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Lecture 4: Hematological/Hereditary Disorders
Terms in this set (73)
Increased level of WBCs. This is a normal response but if the elevation persists when need is decreased, it needs to be investigated.
Which WBC is elevated in infection or trauma
Which WBC is elevated in parasitic disease or allergic reactions
Which WBC is increased in viral infection or hemolytic disorders
___________ is a significant cause of leukocytosis.
Malignancy - the production of blood cells are carefully regulated, if this process is disrupted, the cells can proliferate dangerously.
Broad term describing a group of malignant diseases that affect the blood and blood forming tissues of the bone marrow, lymph system, and spleen.
Malignant neoplasms that originate in the bone marrow and lymphatic structures causing proliferation of lymphocytes
Name two types of lymphomas
1. Hodgkin's disease
2. Non-Hodgkin's lymphoma
Malignancy of the most mature form of the B lymphocyte (the plasma cell). Neoplastic plasma cells infiltrate the bone marrow and destroy bone. It is not classed as a lymphoma.
Common Features of the blood/lymph cancers
- they are all unregulated proliferation of WBCs in the bone marrow.
- in acute forms or late stages of chronic forms, proliferation of leukemic cells leave little room for normal cell production.
- extramedullary hematopoiesis can also cause proliferation
What causes leukemia?
The cause of leukemia is not fully known, some evidence indicates that it is influenced by genetics, viral pathogenesis, bone marrow damage from radiation, exposure from alkylating agents or benzene.
How are leukemias classed?
Acute or chronic - they are classed based on onset of symptoms and phase of cell development that is halted.
If a leukemia has an abrupt onset (a few weeks), the WBCs are undifferentiated (or are blasts), and progresses rapidly with death occuring within weeks-months without treatment, is it most likely acute leukemia or chronic?
If a leukemia occurs over months-years, the WBCs produced are mature, and it progresses slowly with the trajectory taking years, is it most likely acute leukemia or chronic?
Main cells of the immune system, control intensity and specificity of the immune response
Name the 3 main types of lymphocytes
B lymphocytes, T lymphocytes, and natural killer cells
A relatively rare malignancy. Makes up 12% of all lymphoma. Originates in the B lymphocytes.
In 2008, approx. 900 people will be diagnosed with Hodgkin's lymphoma in Canada.
True or False: is Hodgkin's disease unicentric in origin (initiates in a single node)?
When are you most likely to get Hodgkin's disease?
Early 20s and after 50
What is the cause of Hodgkin's disease?
The cause is unknown, however, viral etiology is suspected. Fragments of Epstein-Barr virus have been found in 40-50% of especially the younger patients. There is a familial pattern.
What is the malignant cell of Hodgkin's disease?
The Reed-Sternberg cell is the malignant cell of Hodgkin's disease. Its presence is the hallmark for Hodkin's disease and it is an essential diagnostic criterion. It is a unique gigantic tumor cell.
Who are most likely to get Hodgkin's disease: men or women?
Men - it is twice as prevalent in men as in women.
Clinical manifestations of Hodkin's disease
- Usually begins as a painless enlargement of the lymph nodes on one side of the neck
- Pain after ETOH
- Pain at the site of disease
All organs vulnerable to invasion - symptoms result from compression of the organs by the tumor:
- Jaundice - common bile duct obstruction
- Cough/pulmonary effusion - pulmonary infiltrates
- Abdominal pain - splenomegaly
- Bone pain - skeletal involvement
What is usually the first indicator of someone with Hodgkin's disease?
Painless enlargement of lymph nodes on one side of the neck
Treatment for Hodgkin's disease
- Chemotherapy and radiation
- Bone marrow transplant or stem cell transplant
Originates from neoplastic growth of lymphoid tissue. Thought to arise from a single clone of lymphocytes although cells may differ morphologically.
What is different about non-Hodgkin's lymphoma compared to Hodgkin's lymphoma?
In non-Hodgkin's lymphoma, the lymphoid tissues involved are largely infiltrated with malignant cells. Spread of these cells is unpredictable. Lymph nodes from multiple sites may be infiltrated.
What is the average age of diagnosis of non-Hodgkin's lymphoma?
True or false: in non-Hodgkin's lymphoma, the incidence increases with each decade of life
What causes non-Hodgkin's lymphoma?
There is no common etiologic factor identified however, immunodeficiencies, autoimmune disorders, viral infections (Epstein-Barr virus & HIV), and exposure to pesticides, solvents or dyes have all been seen.
Clinical manifestations of non-Hodgkin's lymphoma
May be minor or absent until disease progression is extensive. Constitutional or B symptoms include:
- recurrent fever
- drenching in night sweats
- weight loss of more than 10% of body weight
Treatment of non-Hodgkin's lymphoma
- Nonaggressive & localized forms - radiation only
- Intermediate forms - chemotherapy & radiation
- Aggressive forms - aggressive combinations of chemotherapies
- Bone marrow transplant is possible for patients younger than 60 years old
What is the most significant consideration a nurse must make when treating someone with non-Hodkin's lymphoma?
Risk for infection from treatment related to myelosuppression and defective immune response
With multiple myeloma, what is a marker (lab value) to determine the extent of disease?
Increased total protein level- because the malignant plasma cells increase the amounts of a specific immunoglobulin (monoclonal protein or M protein) the total protein levels are increased.
Clinical manifestations of multiple myeloma
- Bone pain - back/ribs common. Unlike arthritic pain, this bone pain worsens with activity and decreases with rest.
