heme Flashcards
Blood Formation
Starts as stem cells
Ability to transform into more than one type of blood cell
Every blood cell in the body arises from a stem cell
Hematopoiesis (blood formation)
Complete Blood Count
Red blood cell (RBC or erythrocyte)
Blood cell that carries oxygen
Hematocrit (HCT)
Volume of the blood that consists of rbc’s
Dependent on age and sex (after adolescence)
Hemoglobin (HGB)
Protein molecule in RBC’s
Carries/transports oxygen from the lungs to the body’s tissues
Returns carbon dioxide from the tissues back to the lungs
White blood cell (WBC or leukocyte)
Infection-fighting cells in the blood Platelets (thrombocytes)
Help to clot blood
MATURE RED BLOOD CELL- what number?
(mature at 12)
Contains Hemoglobin
Transports Oxygen to tissue and Carbon Dioxide away from tissue (Gas Exchange)
Stored in bone marrow, liver, spleen
HGB count
Neonate 15-20 g/dl
2 mo and older 12-15 g/dl
RETICULOCYTES - when do they increase?
immature red blood cells
Reticulocytes comprise about 1% of all RBC
Reticulocytes increase in count with chronic anemia or when medications are added to increase RBC and Hemoglobin
babies have more RBC bc
there is bruising from the birth process, can cause hyperbilirubinia.
HEMOGLOBIN F - how long do they last?
RBC with Fetal Hemoglobin has higher affinity with oxygen molecule
Lasts 90 days
ADULT HEMOGLOBIN
Typical RBC with Adult Hemoglobin lasts about 120 days
HGB count
Neonate 15-20 g/dl
2 mo and older 12-15 g/dl
HCT count
Approx 3x HGB
35%-45%
RETICULOCYTES
immature RBC -
Platelets - normal count?
Coagulation
Adhere to one another to plug holes in vessels or tissues where there is bleeding
Releases serotonin to injured tissue
Vasoconstrictor (decreases blood flow to area)
Plt count 150,000-500,000
White Blood Cells - count for babies?
Fights infection
The body’s army
WBC count
Healthy neonate 15,000-20,000
Children >2yrs and Adults 5,000-10,000
Assessment of Hematologic Function
Complete blood count
Decrease in any cell line may indicate disorder r/t bone marrow or immune system
History
Physical assessment
Child’s energy and activity level
Growth patterns
common leukemia in children
(children B leukemia)
pre-B cell
ANEMIA
The most common hematologic disorder of childhood
Decrease in number of RBCs and/or hemoglobin concentration below normal
Decreased oxygen-carrying capacity of blood
Consequences of Anemia
Decrease in oxygen-carrying capacity of blood and decreased amount of oxygen available to tissues
When anemia develops slowly, child adapts
Effects of Anemia on Circulatory System - what about peripheral resistance?
Hemodilution
Decreased peripheral resistance
Increased cardiac circulation and turbulence
May have murmur
May lead to cardiac failure
Cyanosis
Growth retardation
Decreased energy
types OF ANEMIA - NORMACYTIC (NORMACHROMIC) - think norm
NORMACYTIC (NORMACHROMIC)
RBC of normal size and color
Acute blood loss, Hemolysis, Malignancy of bone marrow
INTRA-ABDOMINAL HEMORRHAGE
Causes
Spleen or Liver lacerations
Seatbelts
Contact sports
Physical attacks
Treatment
Surgical Repair or Resection of Liver or Spleen
Observation and Bedrest
Administration of Iron - give it when?
Give in 2 divided doses between meals
Iron is absorbed best in an acidic environment
May add ascorbic acid
Vomiting and diarrhea may occur
If so give with meals and reduce dosage (gradually increase as tolerance develops)
Turns stool green
Lack of color indicates insufficient iron
Liquid preparations stain teeth
administer through a straw and rinse mouth after administration
B12 - give for what?
for pernicous anemia and alcohol
Blood Transfusion Therapy - maximum time to infuse?
