QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
Hematologic disorders
2. The blood and the blood forming sites, including the
bone marrow and the reticuloendothelial system
Blood
› Plasma
› Blood cells
Hematopoiesis
4. Transport of:
› Gases, nutrients, waste products
› Processed molecules
› Regulatory molecules
Regulation of pH and osmosis
Maintenance of body temperature
Protection against foreign substances
Clot formation
5. Antibodies & complements
–part of immune system
Clotting factor
Once activated, converted
to fibrin (threadlike protein
that forms blood clot
6. Liquid part of blood
› Pale yellow made up of 91% water, 9% other
Colloid: Liquid containing suspended substances
that don’t settle out
› Albumin: Important in regulation of water movement
between tissues and blood
› Globulins: Immune system or transport molecules
› Fibrinogen: Responsible for formation of blood clots
7. Erythrocyte: RBC
Leukocyte: WBC
› Neutrophil
› Monocyte
› Eosinophil
› Basophil
› Lymphocyte: T lymphocyte and B lymphocyte
Thrombocyte: platelet
9. Hematopoiesis or hemopoiesis:
› Process of blood cell production
› Fetus: liver, thymus, spleen, lymph nodes, red bone
marrow
› After birth: red bone marrow, for WBC in some
lymphatic tissues
Stem cells (hemocytoblast): All formed elements
derived from single population
› Proerythroblasts: Develop into red blood cells
› Megakaryoblasts: Develop into platelets
11. Structure
› Biconcave, anucleate
Components
› Hemoglobin (makes it red
in color)
› Lipids, ATP, carbonic
anhydrase
Function
› Transport oxygen from
lungs to tissues and
carbon dioxide from
tissues to lungs
13. Consists of:
› 4 globin molecules: Transport carbon dioxide (carbonic anhydrase
involved), nitric oxide
2 alpha and 2 beta globin chains
› 4 heme molecules: Transport oxygen
Each heme contains 1 iron
Iron is required for oxygen transport
› Hemoglobin + oxygen = bright red
› Hemoglobin with no oxygen = darker red
15. Happens in red bone marrow
Components required:
› Precursor cells: PROERYTHROBLAST
› Proper microenvironment
› Adequate supplies of iron, vitamin B12, folic acid, protein,
pyridoxine and traces of copper
Erythropoietin:
› Hormone to stimulate RBC production
16. During cell division, the process requires B
vitamins folate and B12, necessary for synthesis
of DNA.
Iron is required for production of hemoglobin
RBC production is triggered by low blood
oxygen levels
25. IgG
› chronic infxn, transplacental;
IgA
› external secretions; “mucosal paint”
› through breastfeeding
IgM
› acute infxn; 1O response Ab, 1st produced by infants
IgE
› parasitic / allergic reactions
IgD
› no specific function; antigen-binding
28. factor I (fibrinogen)
factor II (prothrombin) – Vit K dependent
factor III (tissue thromboplastin)
factor IV (calcium)
factor V (proaccelerin)
factor VI (no longer considered active in hemostasis)
factor VII (proconvertin) - Vit K dependent
factor VIII (antihemophilic factor)
factor IX (plasma thromboplastin component; Christmas factor) -
Vit K dependent
factor X (stuart factor) - Vit K dependent
factor XI (plasma thromboplastin antecedent)
factor XII (hageman factor)
factor XIII (fibrin stabilizing factor).
