Blood & Lymph Fall 2006
Terms
undefined, object
copy deck
- When is sickle cell disease diagnosed?
- After age 4 months
- What type of hemoglobin do well people have?
- HgbA
- What type of hemoglobin do infants have?
- HgbF
- What type of hemoglobin is found in a person who has sickle cell disease?
- HgbS
- Explain why sickle cell disease affects males and females alike:
- It is autosomal: the affected gene is located on one of the 1st 22 pairs of chromosomes that do not determine gender
- If a child inherits only one abnormal "sickle cell" gene, does he or she have the disease?
- No; only the trait
- What type of disorder is sickle cell disease classified as?
- Autosomal, recessive disorder
- In order for a child to inherit sickle cell disease, what must happen?
- Both parents must have the defective genes
- What is the lifespan of a sickled red blood cell?
- 7 - 20 days
- What is the lifespan of a normal red blood cell?
- 120 days
- What can cause sickling of the RBC's?
-
*Dehydration
*Acidosis
*Infection
*Hypoxia
*Venous stasis
*Strenuous exercise
*Anesthesia - What are the symptoms of sickle cell?
-
*Growth retardation
*Chronic anemia
*Susceptibility to sepsis
*Delayed sexual maturation
*Fever
*Pain - What is the most common and most distressing symptom of sickle cell?
- Pain
- What is priapism?
- A nocturnal penile erection that lasts as long as 4 or more hours. Very painful
- How is priapism treated?
-
*Estrogens
*Vasodilators - What are reticulocytes?
- Immature RBC's
- What is the WBC count like in a patient with sickle cell?
- Elevated
- What is the total bili count like in a patient with sickle cell?
- Elevated
- What is the sickle cell turbidity test?
- A screening tool only
- How is sickle cell disease determined for sure?
- Hemoglobin electrophoresis
- How are results from a hemoglobin electrophoresis test interpreted?
-
*Less than 40% HgbS = Trait
*More than 40% HgbS = Disease - What is the most common sickle cell crisis?
- Vaso-occlusive crisis
- Which sickle cell crisis causes lung/chest pain in the elderly?
- Vaso-occlusive crisis
- Which sickle cell crisis causes painful swelling of the hands/ feet and extreme pain in infants?
- Vaso-occlusive crisis
- Which sickle cell crisis commonly affects the knees/back of adults, and can cause infarcts of the small bones?
- Vaso-occlusive crisis
- Which sickle cell crisis is also known as splenic sequestration?
- Sequestration crisis
- Describe sequestration crisis:
-
*Blood is blocked into spleen
*Spleen enlarges
*Spleen can become scarred/useless - Why can a patient become "shocky" during a sequestration crisis?
- Blood is trapped in the spleen and therefore not circulating throughout the body
- In patients with sequestration crisis, what can happen by age 1?
- Spleen can lose up to 29% of function
- In patients with sequestration crisis, what can happen by age 2?
- Spleen can lose greater than 40% of functionality
- What is G6PD stand for?
- Glucose 6 Phosphate Deficiency
- What type of sickle cell crisis is caused by G6PD?
- Hyperhemolytic Crisis
- What does G6PD's function in the body?
- It is an enzyme that stimulates the RBC's (gives them energy)
- How much fluid intake is encouraged for patients who have sickle cell disease?
- 4-6 quarts per day
- How much fluid intake is encouraged for patients who are in sickle cell CRISIS?
- 6-8 quarts per day
- List some drugs that are used to manage pain in sickle cell patients:
-
*Methadone
*Morphine
*Hydroxyuria
*Dilaudid
*Fentanyl patch - What treatment for sickle cell disease is aimed at diluting the HgbS levels?
- Chronic transfusions
- What lab levels should be monitored in a patient who is undergoing chronic transfusions?
-
*Iron
*TIBC
*Ferritin - What should be avoided in patients with sickle cell disease?
-
*Caffeine
*Cold liquids - In the pathophysiology of sickle cell disease, what happens to the Hgb S cell?
- It assumes a sickled appearance
- What happens to the blood's viscosity when the RBC's sickle?
- The viscosity is increased
- True or False: sickled cells increase RBC destruction
- True
- What happens to the oxygen carrying capacity of red blood cells when they are sickled?
- It is decreased
- What happens to circulation when the RBC's are sickled?
- Circulatory stasis occurs
- What is a critical intervention for a patient in sickle cell crisis?
- Bedrest
- What kind of gene is affected in hemophilia A?
- Sex-linked recessive gene
- Why is almost 100% of hemophilia A found in men?
- The gene is linked to the X chromosome, and males only have one X chromosome.
- What clotting factor is deficient in hemophilia A?