Two RN check
Identify:
Donor and recipient blood types and groups
Expiration date
Use appropriate filter
Use blood within 30 minutes of arrival
Monitor vital signs
Transfuse slowly for first 15 to 20 minutes
Infuse over 4 hours maximum
Observe carefully for patient response
Stop transfusion immediately if signs or symptoms of transfusion reaction; notify practitioner
give iron with what?
oj
Transfusion Reactions - which is the most severe?
Hemolytic—the most severe, but rare
Febrile reactions—fever, chills
Allergic reactions—urticaria, pruritus, laryngeal edema
Air emboli—may occur when blood is transfused under pressure
Hypothermia
Delayed Reactions to Blood Transfusion
Transmission of infection
Hepatitis, HIV, malaria, syphilis, other
Blood banks test vigorously and discard units of infected blood
Delayed hemolytic reaction
Manifestations of Circulatory Overload - what color is skin?
Precordial pain (pain near heart)
Dyspnea
Distended neck veins
Cyanosis
Dry cough
IRON DEFICIENCY ANEMIA - what is lacking?
Production of HGB is inadequate
Caused by
Inadequate supply of dietary iron
Malabsorption of iron through GI tract
Chronic blood loss
Premature infants at risk
Last month gestation fetus stores enough iron for 6-12 mo
Adolescents at risk d/t
Rapid growth
Poor eating habits
Girls irregular menses
Iron Deficiency Anemia Treatment - what to use if babies in the first 12 months?
Correct underlying problem
Nutrition is generally is preventable
Breast milk or iron-fortified formula should be used for the first 12 months
SICKLE CELL ANEMIA
A hereditary hemoglobinopathy
Ethnicity
Occurs primarily in African-Americans
Occurrence 1 in 375 infants born in United States
1 in 12 have sickle cell trait
Occasionally also in people of Mediterranean descent
Also seen in South American, Arabian, and East Indian descent
Etiology of Sickle Cell
In areas of the world where malaria is common, individuals with sickle cell trait tend to have a survival advantage over those without the trait (they don’t get sick from malaria!)
Autosomal recessive disorder
1 in 12 African-Americans are carriers (have sickle cell trait)
If both parents have trait, each offspring will have 1 in 4 likelihood of having disease
Diagnosis of Sickle Cell - what part of body to test?
Cord blood in newborns
Newborn screening done in 43 states
Genetic testing to identify carriers and children who have disease
Sickle turbidity test
Quick screening purposes in children older than 6 months
Pathophysiology - sickle cell
Partial or complete replacement of normal Hgb with abnormal hemoglobin S (HgbS)
Hemoglobin in the RBCs takes on an elongated (“sickle”) shape
Sickled cells are rigid and obstruct capillary blood flow
Microscopic obstructions lead to engorgement and tissue ischemia
Hypoxia occurs and causes sickling
Large tissue infarctions occur
Damaged tissues in organs lead to impaired function
Splenic sequestration
May require splenectomy at early age
Results in decreased immunity
Sickle Cell Crisis
Precipitating factors
Anything that increases the body’s need for oxygen or alters transport of oxygen
Trauma
Infection, fever
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia
From high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia
Types of sickle cell crisis - occlusive
Vaso-occlusive (VOC) thrombotic or “Painful”
Most common
Stasis of blood with clumping of cells in microcirculation → ischemia → infarction
Signs and symptoms
Tissue engorgement Severe abd pain, thoracic/muscle/bone pain, increased jaundice, dark urine, low-grade fever, pale lips/tongue/palms/nail beds
Types of Crisis sickle cell - acute - where are RBCs trapped?