30. Spleen
› Left upper quadrant of the abdomen behind abdomen
› Produces RBC during fetal development
› Filter function – remove old defective cells from circulation and
breaks down RBC and recycles the iron to bone marrow
Liver
› Receives blood from the spleen through portal circulation
› Produce procoagulants necessary for hemostasis and blood
coagulation
› Storage of iron and B12
› Detoxify drugs
› Kupffer cells carry out phagocytosis
› Bile production from erythrocyte destruction
31. Lymphatics
Lymph capillaries
› Carries lymph to the subclavian vein in the
chest
Lymph nodes
› Small rounded structures that filter bacteria
and foreign particles
33. Reduction below normal in the # of RBC,
the quantity of hemoglobin and volume of
RBC (hematocrit)
Result from:
› ↓RBC production
› ↑RBC destruction
› Acute or chronic blood loss
34. Three broad categories
1. Loss of RBC- occurs with bleeding
2. Decreased RBC production
› IDA
› FADA
› Thalassemia
3. Increased RBC destruction
35. Normocytic
› Normal RBC size
› IDA
Macrocytic
› Large RBC due to impaired division of RBC precursor cells
› Cobalamin and folate deficiency
Microcytic
› Small RBC
Normochromic
› Normal hemoglobin concentration
Hypochromic
› Decrease hemoglobin concentration
Hyperchromic
› Increased hemoglobin concentration
37. Iron Deficiency Anemia
› dietary intake of iron is inadequate to produce
hemoglobin
› Most common type of anemia
› May occur with removal of the duodenum
› Associated with chronic blood loss.
38. Iron Deficiency Anemia
Etiologic Factors
1. Bleeding- the most common cause
2. Mal-absorption
3. Malnutrition
4. Alcoholism
39. Iron Deficiency Anemia
Pathophysiology
› The body storage of iron decrease, leading
to depletion of hemoglobin synthesis
› The oxygen carrying capacity of
hemoglobin is reduced tissue hypoxia
40. Iron Deficiency Anemia
Assessment Findings
1. Pallor of the skin and mucous membrane
2. Weakness and fatigue
3. General malaise
4. Pica
42. Iron Deficiency Anemia
Laboratory findings
1. CBC
› Low levels of Hct, Hgb and RBC count
2. Low serum iron, low ferritin
3. Bone marrow aspiration- MOST definitive
43. Iron Deficiency Anemia
Medical management
1. Iron replacement
2. Blood transfusion
44. Nursing Management
1. Provide iron rich-foods
› Organ meats (liver)
› Beans
› Leafy green vegetables
› Raisins and molasses
2. Administer iron
› Oral preparations tablets- Fe fumarate, sulfate and
gluconate
› Advise to take iron ONE hour before meals
› Take it with vitamin C
› Monitor for adverse reaction like pyrosis (heartburn),
constipation, diarrhea, dark stool
› Continue taking it for several months even with normal iron
level
45. Nursing Management
2. Administer iron
› Oral preparations- liquid
It stains teeth
Drink it with a straw
› Stool may turn blackish- dark in color
› Advise to eat high-fiber diet and to ↑fluid
intake to counteract constipation
46. Nursing Management
2. Administer iron
› IM preparation
Administer DEEP IM
Include 0.5ml air in syringe to clear iron from the needle
Use the Z-track method
› Avoid vigorous rubbing
› Can cause local pain and staining
Note:
› Iron should be taken for 2-3 mos after Hb level returns to
normal
49. Normally hemoglobin contain 4 globin chains
Decreased production of hemoglobin due to abnormal hemoglobin synthesis
Reduced production or no production of 1 of the globin chains that make up
hemoglobin
Microcytic and hypochromic
Autosomal recessive genetic disorder common among Mediterranean people.
Chronic bone marrow hyperplasia
Altered globin synthesis of hemoglobin
Treatment causes chronic iron toxicity
50. Thalassemia minor
› Has 1 thalassemic and 1 normal gene with
mild clinical manifestations
› Requires no tx
Thalassemia major
› Has 2 thalassemic genes causing severe
conditions
54. Nursing Management
1. Administer BT and chelation therapy (remove heavy
metals) to reduce iron overload
2. Monitor for transfusion reactions or diseases
acquired through transfusions
3. Instruct about tx, medications and physical energy
conservation techniques
4. Strengthen client support and family systems.
56. Life-threatening stem cell disorder with many possible
etiological mechanisms
Decreased number of RBC as well as WBC and
platelets
Characterized as:
› hypoplastic( incomplete development of a tissue or
organ)
› Fatty bone marrow
› Pancytopenia (reduction in # of RBC, WBC and platelets)
61. LABORATORY FINDINGS
1. CBC
› decreased blood cell numbers
2. Bone marrow aspiration confirms the
anemia- hypoplastic or acellular marrow
replaced by fats
62. Medical Management
1. Bone marrow transplantation
2. Immunosupressant drugs
3. Rarely, steroids
4. Blood transfusion
63. Nursing management
1. Assess for signs of bleeding and infection
2. Pancytopenia plan of care to prevent
complications like infection and bleeding.