- Factor VIII
- What should be taught to parents of sickle cell patients?
-
*Recognizing s/sx
*Charting growth/development
*Give folic acid early
*Give protein supplements
*Get eyes tested early - What is Partial chimerism?
- A transfusion in which a donor's bone marrow is mixed with the patient's bone marrow and administered to the patient
- What clotting factor is deficient in hemophilia B?
- Factor IX
- What clotting factor is deficient in hemophilia C?
- Factor XI
- What is the difference between hemophilia and DIC?
-
Hemophilia: involves only intrinsic clotting factors
DIC: involves both intrinsic and extrinsic clotting factors - What is a good physical activity to suggest for a patient with hemophilia?
- Swimming
- When is hemophilia usually diagnosed?
- In early childhood (when the child begins to walk)
- What is the usual treatment for hemophilia?
- Replacement of coagulation factor that is affected
- What type of deformities are a complication of hemophilia?
- Joint deformities
- What are the signs/symptoms of hemophilia?
-
*Prolonged bleeding anywhere in the body
*Bruising
*Hematuria - What laboratory finding will be abnormal in a patient with hemophilia?
- PTT
- Will the PT lab value be normal or abnormal in a patient with hemophilia?
- Normal
- What is DIC?
- Disseminated Intravascular Coagulation
- How will the PT / INR reflect in lab reports of a patient with DIC?
- Prolonged
- How will the activated partial thromboplastin time (APTT) reflect in lab reports of a patient with DIC?
- Prolonged
- How will the platelet count reflect in lab reports of a patient with DIC?
- Decreased
- How will the fibrinogen levels reflect in lab reports of a patient with DIC?
- Decreased
- How will the fibrinogen degradation product levels reflect in lab reports of a patient with DIC?
- Increased
- How will the D-Dimer test reflect in lab reports of a patient with DIC?
- Increased
- What is the cure for DIC?
- Treat the cause
- True or False: Once you have DIC, you are prone to get it again
- False
- What are some nursing priorities in patients with DIC?
-
*Maintain fluid balance
*Control bleeding
*Restore normal clotting factors - What are some important things to remember when caring for a DIC patient?
-
*No blood pressure cuffs
*Turn carefully
*Be careful with catheters, ET tubes, etc. - What level of D-Dimer proteins indicate DIC?
- Greater than 250 per ml
- Explain erythropoesis:
- Kidneys respond to low O2 and release erythropoetin, which travels to the bone marrow to stimulate RBC production
- What are the 5 types of WBC's?
-
*Neutrophils
*Lymphocytes
*Eosinophils
*Monocytes
*Basophils - What is the largest components of white blood cells?
- Neutrophils
- In regards to WBC's, what indicates a patient's ability to fight infection?
- The number and percentage of neutrophils
- How is the absolute neutrophil count calculated?
-
*Add the neutrophils plus the bands
*Write the sum as a decimal or percentage
*Multiply this number by the total WBC number - With regards to WBC's, what is a "left shift?"
- The number of bands is greater than everything else
- What do the granules of basophils and mast cells release?
- Histamines
- What happens to the lungs when histamines are released?
- Airways swell
- What happens in the nasal passages when histamines are released?
-
*Vasodilation
*Stuffy, runny nose - What is the normal range for platelets?
- 150-440
- What happens to platelets in a patient with sickle cell disease?
- They are increased
- When is AIDS diagnosed?
- When the body loses the ability to fight opportunistic infections
- What must the CD4 count be in order to be classified as AIDS?
- Below 200
- What does the HIV retrovirus have that helps viral replication?
- Reverse transcriptase (RT)
- What does Reverse Transcriptase do?
- Forces DNA to use the virus as a "pattern"
- After the Reverse Transcriptase forces the body's DNA to use HIV as a pattern, what takes place?
- The new viral DNA gets into the body's DNA
- In AIDS, how many viral particles per day can synthesize?
- 2 billion
- What does the HIV virus do to the CD4 receptors?
- Attaches to, infects and kills all immune cells with the CD4 receptors
- What is significant of AIDS as opposed to HIV?
- Profound immunodeficiency
- What is the most common life threatening opportunistic infection in AIDS patients?
- Pneumocystis Carinii (PCP)
- What type of infection is pneumocystis carinii?
- Protozoa and fungal infection
- How is pneumocystis carinii (PCP) transmitted?
- Via air, food, water
- What percentage of AIDS patients have PCP as their first bout of AIDS-related opportunistic infection?
- 60%
- What are the symptoms of pneumocystis carinii (PCP)?
-
*Fever
*Fatigue
*Weight loss
*SOB
*Crackles
*Cough - What is the treatment for pneumocystis carinii (PCP)?
