hematology 06

8/11/2019 Hematology 06

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Hematology

Jan Bazner-Chandler

CPNP, CNS, MSN, RN


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Blood

Blood is the fluid of life

Blood is composed of:

Plasma

RBC

WBC

Platelets


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Plasma

Plasma consists of:

90% water.

10 % solutes: albumin, electrolytes and proteins.

Proteins consist of clotting factors, globulins,

circulating antibodies and fibrinogen.


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Red Blood Cells

RBCs travel through the body delivering

oxygen and removing waste.

RBCs are red because they contain a protein

chemical called hemoglobin which is brightred in color.

Hemoglobin contains iron, making it an

excellent vehicle for transporting oxygen andcarbon dioxide.


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RBCs

Average life cycle is

120 days.

The bones are

continually producingnew cells.


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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.


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WBCs

WBC life span is from a few days to a few

weeks.

WBCs will increase when fighting infection.


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Platelets

Platelets are irregularly-

shaped, colorless

bodies that are present

in blood. Their sticky surface lets

them form clots to stop

bleeding.


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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


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Hematocrit

Normal hematocrit levels are 35 to 44%.

Panic levels:

Hmct less than 15 %

Hmct greater than 60%


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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.


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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 cellscarry oxygen to tissue

Plateletshelp blood to clot

White blood cellsfight infection


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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 intothe patients blood just like at transfusion.


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Bone Marrow Aspiration


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Treatment Modalities

Transfusion:

Packed red blood cellsanemia

Plateletsplatelet dysfunction

Fresh frozen plasmacoagulation factors


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Blood Transfusions

3 types of transfusion reactions

Hemolytic

Allergic

Febrile


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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 canstimulate B lymphocytes to produce

antibodies to the red blood cell antigens.


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Hemolytic reaction

Flank pain

Fever

Chills

Bloody urine

Rash

Low blood pressure

Dizziness / fainting


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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


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Febrile Reaction

Often occurs after multiple blood

transfusions.

Symptoms:fever, chills, and diaphoresis.

Interventions:

Slow transfusion and administer antipyretic.

Administer antipyretic prior to administration.


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Allergic Reaction

Symptoms: rash,

urticaria, respiratory

distress, or

anaphylaxis.

Interventions:

administer antihistamine

before transfusion

Physician may order

washed rbcs


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Hematologic Conditions


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Alteration in Hematologic Status

Disorders of hemostasis or clotting factors

Structural or quantitative abnormalities in the

hemoglobin.

Anemias

Aplastic Anemia


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Genetic Implications

The following have a genetic link: implications

for genetic screening and fetal diagnosis

Sickle cell anemia

Thalassemia

Hemophilia


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Bleeding Disorders

Three types Hemophilia: males only

Type A most commonfactor VIII deficiency

Type B - lack of factor IX (Christmas Disease)

Type Clack of factor XI

Von Willebrand Disease1% of populationmen

or womenprolonged bleeding time


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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


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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


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Historical Perspective

First recorded case in Talmud Jewish text by

an Arab physiciandocumentation of two

brothers with bleeding after circumcision.

Queen Victoria is carrier and spread thedisease through the male English royalty.


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Goals of Care

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.


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Symptoms

Circumcision may produce prolonged

bleeding.

As child matures and becomes more active

the incidence of bleeding due to traumaincreases


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Symptoms

May be mild, moderate or severe

Bleeding into joint spaces, hemarthrosis

Most dangerous bleed would be intracranial.


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Diagnosis

Presenting symptoms

Prolonged activated aPTT and decreased

levels of factor VIII or IX.

Genetic testing to identify carriers


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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.


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Nursing Diagnoses

Risk for injury

Pain with bleed especially into a joint

Impaired physical mobility

Knowledge deficit regarding disease and

management of disease


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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.


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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.


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Family Education

Medic-Alert bracelet

Injury prevention appropriate for age

Signs and symptoms of internal bleeding or

hemarthrosis

Dental checkups

Medication administration


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Long Term Complications

20% develop neutralizing antibodies that

make replacement products less effective.

Gene therapy providing continuous

production of the deficient clotting factorcould be the next major advance in

hemophilia treatment.


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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.


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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.


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Management of DIC

Treatment of the precipitating disorder.

Supportive care with administration of plateletconcentration and fresh frozen plasma and

coagulation factors. Administration of heparin (controversial in

children).

Heparin potentates anti-thrombin III which

inhibits thrombin and further development ofthrombosis.


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Nursing Diagnoses

Altered tissue perfusion

Risk for injury

Anxiety


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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.