1. Private room
2. Strict hand washing
3. Minimizing invasive procedures
3. Provide client and family with support for
lengthy hospitalization and tx
65. abnormally large RBC secondary to
impaired DNA synthesis
due to deficiency of Folic acid and/or
vitamin B12
66. 1. Folic Acid deficiency
› Folate is required for DNA synthesis in erythrocyte formation
› Without neurologic involvement
Causative factors
1. Alcoholism
2. Mal-absorption
3. Diet deficient in uncooked vegetables
4. Use of oral contraceptives
67. Pathophysiology of Folic acid deficiency
Decreased folic acid
↓
impaired DNA synthesis in the bone marrow
↓
impaired RBC development
impaired nuclear maturation but Cytoplasmic maturation continues
↓
large size
68. Assessment (Folate Deficiency)
1. Poor nutrition
2. Alcohol abuse
3. Anorexia
4. Impaired absorption in the small intestine
5. Undergoing hemodialysis (folate is dialyzable)
6. Certain drugs can block folate absorption
1. Oral contraceptives
2. Antiseizure drugs (phenytoin)
3. Antibiotics
70. Nursing Management
1. Promote compliance with replacement therapy
(1-5 mg oral folate/day)
2. Administer prenatal vitamins in pregnancy
3. Instruct on food high in folate such as leafy
greens, liver, citrus fruits, nuts and grains
73. Vitamin B12 deficiency
(Pernicious Anemia)
› Lack of intrinsic factor (needed for B12 absorption)
› Common among Northern European ancestry over age of 40 and young African
American
Causative factors
1. Strict vegetarian diet
2. Gastrointestinal malabsorption
3. Crohn's disease
4. Yrs of Gastritis
5. Gastrectomy
74. Vitamin B12 deficiency
Pernicious Anemia
Due to the absence of intrinsic factor secreted by
the parietal cells
Intrinsic factor binds with Vit. B12 to promote
absorption
75. Assessment findings
1. Weakness
2. Fatigue
3. Neurologic manifestations are present only in
Vit. B12 deficiency
1. Weakness, paresthesia of feet and hands, impaired
thought processes
4. Coagulation deficiencies
77. Laboratory findings
1. Peripheral blood smear- shows giant RBCs, WBCs with giant
hypersegmented nuclei
2. Very high MCV
3. Schilling’s test
› Radioactive vit B12 malabsorption is measured by small amount of
secreted in urine
› When vit B12 us administered with gastric IF parenterally, its absence in
urine will diagnose pernicious anemia
4. Intrinsic factor antibody test
78. Medical Management
1. Vitamin supplementation
Folic acid 1 mg daily
2. Diet supplementation
Vegetarians should have vitamin intake
3. Lifetime monthly injection of IM Vit B12
› w/o cobalamin replacement, patient can die in 1-3yrs.
