets them form clots to stop bleeding.
Blood Values
CBC with differential and platelet count.
Hgb:
Normal levels are 11 to 16 g / dl
Panic levels are:
Less than 5 g / dl
More than 20 g / dl
Hematocrit
Normal hematocrit levels are 35 to 44%.
Panic levels:
Hmct less than 15 %
Hmct greater than 60%
Hemoglobin and Hematocrit
Can be used as a simple blood test to screen for anemia.
The CBC with differential would be used to help diagnose a specific
disorder.
A bone marrow aspiration would be the most conclusive in determining cause
of anemia – aplastic / leukemia.
Bone Marrow
Bone marrow is the spongy substance found in the center of the bones.
It manufactures bone marrow stem cells, which in turn produce blood cells.
Red blood cells – carry oxygen to tissue
Platelets – help blood to clot
White blood cells – fight infection
Bone Marrow Transplant
Donor is placed under anesthesia.
Marrow is aspirated out of the iliac crest.
Marrow is filtered and treated to remove bits of bone and other unwanted
cells and debris, transferred to a blood bag, and is infused into the
patient’s blood just like at transfusion.
Bone Marrow Aspiration
Treatment Modalities
Transfusion:
Packed red blood cells – anemia
Platelets – platelet dysfunction
Fresh frozen plasma – coagulation factors
Blood Transfusions
3 types of transfusion reactions
Hemolytic
Allergic
Febrile
Hemolytic Reaction
Refers to an immune response against transfused blood cells.
Antigens, on the surface of red blood cells, are recognized as "foreign
proteins" and can stimulate B lymphocytes to produce antibodies to the red
blood cell antigens.
Hemolytic reaction
Flank pain
Fever
Chills
Bloody urine
Rash
Low blood pressure
Dizziness / fainting
Nursing Management
Stop the blood transfusion.
Start normal saline infusion.
Take vital signs with blood pressure
Call the MD
Obtain blood sample and urine specimen.
Return blood to blood bank.
Document
Febrile Reaction
Often occurs after multiple blood transfusions.
Symptoms:fever, chills, and diaphoresis.
Interventions:
Slow transfusion and administer antipyretic.
Administer antipyretic prior to administration.
Allergic Reaction
Symptoms: rash, urticaria, respiratory distress, or anaphylaxis.
Interventions:
administer antihistamine before transfusion
Physician may order washed rbc’s
Hematologic Conditions
Alteration in Hematologic Status
Disorders of hemostasis or clotting factors
Structural or quantitative abnormalities in the hemoglobin.
Anemias
Aplastic Anemia
Genetic Implications
The following have a genetic link: implications for genetic screening and
fetal diagnosis
Sickle cell anemia
Thalassemia
Hemophilia
Bleeding Disorders
Three types Hemophilia: males only
Type A most common – factor VIII deficiency
Type B - lack of factor IX (Christmas Disease)
Type C – lack of factor XI
Von Willebrand Disease – 1% of population – men or women – prolonged
bleeding time
Hemophilia Type A
Hemophilia type A is the deficiency of clotting factor VIII.
A serious blood disorder
Affects 1 in 10,000 males in the US
Autoimmune disorder with lowered level of clotting factor
All races and socio economic groups affected equally
Hemophilia
Hemophilia is a sex-linked hereditary bleeding disorder
Transmitted on the X chromosome
Female is the carrier
Women do not suffer from the disease itself
Historical Perspective
First recorded case in Talmud Jewish text by an Arab physician –
documentation of two brothers with bleeding after circumcision.
Queen Victoria is carrier and spread the disease through the male English
royalty.
Goals
of care
Provide factor VIII (IX) to aid blood in clotting.
To decrease transmission of infectious agents in blood products; hepatitis
& AIDS.
Future: gene therapy to increase production of clotting factor.
Symptoms
Circumcision may produce prolonged bleeding.
As child matures and becomes more active the incidence of bleeding due to
trauma increases
Symptoms
May be mild, moderate or severe
Bleeding into joint spaces, hemarthrosis
Most dangerous bleed would be intracranial.
Diagnosis
Presenting symptoms
Prolonged activated aPTT and decreased levels of factor VIII or IX.
Genetic testing to identify carriers
Treatment
Products used to treat hemophilia are:
Fresh frozen plasma and cryoprecipitate which are from single blood donors
and require special freezing.