Acute sequestration
Sudden, massive entrapment of RBC’s in spleen and liver
Life-threatening, death can occur within hours
Signs and symptoms
Profound anemia
Lethargy
Hypovolemic shock
Death
Types of Crisis - sickle cell - aplastic
(a plastic bone marrow is viral)
Aplastic (bone marrow depression)
Diminished production and increased destruction of RBCs
Triggered by viral infection or depletion of folic acid
Pallor, lethargy, sleepiness, dyspnea, possible coma, decreased bone marrow activity, RBC hemolysis
Acute Chest Syndrome (related to sickle cell)
(pneumonia is a cutie)
Similar to pneumonia
VOC or infection results in sickling in the lungs
Chest pain, fever, cough, tachypnea, wheezing, and hypoxia
Repeated episodes may lead to pulmonary hypertension
Prognosis - sickle cell
No cure (except possibly bone marrow transplants)
Supportive care/prevent sickling episodes
Frequent bacterial infections may occur due to immunocompromise
Bacterial infection is leading cause of death in young children with sickle cell disease
Strokes in 5% to 10% of children with disease
Result in neurodevelopmental delay, mental retardation
Therapeutic Management - sickle cell
Focus on prevention of triggers
Aggressive treatment of infection
Possibly prophylactic with oral penicillin from age 2 months to 5 years
Monitor reticulocyte count regularly to evaluate bone marrow function
Blood transfusion, if given early in crisis, may reduce ischemia
Exchange transfusion may be appropriate in some situations
Medications
Analgesics, Droxia (chemotherapy drug), iron supplements
Frequent transfusion leads to hemosiderosis (iron in tissues)
Treat with iron chelation
Parenterally—deferoxamine
Oral—deferasirox or deferiprone
Used alone or in combination
HTSC Transplant
Human tumor stem cell (HTSC)
Potential cure for patients with SCD (sickle cell disease)
Difficult decision for HTSC transplant
Child may face death without the transplant
Preparing the child for transplant places the child at great risk
No “rescue” procedure if complications follow HTSC transplants
Other HSCT Modalities
Umbilical cord blood transplantation
Haploidentical transplants
Nonmyeloablative conditioning regimens
Sibling donor protocols
THALASSEMIA
Inherited blood disorders of hemoglobin synthesis
Classified by Hgb chain affected and by amount of effect
Autosomal recessive with varying expressivity
Both parents must be carriers to have offspring with disease
THALASSEMIA - just a and b
α-Thalassemia
α chains affected
Occurs in Chinese, Thai, African, and Mediterranean people
β-Thalassemia
β is most common
Occurs in Greek, Italian, and Syrian people
Pathophysiology - THALASSEMIA
Anemia results from defective synthesis of Hgb, structurally impaired RBCs, and shortened life of RBCs
Chronic hypoxia
Headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis
Detected in infancy or toddlerhood
Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6)
Children have small bodies and large heads
Pathologic bone fxs, cardiac arrhythmias, heart failure as a result from iron deposits fro frequent blood transfusions
β-Thalassemia
Types
Thalassemia minor
Asymptomatic silent carrier
Normal life span
Thalassemia intermediate
Moderate to severe anemia + splenomegaly
Develop normally into adulthood but puberty delayed
Thalassemia major
Severe anemia requiring transfusions to survive
Seldom survive to adulthood
Diagnosis - Thalassemia
By hemoglobin electrophoresis
RBC changes often seen by 6 weeks of age
Child presents with severe anemia, FTT
Thalassemia - Therapeutic Management
Blood transfusion to maintain normal Hgb levels
Side effect—hemosiderosis (iron overload)
Treat with iron-chelating drugs such as deferoxamine
Binds excess iron for excretion by kidney
IV or SQ over 8-10 hours multiple times/wk
May be given at home with IV pump per parents
New oral chelation drugs—deferasirox
Worldwide use
Also give oral vitamin C to facilitate binding of iron
thalssemia - Prognosis - what is a potential cure?