79. Since Pernicious anemia results from an
inability to absorb cobalamin, dietary
intake of the vitamin is not a treatment
option, nor is bone marrow transplant
80. Nursing Management
1. Monitor patient
2. Provide assistance in ambulation
3. Oral care for tongue sore
4. Explain the need for lifetime IM injection of vit B12
82. Decrease in erythrocyte precursor
production that occurs in some chronic
conditions
End-stage renal failure
Chronic liver disease
Alcohol abuse
hypothyroidism
85. Nursing Management
1. Facilitate diagnosis and tx if underlying, contributory
condition
2. Inform that this type of anemia does not respond to
folic acid, iron or vitamin B12
3. Erythropoietin therapy is administered to a client
with anemia related to renal failure
87. 2 SITES OF HEMOLYSIS
1. INTRAVASCULAR DESTRUCTION
› Occurs within the circulation
2. EXTRAVASCULAR DESTRUCTION
› Occurs in liver, spleen, or bone marrow
89. CLASSIFICATION OF HEMOLYTIC ANEMIAS
2. EXTRINSIC HEMOLYTIC ANEMIAS
› Normal RBCs are damaged by external factors
› Cause:
antibodies, toxins,
mechanical injury (prosthetic heart valves)
dialysis,
transfusion reaction
trapping of cells within the liver and spleen
92. A severe chronic incurable hemolytic anemia that results from
heritance of the sickle hemoglobin gene.
Produces specific mutant form of beta-globin
Hypoxia-induced change in RBCs
Associated with vascular occlusion and tissue infarction
Anemia is caused by accelerated breakdown of abnormal RBC
93. Causative factor
› Genetic inheritance of the sickle
gene- Hbs gene
94. Pathophysiology
Decreased O2, Cold, Vasoconstriction can
precipitate sickling process
95. Pathophysiology
Factors
↓
cause defective hemoglobin to acquire a rigid, crystal-like C-
shaped configuration
↓
Sickled RBCs will adhere to endothelium
↓
pile up and plug the vessels
↓
ischemia results
↓
pain, swelling and fever
97. Assessment Findings
1. jaundice
2. enlarged skull and facial bones
3. tachycardia, murmurs and cardiomegaly
98. Assessment Findings
Primary sites of thrombotic occlusion: spleen,
lungs and CNS
Chest pain, dyspnea
99. Assessment Findings
1. Sickle cell crises
› Results from tissue hypoxia and necrosis
2. Acute chest syndrome
› Manifested by a rapidly falling hemoglobin
level, tachycardia, fever and chest infiltrates in
the CXR
100. During the sickling crisis,
the sickling cells clog small capillaries and the resulting hemotasis
promotes a self-prepetuating cycle of local hypoxia,
deoxygenation of more erythrocytes and more sickling.
MEDICAL MGT:
Administration of large doses of narcotic analgesics
Since pain last 4-6 days
102. Nursing Management
1. manage the pain
› Support and elevate acutely inflamed joint
› Relaxation techniques to reduce metabolic
needs
› analgesics
103. Nursing Management
2. Prevent and manage infection
› Monitor status of patient
› Initiate prompt antibiotic therapy
104. Nursing Management
3. Promote coping skills
› Provide accurate information
› Allow patient to verbalize her concerns about
medication, prognosis and future pregnancy
105. Nursing Management
4. Monitor and prevent potential
complications
› Provide always adequate hydration
› Avoid cold, temperature that may cause
vasoconstriction
107. Nursing Management
4. Monitor and prevent potential complications
Priapism
Sudden painful erection
Instruct patient to empty bladder, then take a warm
bath
5. Maintain F & E balance to reduce blood
viscosity
109. Glucose-6-phosphate dehydrogenase
Results from lack of an RBC enzyme that
leads to RBC damage when metabolic
needs of RBCs are increased.
110. A group of X-linked familial hemolytic mutations of the
gene
Deficiency predisposes to oxidative denaturation of
hemoglobin, with resultant red cell injury and lysis.
Hemolysis occurs as the result of oxidative stress generated
by either an infection or exposure to certain drugs.
More common among males of Mediterranean or African
descent
112. Diagnostic:
Use of G6PD assay or screening test
Quantification of G6PD levels during
nonhemolytic phase
113. Nursing Management
1. Provide periods of rest
2. Provide adequate hydration
3. Administer antibiotics and other tx as
ordered to minimize complications.