-
*Pentamidine (aerosol)
*Mechanical ventilation
*aerosolized bronchodilators
*TMP-SMX (sulfa compound)
*Steroids - What is a serious side effect of TMP-SMX?
- Nephrotoxicity
- What is the most common malignancy in HIV patients?
- Kaposi's Sarcoma
- Besides skin lesions, what are some other symptoms of Kaposi's Sarcoma?
-
*Diarrhea
*Blockage of lymph leading to edema of face or extremities
*Respiratory distress
*Cognitive changes - What is the treatment for Kaposi's Sarcoma?
-
*Radiation
*Chemotherapy
*Interferon - What is Cryptosporidium?
- A parasite
- How is Cryptosporidiosis transmitted?
- Animal to human or human to human
- What is the most common site of cryptosporidiosis infection?
- Small intestine
- What is the most distressing symptom of cryptosporidiosis?
- Copious explosive diarrhea (15-20 liters/day)
- True or False: cryptosporidium can be killed by bleach
- False
- By what cycle is cryptosporidium transmitted?
- Fecal-oral cycle
- Besides the diarrhea, what are some other symptoms of cryptosporidiosis?
-
*Weight loss
*Dehydration - Is there an effective treatment for cryptosporidiosis?
- No
- What drug can be given to patients with cryptosporidiosis?
- Octreotide (to lessen diarrhea)
- What is significant of Octreotide?
- It has lots of side effects
- What is the screening test that measures HIV antibodies?
- ELISA
- What is the confirming test for HIV?
- Western Blot
- Why is the CD4 count measured in HIV patients?
- This is used to measure the progression of the disease and the effectivity of the medication
- How often should an HIV patient have CD4 counts measured initially?
- Every 3-6 months
- What is the WBC count like in a patient with HIV/AIDS?
- Low
- What does the Viral culture/Viral Load Test measure?
- The genetic material of HIV; this indicates amount of disease progression and effectivity of medication
- How often should an AIDS patient have a Viral Load Test?
- Every 2-8 weeks
- What is the drug regimen for HIV patients who are pregnant?
- ZDV (a combination of 3 drugs)
- When is ZDV therapy started in a pregnant woman with HIV?
- 14-34 weeks gestation
- When is ZDV administered IV in a pregnant woman with HIV?
- During delivery
- ZDV is given in liquid form to babies of HIV mothers at what frequency and duration?
-
*Every 6 hours
*For the 1st 6 weeks of life - How are babies tested for HIV?
- By looking for the virus itself (not the antibodies as in adults)
- ZDV therapy in mothers with HIV is shown to reduce the risk of passing HIV to the baby by what percentage?
- 70%
- When is a c-section delivery recommended for pregnant women with HIV?
-
*If viral load is greater than 1000
*If membranes are ruptured
*If no HIV drug therapy
*if no prenatal care - How often should a room/bathroom be cleaned for immunocompromised patients?
- Every day
- How often should the vital signs be checked in an immunocompromised patient?
- Every 4 hours
- A minor elevation in temperature for an immunocompromised patient can indicate what?
- Sepsis
- How often should the mouth and skin of an immunocompromised patient be assessed?
- Every 8 hours
- How often should open areas/IV sites, etc. be assessed in an immunocompromised patient?
- Every 4 hours
- How often should IV tubing be changed for an immunocompromised patient?
- Every day
- How often should wound dressings be changed in patients who are immunocompromised?
- Every day
- How often should WBC's (esp. ANC) be monitored in immunocompromised patients?
- Every day
- Immunocompromised patients should not drink water that has been standing for how long?
- Longer than 15 minutes
- What do the initials HAART stand for?
- Highly Active Antiretroviral Therapy
- What is HAART?
- A combination of 3 or more anti-HIV medications in a daily regimen
- How do the Nonnucleoside Reverse Transcriptase Inhibitors (NNRTI's) work?
- Bind to and disable reverse transcriptase
- How do Nucleoside Reverse Transcriptase Inhibitors (NRTI's) work?
-
*NRTI's are faulty versions of building blocks HIV needs to make more copies of itself
*When HIV uses an NRTI instead of a normal building block, reproduction of the virus is stalled - How do Protease Inhibitors (PI's) work?
- Disable protease, a protein that HIV needs to make more copies of itself
- How do Fusion Inhibitors work?
- By blocking HIV entry into cells
- What does HIV do to CD4 cells?
- Destroys them
- In the morphology of leukemia, what does "lympho-" indicate?
- Originating from lymphoid or lymphatic system
- In the morphology of leukemia, what does "Myelo-" indicate?