Retarded growth
Delayed or absent secondary sex characteristics
Expect to live well into adulthood with proper clinical management
Bone marrow transplant is potential cure
ANEMIAS CAUSED BY IMPAIRED OR DECREASED PRODUCTION OF RED BLOOD CELLS - think bone
Bone marrow fails to produce RBCs
Leukemia or other malignancy
Chronic renal disease
Collagen diseases
Hypothyroidism
APLASTIC ANEMIA
(a plastic is A for all forms)
All formed elements of the blood are simultaneously depressed—“pancytopenia”
Hypoplastic anemia—profound depression of RBCs but normal WBCs and platelets
Aplastic Anemia (cont’d)
Etiology
Primary (congenital)
Secondary (acquired)
Diagnostic evaluation
Therapeutic management
Bone marrow transplant
Stem cell transplant (HSCT)
Immunosuppressive therapy
Nursing considerations
hemophelia - Blood Clotting- 3 basic steps for clotting to occur
(clotting factors, thrombin, fibrin)
3 basic steps for clotting to occur:
Release of clotting factors from both injured tissue cells and sticky platelets at the injury site (which form a temporary platelet plug)
Series of chemical reactions that eventually result in the formation of thrombin
Formation of fibrin and trapping of red blood cells (RBCs) to form a clot
Types of Hemophilia - Hemophilia A
Hemophilia A
Classic hemophilia
Deficiency of factor VIII
Accounts for 80% of cases of hemophilia
Occurrence—1 in 5000 males
Hemophilia B
Also known as Christmas disease
Caused by deficiency of factor IX
Accounts for 15% of cases of hemophilia
Etiology of Hemophilia A
X-linked recessive trait
Males are affected
Females may be carriers
Degree of bleeding depends on amount of clotting factor and severity of a given injury
Up to one third of cases have no known family history
In these cases disease is caused by a new mutation
hemophelia - Clinical Manifestations
Bleeding tendencies range from mild to severe
Symptoms may not occur until 6 months of age
Mobility leads to injuries from falls and accidents
Hemarthrosis
Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility
Ecchymosis
Epistaxis
Bleeding after procedures
Minor trauma, tooth extraction, minor surgeries
Large subcutaneous and intramuscular hemorrhages may occur
Bleeding into neck, chest, mouth may compromise airway
hemophelia - Clinical Therapy- how can it be diagnosed?
Can be diagnosed through amniocentesis
Genetic testing of family members to identify carriers
Diagnosis on basis of history, labs, and exam
Lab tests
Coagulation screen shows normal PT with prolonged PTT
Factor VIII decreased in A and normal in B
Factor IX decreased in B and normal in A
Platelet aggregation and count normal in both A and B
hemophelia - Therapeutic Management
Replace missing clotting factors ASAP
DDAVP (vasopressin)
Synthetic vasopressin analog
IV
Minimal antidiuretic effect
Increases in factor VIII x4
No effect on factor IX
Used for mild hemophilia
Transfusions
At home with prompt intervention to decrease complications
Following major or minor hemorrhages
hemophelia - prognosis
Historically, most died by age 5 years
Now those with mild to moderate hemophilia live near-normal lives
Gene therapy for the future
Infuse carrier organisms into patient; these act on target cells to promote manufacture of deficient clotting factor
hemophelia - Interventions
Close supervision and safe environment
Age appropriate
Infants pad side rails
Toddlers wear helmets
Children wear protective sports gear
Dental procedures in controlled situation
Shave only with electric razor
Superficial bleeding—apply pressure for at least 15 minutes and ice to vasoconstrict
If significant bleeding occurs, transfuse for factor replacement ASAP
Idiopathic Thrombocytopenic Purpura (ITP) (break down the word)
(P for penic, P for platelets)
An acquired hemorrhagic disorder characterized by
Thrombocytopenia—excessive destruction of platelets
Purpura—discoloration caused by petechiae beneath the skin
Normal bone marrow with unusual increase in large immature platelets
ITP Forms - just acute and chronic
Acute, self-limiting
Often follows URI or other infection
Chronic (more than 6 months’ duration)
THE IMMUNE SYSTEM
Protects body from foreign invaders
Bacteria, viruses, parasites, fungus
Immune system organs and tissue
Lymph nodes
Small oval-shaped structures that run along channels
Filter lymphatic fluid and return it to bloodstream
Thymus
Located in mediastinal area
uses hormones to mature lymphocytes
Spleen
Acts as reservoir and filter of RBCs
Tonsils
Storage site for lymphocytes
IMMUNE SYSTEM - Immune system cells - made where?