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Prognosis

Depends on the underlying disorder and the

severity of the DIC.


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ITP

Idiopathic thrombocytopenic purpura

Idiopathic = cause is unknown

Thrombocytopenic = blood does not have enough

platelets Purpura = excessive bleeding / bruising


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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


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Symptoms

Random purpura

Epistaxis, hematuria, hematemesis, and

menorrhagia

Petechiae and hemorrhagic bullae in mouth


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Diagnostic Tests

Low platelet count

Peripheral blood smear

Antiplatelet antibodies

Normal platelet count: 150,000 to 400,000


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Management

IV gamma globulin to block antibody

production, reduce autoimmune problem

Corticosteroids to reduce inflammatory

process IV anti-D to stimulate platelet production


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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


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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 tocongestion and tissue hypoxia; clinically, this

hypoxia causes additional sickling and

extensive infarctions.


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Whaley & Wong Text


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Crescent Shaped Cells


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Body Systems Affected by SS

Brain: CVAparalysis - death

Eyes: retinopathyblindness

Lungs: pneumonia

Abdomen: pain, hepatomegaly, splenomegaly(medical emergency due to possible rupture

Skeletal: joint pain, bone painosteomyelitis

Skin: chronic ulcerspoor wound healing


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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


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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

A l


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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

i Di


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Nursing Diagnoses

Altered tissue perfusion

Pain

Risk for infection

Knowledge deficit regarding disease process

N i M H i l


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Nursing Management - Hospital

Increase tissue perfusion Oxygen

Blood transfusion if ordered

Bed rest Pain management

Hydration IV fluids as ordered

Oral intake of fluids


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P i Ed i


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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

B Th l i


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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 Ch i i


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RBC Characteristics

Microcytosis = small in

size

Hypochromia = decreasehemoglobin

Poikilocytosis = abnormal

shape

T / P i


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Treatment / Prognosis

Supportive

Blood transfusions as needed

Bone marrow transplant

Poor prognosis / death within 1styear due to septicemia orheart failure.

I D fi i A i


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Iron Deficiency Anemia

Most common nutritional deficiency

Depletion of iron stores

Ab l L b V l


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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


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IDA

Occurs in children experiencing:

Rapid physical growth

Low iron intake

Inadequate iron absorption Loss of blood

S t


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Symptoms

Associated with low oxygenation of tissue:

Pallor

Fatigue

Shortness or breath Irritability

Intolerance of physical work / exercise

M t


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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

M t


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Management

Nutritional counseling

Infants younger than 12 months should be onformula 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

Apl ti A i


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Aplastic Anemia

Acquired or inherited

Normal production of blood cells in the bone

marrow is absent or decreased.

A marked decrease in RBCs, WBCs and

platelets.

C s s


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Causes

Exposure to drugs

Exposure to chemicals

Exposure to toxins

Infection

Idiopathic in nature

Blood Characteristics


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Blood Characteristics

Neutophil less than500

Platelet less than20,000

Hemoglobin less than7

Reticulocytes 1%

Nursing Diagnosis?

Bone marrow reveals hypo-cellular and fatty marrow.

Management


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Management

Immunosuppressive therapy

Antithymocyte globulin

Administered IV over 4 days Response seen within 3 months

Bone Marrow Transplant

Hyper bilirubinemia


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Hyper-bilirubinemia

Hyperbilirubinemia


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Hyperbilirubinemia

Many babies have some jaundice. When they are a fewdays old, their skin slowly begins to turn yellow. Theyellow color comes from the color of bilirubin. When redblood cells die, they break down and bilirubin is left. Thered blood cells break down and make bilirubin. Innewborns, the liver may not be developed enough to getrid of so much bilirubin at once. So, if too many red bloodcells die at the same time, the baby can become veryyellow or may even look orange. The yellow color doesnot hurt the baby's skin, but the bilirubin goes to thebrain as well as to the skin. That can lead to braindamage.

Signs and symptoms


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Signs and symptoms

Very yellow or orange skin tones (beginning atthe 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


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Photo Therapy

Fiberoptic Blanket


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Fiberoptic Blanket



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Monitor bilirubin levels Assess activity levelmuscle toneinfant

reflexes

Encourage po intake: May need to supplement

with formula if inadequate breastfeeding Weight daily to assess hydration status

Monitor stoolsamount and number

Cover eyes while under bili-lights

Facilitate parent - infant bonding

Loss of moro or startle reflex can indicate possible brain damage due toKernicterus

hematology 06

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