- Originating from bone marrow
- In the morphology of leukemia, what is indicated by the words "blastic" and "acute"?
- Sudden; involves immature white cells
- In the morphology of leukemia, what is indicated by the words "cytic" and "chronic?"
- Over time; involves mature white cells
- To what area/body system is leukemia prone to travel, yet chemotherapy does not reach this area/body system?
- Brain/CNS
- From what cells does Acute Lymphocytic Leukemia (ALL) arise?
- Lymphoblasts
- 80% of ALL diagnoses are what age group
- children
- What is the peak age of diagnosis of ALL?
- 2-4 years
- There is a sharp decline in diagnosis of ALL after what age?
- 10 years
- What percentage of children with ALL can be cured?
- 50%
- ALL is more common in what gender?
- Males
- In patients with ALL, a higher WBC indicates what?
- A poorer prognosis
- In regards to ALL prognosis, which diagnosis age groups do better?
-
*Diagnosed at age 2-9: better prognosis
*Diagnosed before age 2 or after age 10, poorer prognosis - Which gender has a better prognosis with ALL?
- Females
- What should be done for patients who have an ANC of less than 500?
-
*Neutrapenic precautions
*Reverse isolation - What is a normal ANC level?
- Greater than 500
- From what kind of cells does Acute Myelogenous Leukemia (AML) arise?
- Single myeloid stem cell (myeloblast)
- What is a myeloblast?
- Precursor to a granulocyte
- AML causes the development of what in the bone marrow?
- Immature myeloblasts
- At what age does AML occur more frequently?
-
*In adolescence (12-20 years)
*After age 55 - What is the common treatment for AML?
- Bone marrow transplants
- From what type of cells does Chronic Lymphocytic Leukemia (CLL) arise?
- Lymphocytes
- CLL is more common in what age group?
- 50-70 years old
- CLL is more common in which gender?
- Males
- What are the WBC levels like in a patient with CLL?
- High
- What is the survival rate for those diagnosed with CLL?
- 4-5 years
- From what cells does Chronic Myelogenous Leukemia (CML) arise?
- Granulocytes
- Which leukemia has the Philadelphia chromosome marker?
- CML
- What is the survival rate for those with CML?
- 5-6 years
- Which leukemia is associated with "blast crisis"?
- CML
- What is the prognosis for a CML patient in blast crisis?
- 2-4 months
- What is the treatment for CML patients?
- Bone marrow transplant
- What are some symptoms of CML?
-
*Fatigue
*Anorexia
*Splenomegaly
*Weakness
*Weight Loss - How should the nurse explain the pain of bone marrow aspiration procedure?
- "you will experience a brief, sharp pain"
- What is involved in the diagnosis of CML?
-
*Elevated WBC (15,000-500,000)
*Granulocytic hyperplasia in the bone marrow - What should the nurse monitor in leukemia patients in regards to oxygenation and circulation?
-
*Anemia
*Hemorrhage - What is the hallmark for Hodgkin's Disease?
- Reed-Sternberg cell
- What age groups are affected by Hodgkin's Disease?
- Any age group
- What are the major symptoms of Hodgkin's Disease?
-
*Painless enlargement of a lymph node
*Fatigue
*Anorexia
*Unexplained fever
*Night sweats - What are some diagnostic tools for Hodgkin's disease?
-
*Lymph node biopsy
*Chest X-Ray
*CT scan - What is Multiple Myeloma?
- Bone cancer; cancer of the plasma cells
- Multiple myeloma is a WBC cancer that involves what kind of cells?
- More mature cells
- What is the onset of multiple myeloma like?
- Slow and insiduous
- What are the main problems with multiple myeloma?
-
*Pathologic bone fractures
*Renal failure - What are some other symptoms of multiple myeloma?
-
*Back pain
*Paralysis
*Constipation
*Hypercalcemia
*Hyperuricemia
*URI - Is multiple myeloma common?
- No; very uncommon
- At what age is multiple myeloma usually diagnosed?
- Around age 50
- What is a very important treatment for people with multiple myeloma?
- Hydration: approx. 3000 cc's per day
- Besides hydration, what are some other treatment measures for multiple myeloma?
-
*Glucocorticoids
*Calcitonin
*Chemotherapy
*Radiation
*Bone marrow transplant - Which multiple myeloma treatment has a poor prognosis?
- Bone marrow transplant
- What is the percentage of remission with multiple myeloma?
- 50%
- What is the hallmark of multiple myeloma?
- Bence Jones protein
- What type of diet is recommended to prevent calculus formation in a multiple myeloma patient?
- Low calcium
- What lab values should be checked often in multiple myeloma patients?
- BUN/Creatinine