(immune marrow)
Immune system cells
Made in the bone marrow
B cells
Travel in blood and lymph
IMMUNE SYSTEM (cont’d)
Complement system
Primary defense system
Proteins responsible for the inflammatory response
Activated by antigen-antibody complexes
Toxins released by antigens
Hypersensitivity
Atopy or anaphylaxis (wheezing, hives, rhinorrhea to cardiac arrest)
Cytotoxic response (transfusion reactions)
Immune complex (lupus, JIA, glomerulonephritis)
Cell-mediated hypersensitivity (transplant rejection or dermatitis from plant exposure)
cytotoxic
(toxic transfusion)
transfusion reaction
HIV+/AIDS
Etiology
Born to infected mothers, breast-feeding from infected mother, tainted blood transfusion
Pathophysiology
HIV virus invades T cells making them nonfunctional
Suppresses cell-mediated and humoral immunity
Immunosuppression progression results in opportunistic infection and death
Diagnostics
Incubation period 17 mo
ELIZA (enzyme-linked immunosorbent assay)
Must re-check and then confirm with other c-cell tests
Common Clinical Manifestations of HIV Infection in Children
Lymphadenopathy
Hepatosplenomegaly
Susceptible to fungal infections
Oral candidiasis
Repeated bacterial infections such as OM (don’t respond to antibiotics)
Chronic or recurrent diarrhea
Failure to thrive
Developmental delay
Parotitis
Common AIDS-Defining Conditions in Children
Pneumocystis carinii pneumonia
Lymphoid interstitial pneumonitis
Recurrent bacterial infections
Wasting syndrome
Candidal esophagitis
HIV encephalopathy
CMV
Cryptosporidiosis
Severe Combined Immunodeficiency Disease (SCID)
Absence of both humoral and cell-mediated immunity
Pathophysiology
Graft-versus-host reaction
Therapeutic management
Nursing considerations
LEUKEMIAS
ALL is most common form of childhood cancer
3 or 4 cases per 100,000 white children younger than 15 years old
More frequent in males older than 1 year
Peak onset between 2 and 6 years old
Survivability
Pathophysiology - leukemia- which organs are mostly affected?
Leukemia is an uncontrolled, overproduction of WBCs by the stem cells in the bone marrow
Nonfunctional leukemic cells infiltrate body tissue and replace normal cells
Crowd out healthy cells depleting nutrition needed for metabolism
Liver and spleen most severely affected organs
Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count
Cellular destruction takes place by infiltration and subsequent competition for metabolic elements
Consequences of Leukemia
Anemia from decreased RBCs
Infection from neutropenia
Low functioning WBC’s
Bleeding tendencies from decreased platelet production
Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosis
leukemia - Diagnostic Evaluation
Based on history, physical manifestations
Peripheral blood smear
Immature leukocytes
Frequently low blood counts
LP to evaluate CNS involvement
Bone marrow aspiration or biopsy
Therapeutic Management - leukemia
Chemotherapeutic agents
Cranial irradiation (in some cases)
Supportive treatment
Antibiotics, antifungals, antivirals
Colony stimulation factors (Neupogen)
spurs growth of granulocytes, RBC’s, platelets
Platelet transfusions to prevent bleeding and anemia
Bone marrow transplant
leukemia - Four Phases of Therapy
(IPCM)
Induction therapy
CNS prophylactic therapy
Intensification (consolidation) therapy
Maintenance therapy
Managing Problems of Drug Toxicity - leukemia
Nausea/vomiting
Anorexia
Mucosal ulceration
Neuropathy
Hemorrhagic cystitis
Alopecia
Mood changes
Moon face
Allogeneic Bone Marrow Transplant (BMT) - just a transplant
Involves the matching of a histocompatible donor with the recipient
Limited by the presence of suitable marrow donor
Umbilical Cord Blood Stem Cell Transplantation
Rich source of hematopoietic stem cells for use in children with cancers
Stem cells can be found with high frequency in circulation of newborns
The benefit of umbilical cord blood is the blood’s relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful
Peripheral Stem Cell Transplants (PSCT)
A type of autologous transplant
Different type of collection from the patient
Colony-stimulating factor given to stimulate production of many stem cells; then collected by “apheresis” machine
Stem cells are separated from whole blood
Remaining blood cells and plasma are returned to the patient after apheresis
Stem cells frozen for later transfusion to the patient
Umbilical Cord Blood Stem Cell Transplantation
Stems cells in high frequency in umbilical cord blood
Less problematic for transplant match with blood relative of newborn
Source for hematopoiesis (blood cell production process)
cancer from which cell is easier to treat?