- Fractures are common because substances secreted by the plasma cell contribute to bone breakdown. The bone breakdown is severe enough to cause spinal fractures which can impinge the spinal cord and cause spinal compression.
- Hypercalcemia can result from extensive bone breakdown. Symptoms of this are thirst, dehydration, constipation, altered mental status, confusion, and even coma
- Renal failure may also occur related to damage to the renal tubules from the configuration of the circulating immunoglobulin.
- Anemia can develop because bone marrow has less space for RBC production or it can be caused by decreased erythropoietin by kidneys.
Which malignancy of the white blood cells can cause fractures and hypercalcemia?
Which malignancy of the white blood cells can cause pain that worsens with activity and decreases with rest?
Which malignancy of the white blood cells can cause pain after ETOH consumption?
Which malignancy of the white blood cells has these three typical symptoms: night sweats, fever, and weight loss of more than 10% of body weight?
Which malignancy of the white blood cells can cause pruritis?
When are you most likely to be diagnosed with multiple myeloma?
Median age of diagnosis is 68 for men and 70 for women.
Treatment for multiple myeloma
There is no cure for multiple myeloma. Currently, bone marrow transplant or stem cell transplant is an option to prolong remission but it usually is not an option for most people due to concurrent organ dysfunction related to age.
Back pain should be investigated for ____________ in the elderly population especially when there is an increase in total protein levels.
Typically, platelets live ____-____ days
Platelet pooling occurs in the ___________
Spleen - platelets can be released when needed. The spleen typically stores 1/3 of circulating platelets.
At which temperature is a fever considered an emergency with someone who is receiving chemotherapy?
37.4-37.6 is significant because of their immune system is decreased and therefore the increase of temperature related to infection is extremely slow.
Low platelet level
Thrombocytopenia can be caused by:
- A decreased production of platelets
- An increased destruction of platelets
- An increased consumption of platelets
A decreased production of platelets occurs with which conditions?
- Hematologic malignancy
- Metastatic involvement from bone marrow
An increased destruction of platelets is caused by which conditions?
- Immune thrombocytopenia (ITP)
- Malignant lymphoma
An increased consumption of platelets occurs with which condition?
Disseminated intravascular coagulation (DIC)
A common clinical manifestation of thrombocytopenia is _________
What is the treatment for thrombocytopenia if platelet production is impaired and bleeding is occuring?
Platelet transfusion - may raise platelets and stop bleeding
What is the most common cause of a destruction of platelets?
Immune thrombocytopenia (ITP)
Which type of ITP occurs predominately in children, appears 1-6 weeks after a viral infection, and is self-limiting with remission often occurring within 6 months
Which type of medications can induce ITP?
Which conditions can induce ITP?
Lupus and pregnancy
In ITP, platelet survival is reduced to ____-____ days
Treatment of ITP
- If caused by medication - stop it
- Immunosuppressants such as prednisone
- IV immunoglobulin
- Platelet transfusion - ONLY IF EMERGENCY because the platelets will be destroyed anyways
Why is a splenectomy a treatment option for ITP?
1. Spleen contains +++ macrophages that destroy platelets
2. Antiplatelet antibodies decrease after splenectomy
3. Spleen impounds 1/3 of platelets therefore its removal would increase the number in circulation
4. The structure of the spleen enhances activity between macrophages & antibody coated platelets
Why is a platelet transfusion not a good treatment option for ITP?
In ITP, there is a destruction of platelets. If you transfuse platelets, the new platelets will only be destroyed anyways. A platelet transfusion would only be a good option if the patient's platelets were extremely low and the patient was actively bleeding and clotting was necessary right away.
Hemophilia A results from a defective or deficient coagulation factor __________
Hemophilia B results from a defective or deficient coagulation factor __________
With hemophilia, who is affect more often: men or women?
Men are affected more. In fact, it is almost always men who get this condition because it is an x-linked trait.
____________ is the most common congenital bleeding disorder
Von Willebrand's Disease
What is Von Willebrand's diseased caused by?
Von Willebrand's disease is caused by a deficiency in Von Willebrand's factor which is necessary for factor VIII and platelet adhesion at the site of a vascular injury
How is factor VIII affected in Von Willebrand's disease?
Factor VIII synthesis is normal but the half life is shortened, therefore factor VIII levels are usually low (15-50% of normal)
What are clinical manifestations of Von Willebrand's disease?
Nosebleeds, excessively heavy menses, bleeding from cuts, postoperative bleeding
How is Von Willebrand's disease treated?
- Factor deficiency and platelet impairment can be corrected by cryoprecipitate (contains factor VIII)
- Fresh frozen plasma can be used if cryo is not available
- DDAVP (desmopressin) is a synthetic vasopressin analog that can be used to prevent bleeding in dental surgery. It causes a transient increase in factor VIII
What is DDVAP (desmopression) used for?
DDAVP (desmopressin) is a synthetic vasopressin analog that can be used to prevent bleeding in dental surgery. It causes a transient increase in factor VIII. It can also be used for diabetes insipidus.
Principles of Primary Health Care
1. Universal access to health care services
2. Focus on the determinants of health
3. Active participation of the individual and communities in decisions that affect health
4. Partnerships with other disciplines i.e. nutrition services, social services, cardiac wellness clinic
5. Appropriate use of knowledge, skills, strategies, technology, and resources
6. Focus on health promotion - illness prevention across the life span
Normal platelet level
A client with multiple myeloma becomes confused and lethargic. Which of the following explain these clinical manifestations?
d. CNS myeloma
Other sets by this creator
Lecture 5: Assessment of GI Function
Lecture 3: Hematological/Hereditary Disorders
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