Second generation of factor VIII are made with animal or human proteins.=
Nursing Diagnoses
Risk for injury
Pain with bleed especially into a joint
Impaired physical mobility
Knowledge deficit regarding disease and management of disease
Nursing interventions
No rectal temps.
Replace the factor as ordered by physician.
Manage pain utilizing analgesics as ordered.
Maintaining joint integrity during acute phase: immobilization, elevation,
ice.
Physical therapy to prevent flexion contraction and to strengthen muscles
and joints.
Provide opportunities for normal growth and development.
Teaching
Avoid aspirin which prolongs bleeding time in people with normal levels of
factor VIII.
A fresh bleeding episode can start if the clot becomes dislodged.
Natural reactions in the body cause the clot that is no longer needed to
"break down. This process occurs 5 days after the initial clot is formed.
Family Education
Medic-Alert bracelet
Injury prevention appropriate for age
Signs and symptoms of internal bleeding or hemarthrosis
Dental checkups
Medication administration
Long Term Complications
20% develop neutralizing antibodies that make replacement products less
effective.
Gene therapy providing continuous production of the deficient clotting
factor could be the next major advance in hemophilia treatment.
Disseminated Intravascular Coagulation or DIC
DIC is an acquired coagulopathy that is characterized by both thrombosis
and hemorrhage.
DIC is not a primary disorder but occurs as a result of a variety of
alterations in health.
Assessment
The most obvious clinical feature of DIC is bleeding.
Renal involvement = hematuria, oliguria, and anuria.
Pulmonary involvement = hemoptysis, tachypnea, dyspnea and chest pain.
Cutaneous involvement = petechiae, ecchymosis, jaundice, acrocyanosis and
gangrene.
Management of DIC
Treatment of the precipitating disorder.
Supportive care with administration of platelet concentration and fresh
frozen plasma and coagulation factors.
Administration of heparin (controversial in children).
Heparin potentates anti-thrombin III which inhibits thrombin and further
development of thrombosis.
Nursing Diagnoses
Altered tissue perfusion
Risk for injury
Anxiety
Nursing Interventions
Rigorous ongoing assessment of all body systems
Monitor bleeding
No rectal temps
Avoid trauma to delicate tissue areas
All injections sites and IV sites need to be treated like an arterial
stick.
Prognosis
Depends on the underlying disorder and the severity of the DIC.
ITP
Idiopathic thrombocytopenic purpura
Idiopathic = cause is unknown
Thrombocytopenic = blood does not have enough platelets
Purpura = excessive bleeding / bruising
Immune Thrombocytopenic Purpura
Antibodies destroy platelets
Antibodies see platelets as bacteria and work to eliminate them
ITP is preceded by a viral illness
URI
Varicella / measles vaccine
Mononucleosis
Flu
Symptoms
Random purpura
Epistaxis, hematuria, hematemesis, and menorrhagia
Petechiae and hemorrhagic bullae in mouth
Diagnostic Tests
Low platelet count
Peripheral blood smear
Antiplatelet antibodies
Normal platelet count: 150,000 to 400,000
Management
IV gamma globulin to block antibody production, reduce autoimmune problem
Corticosteroids to reduce inflammatory process
IV anti-D to stimulate platelet production
Sickle Cell Anemia
Autosomal recessive disorder
Defect in hemoglobin molecule
Cells become sickle shaped and rigid
Lose ability to adapt shape to surroundings.
Sickling may be triggered by fever and emotional or physical stress
Pathophysiology
When exposed to diminished levels of oxygen, the hemoglobin in the RBC
develops a sickle or crescent shape; the cells are rigid and obstruct
capillary blood flow, leading to congestion and tissue hypoxia; clinically,
this hypoxia causes additional sickling and extensive infarctions.