lymphocitic because it’s just one cell - just WBC
baby after 6-8 weeks if hemoglobin F isn’t working
they will become anemic bc the RBC only lives for 90 days in a baby
more nosebleeds at night why?
air conditioners and heaters
enlarged liver
car accidents, hits, mono
HIV in kids
more diaper rash and ear infections
types of anemia - NORMACYTIC (HYPOCHROMIC)
(normal lead)
RBC of normal size, low hemoglobin
Lead poisoning
types of anemia - MICROCYTIC (HYPOCHROMIC)
(micro iron)
RBC of small size, low hemoglobin
Iron deficiency
types of anemia - MACROCYTIC (HYPOCHROMIC)
(large is pernicous)
RBC of large size, low hemoglobin
Vitamin B12 deficiency (Pernicious Anemia), Malabsorption syndrome
transfusion reactions - hypo or hyperkalemia?
Electrolyte disturbances—hyperkalemia from massive transfusions or with renal problems
delayed blood transfusion reactions - RBCs?
Destruction of RBCs and fever 5 to 10 days after transfusion
Observe for post-transfusion anemia
is milk good for iron deficiency anemia?
Milk is a poor source of iron
Iron-fortified cereals
foods with iron for older kids
Older kids increase iron intake
Red meat, organ meat, legumes, green leafy veggies, raisins, dried apricots, iron fortified cereals
Supplemental Iron
Used for HCT below 34% (HGB <11.3 g/dl)
Not easily absorbed
where does blood form in utero?
In utero blood forms in liver and spleen
In infants and young children in bone marrow of all bones
where does blood form in adolescence?
By adolescence (bone growth ceases) so only made in ribs, sternum, vertebrae, pelvis
Blasts
Primitive blood cells
Become RBC’s, WBC’s, platelets
Mature in the bone marrow
“Differentiation “ occurs in stages
types of sickle cell crisis - Hemolytic
(heman is yellow)
Hemolytic (complication of dz)
Degenerative changes cause liver congestion and chronic jaundice worsens
phases of leukemia - Induction therapy - how long?
Induction therapy—4 to 6 weeks
phases of leukemia - CNS prophylactic therapy
CNS prophylactic therapy—intrathecal (between brain and spinal cord) chemotherapy
phases of leukemia - Intensification (consolidation) therapy
Intensification (consolidation) therapy—to eradicate residual leukemic cells and prevent resistant leukemic clones
phases of leukemia - Maintenance therapy
Maintenance therapy—to preserve remission
Humoral immunity - does what?
(humor my antibodies)
Humoral immunity (produce antibodies)
B cells divide into plasma cells when they meet triggering antigen
Plasma cells secrete antibodies to antigen
immunoglobulin
Phagocytes
Engulf, kill, digest particulate matter of invaders
Activate T cells
Secrete clotting factor, enzymes, regulatory molecules
T cells - what about transplants?
T cells
Cell mediated immunity
Attack antigens, bacteria, viruses, pathogens
Reject incompatible tissue and transplant