115. Refers to an INCREASE volume of RBCs
The hematocrit is ELEVATED to more than
55%
116. POLYCYTHEMIA VERA
› Primary Polycythemia
A proliferative disorder in which the
myeloid stem cells become uncontrolled
Neoplastic disorder where there is an
increased in 3 types of blood cells
118. POLYCYTHEMIA VERA
Pathophysiology
› The stem cells grow uncontrollably
› The bone marrow becomes HYPERcellular and all the
blood cells are increased in number
119. POLYCYTHEMIA VERA
Pathophysiology
› The spleen resumes its function of hematopoiesis and
enlarges
› Blood becomes thick and viscous causing sluggish
circulation
› Overtime, the bone marrow becomes fibrotic
120. POLYCYTHEMIA VERA
Assessment findings
1. Skin is ruddy, pruritic due to histamine release
2. Splenomegaly in primary PV only
3. headache, tinnitus
4. dizziness, blurred vision
5. Angina, intermittent claudication, dyspnea and
thrombophlebitis
121. POLYCYTHEMIA VERA
Laboratory findings
1. CBC- shows elevated RBC mass
2. Normal oxygen saturation
3. Elevated WBC and Platelets
122. POLYCYTHEMIA VERA
Complications
1. Increased risk for:
1. Thrombophlebitis
Due to hypervolemia & hyperrviscosity,
Tx: active/passive leg exercise and ambulation
2. CVA
3. MI
2. Bleeding due to dysfunctional blood cells
123. POLYCYTHEMIA VERA
Medical Management
1. To reduce the high blood cell mass- PHLEBOTOMY
2. Allopurinol
3. Dipyridamole
4. Chemotherapy to suppress bone marrow
124. Nursing Management
1. Primary role of the nurse is EDUCATOR
2. Regularly asses for the development of complications
3. Assist in weekly phlebotomy
4. Advise to avoid alcohol and aspirin
5. Advise tepid sponge bath or cool water to manage pruritus
126. Malignant disorders of blood forming cells
characterized by UNCONTROLLED proliferation of
WBC in the bone marrow- replacing marrow
elements .
The WBC can also proliferate in the liver, spleen
and lymph nodes.
127. named after the specific lines of blood
cells afffected primarily
› Myeloid
› Lymphoid
› Monocytic
128. The leukemias are named also according
to the maturation of cells
ACUTE
› The cells are primarily immature
CHRONIC
› The cells are primarily mature or diferentiated
130. ETIOLOGIC FACTORS
› UNKNOWM
› Probably exposure to radiation
› Chemical agents
› Infectious agents
› Genetic
131. PATHOPHYSIOLOGY of ACUTE Leukemia
Uncontrolled proliferation of immature cells
↓
suppresses bone marrow function
↓
severe anemia, thrombocytopenia and
granulocytopenia
132. PATHOPHYSIOLOGY of CHRONIC Leukemia
Uncontrolled proliferation of DIFFERENTIATED cells
↓
slow suppression of bone marrow function
↓
milder symptoms
134. ASSESSMENT FINDINGS
CHRONIC LEUKEMIA
› Less severe symptoms
› organomegaly
135. LABORATORY FINDINGS
Peripheral WBC count varies widely
Bone marrow aspiration biopsy reveals a large
percentage of immature cells- BLASTS
Erythrocytes and platelets are decreased
137. Nursing Management
1. Manage AND prevent infection
› Monitor temperature
› Assess for signs of infection
› Be alert if the neutrophil count drops
below 1,000 cells/mm3
138. Nursing Management
2. Maintain skin integrity
3. Provide pain relief
4. Provide information as to therapy- chemo
and bone marrow transplantation
140. Read on the following remaining topics:
1. Thrombocytopenia
2. Disseminated Intravascular Coagulation
3. Hemophilia
4. Von Willebrand’s disease
5. Neutropenia
141. On September 12, 2011, we will have
our
UNIT EXAM
on the following topics:
Fluids and Electrolytes
And
Hematologic Disorders
142. NEXT TOPIC TO BE DISCUSSED WOULD BE:
CARDIOVASCULAR DISORDERS
AND
PERIPHERAL DISORDERS BY S’ CLAY
ENDOCRINE DISORDERS BY M’ LUDY