Crescent Shaped Cells
Body Systems Affected by SS
Brain: CVA – paralysis - death
Eyes: retinopathy – blindness
Lungs: pneumonia
Abdomen: pain, hepatomegaly, splenomegaly (medical emergency due to
possible rupture
Skeletal: joint pain, bone pain – osteomyelitis
Skin: chronic ulcers – poor wound healing
Vaso-occlusive Crisis
Stasis of blood with clumping of cell in the microcirculation, ischemia,
and infarction
Most common type of crisis; painful
Signs include fever, pain, tissue engorgement
Splenic Sequestration
Life-threatening / death within hours
Pooling of blood in the spleen
Signs include profound anemia, hypovolemia, and shock
Abdominal distention, pallor, dyspnea, tachycardia, and hypotension
Aplastic Crisis
Diminished production and increased destruction of red blood cells
Triggered by viral infection or depletion of folic acid
Signs include profound anemia, pallor
Nursing Diagnoses
Altered tissue perfusion
Pain
Risk for infection
Knowledge deficit regarding disease process
Nursing Management - Hospital
Increase tissue perfusion
Oxygen
Blood transfusion if ordered
Bed rest
Pain management
Hydration
IV fluids as ordered
Oral intake of fluids
Nursing Management
Adequate nutrition
Emotional Support
Discharge instructions
Information about disease management
Daily folic acid
Control of triggers
Prophylactic antibiotics
Immunizations / Pneumococcal
Patient Education
Necessity of following plan of care
Signs and symptoms of impending crisis.
Signs and symptoms of infection
Preventing hypoxia from physical and emotional stress
Proving adequate rest
Beta-Thalassemia
Hereditary / autosomal defect
Genetic defect on chromosome 11
Mediterranean descent
Defect in the beta globin gene
Beta globin chains are required for synthesis of hemoglobin A
RBC Characteristics
Microcytosis = small in size
Hypochromia = decrease hemoglobin
Poikilocytosis = abnormal shape
Treatment / Prognosis
Supportive
Blood transfusions as needed
Bone marrow transplant
Iron Deficiency Anemia
Most common nutritional deficiency
Depletion of iron stores
Abnormal Laboratory Values
Hemoglobin levels less than 8 g/dL
Decreased levels of Serum Iron or Total Iron Binding or Serum Ferritin
Microcytic and hypochromic red blood cells
IDA
Occurs in children experiencing:
Rapid physical growth
Low iron intake
Inadequate iron absorption
Loss of blood
Symptoms
Associated with low oxygenation of tissue:
Pallor
Fatigue
Shortness or breath
Irritability
Intolerance of physical work / exercise
Management
Iron supplementation
Given in a.m. on an empty stomach
To avoid staining of teeth, give using a syringe, dropper or straw
Instruct caretaker that child may have dark-colored stools
Management
Nutritional counseling
Infants younger than 12 months should be on formula until around 12 months
of age
Infants 12 months or older
Decrease intake of milk
Introduce solid foods
Children: iron fortified cereals, foods, meat, green leafy vegetables
Teenagers: reduce junk food
Aplastic Anemia
Acquired or inherited
Normal production of blood cells in the bone marrow is absent or decreased.
A marked decrease in RBC’s, WBC’s and platelets.
Causes
Exposure to drugs
Exposure to chemicals
Exposure to toxins
Infection
Idiopathic in nature
Blood Characteristics
Neutophil less than 500
Platelet less than 20,000
Hemoglobin less than 7
Reticulocytes 1%
Nursing Diagnosis?
Management
Immunosuppressive therapy
Antithymocyte globulin
Administered IV over 4 days
Response seen within 3 months
Bone Marrow Transplant
Hyperbilirubinemia
Many babies have some jaundice. When they are a few days old, their skin
slowly begins to turn yellow. The yellow color comes from the color of
bilirubin. When red blood cells die, they break down and bilirubin is left.
The red blood cells break down and make bilirubin. In newborns, the liver may
not be developed enough to get rid of so much bilirubin at once. So, if too
many red blood cells die at the same time, the baby can become very yellow or
may even look orange. The yellow color does not hurt the baby's skin, but the
bilirubin goes to the brain as well as to the skin. That can lead to brain
damage.
Signs and symptoms
Very yellow or orange skin tones (beginning at the head and spreading to
the toes)
Increased sleepiness, so much that it is hard to wake the baby
High-pitched cry
Poor sucking or nursing
Weakness, limpness, or floppiness
Photo Therapy
Fiberoptic Blanket
Nursing Interventions
Monitor bilirubin levels
Assess activity level – muscle tone – infant reflexes
Encourage po intake: May need to supplement with formula if inadequate
breastfeeding
Weight daily to assess hydration status
Monitor stools – amount and number
Cover eyes while under bili-lights
Facilitate parent - infant bonding
Loss of moro or startle reflex can indicate possible brain damage due to
Kernicterus
