Chapter 30 - Hematologic Problems
Anemia
➢ Definition and Classification
• Anemia is a deficiency in the number of erythrocytes, the quantity or quality of hemoglobin and/or
volume of packed RBCs (hematocrit).
•
Ultimately lead to tissue hypoxia due to blood loss, impaired production and destruction of erythrocytes which accounts for the signs and symptoms of anemia.
•
It is a manifestation of a pathologic process not a specific disease.
•
Anemia is diagnosed by CBC, reticulocyte, and peripheral blood smear.
•
Anemia can result from primary hematologic problems or can develop as a secondary consequence of
disease or disorders of other body systems.
➢ The various types of anemia can be classified by either morphology (cellular characteristics or etiology (cause))
• Morphologic - RBC size and color.
• Etiologic - clinical condition causing the anemia.
• Morphologic - discuss patient care by focusing on the etiology.
MORPHOLOGIC CLASSIFICATION OF ANEMIA
Morphology
Etiology
Normocytic,
normochromic
Acute blood loss, hemolysis, chronic kidney disease, cancers, sideroblastic anemia,
endocrine disorders, starvation, aplastic anemia, sickle cell anemia, pregnancy
Microcytic,
hypochromic
Iron deficiency anemia, vitamin B6 deficiency, copper deficiency, thalassemia, lead
poisoning
Macrocytic
(megaloblastic)
normochromic
Cobalamin (vitamin B12) deficiency, folic acid deficiency, liver disease (including
effects of alcohol abuse)
ETIOLOGIC CLASSIFICATION
Decreased RBC production
Blood Loss
Decreased Hgb Synthesis
Iron deficiency
Thalassemia (decresed globin synthesis
Sideroblastic anemia (decreased porphyrin
Acute
Trauma
Blood vessel rupture
Splenic sequestration crisis
Defective DNA Synthesis
Cobalamin (vitamin B12) deficiency
Folic acid deficiency
Chronic
Gastritis
Menstrual Flow
Hemorrhoids
Decreased Number of RBC Precursors
Aplastic anemia and inherited disorders (e.g.
Fanconi syndrome
Increased RBC Destruction (Hemolytic Anemias)
Hereditary (Intrinsic)
Abnormal Hgb (sickle cell disease)
Enzyme deficiency (G6PD)
Anemia of myeloproliferative disease (e.g.
leukemia) and myelodysplasia
Chronic diseases or disorders
Medications and chemicals (e.g. chemo and
lead)
Radiation
Membrane abnormalities (paroxysmal nocturnal
hemoglobinuria, hereditary spherocytosis)
Acquired (Extrinsic)
Physical destruction
Prosthetic heart valves
Extracorporeal circulation
Disseminated intravascular coagulation (DIC)
Thrombotic Thrombocytopenic Purpura (TTP)
Antibodies against RBCs
Infectious agents (e.g. malaria) and toxins
➢ Causes Of Anemia
•
•
•
Decreased RBC Production
o Deficient nutrients
- Iron
- Cobalamin
- Folic acid
Blood Loss
o Chronic
- Bleeding duodenal ulcer
- Colorectal cancer
- Liver disease
o Acute
- Acute trauma
- Ruptured aortic aneurysm
- GI bleeding
Increased RBC Destruction
o Hemolysis
- Sickle cell disease
- Medication (e.g. methyldopa [aldomet]
- Incompatible blood
- Trauma (e.g. cardiopulmlonary bypass)
➢ Cultural and Ethnic Health Disparities
• Sickle cell disease
o high incidence among African Americans
• Thalassemia
o high incidence among African Americans and people of middle eastern origin
• Tay-Sachs
o highest incidence in families of Eastern European Jewish origin, especially the Ashkenazi Jews.
• Pernicious anemia
o high incidence among Scandinavians and African Americans.
➢ Clinical Manifestations
• Clinical manifestations of anemia are caused by the body response to tissue hypoxia.
• Hgb levels are often used to determine the severity of anemia.
•
Mild states of anemia (Hgb 10-12 g/dL [100-200 g/L]
o May exist w/o symptoms unless pt has an underlying disease experiencing a compensatory
response to exercise.
o Symptoms include palpitations, dyspnea, and mild fatigue
•
Moderate anemia (Hgb 6-10 g/dL [60-100 g/L]
o The above cardiopulmonary symptoms are increased
o
•
Pt may experience them while resting as well as with activity
Severe anemia (Hgb less than 6 g/dL [60 g/L]
o Pt may have clinical manifestations involving many body systems
➢ Integumentary Manifestations
• Pallor (reduced Hgb and reduced blood flow to the skin)
• Jaundice (hemolysis of RBC resulting in increased serum bilirubin)
• Pruritus (increased serum and skin bile salt concentrations)
*The sclera of the eyes and mucous membranes should be evaluated as well because it reflects integumentary changes
more accurately especially those of darker complexion. *
➢ Cardiopulmonary Manifestations
•
•
Result from additional attempts of the heart and lungs to provide adequate amounts of O2 to the tissues.
o CO is maintained by increasing HR and SV.
o Low viscosity of the blood contributes to heart murmurs and bruits.
MI, angina pectoris, HF, cardiomegaly, pulmonary and systemic congestion, ascites, and peripheral
edema may develop if heart is overworked for an extended period of time.
➢ Pulmonary
• Tachypnea, orthopnea, dyspnea at rest
➢ Neurological
• Headaches, vertigo, irritability, depression, impaired though process
➢ GI
•
Anorexia, hepatomegaly, splenomegaly, difficulty swallowing, sore mouth (glossitis and smooth pruritus)
➢ Musculoskeletal
• Bone pain
➢ General
• Fatigue, sensitivity to cold, weight loss, lethargy
NURSING AND INTERPROFESSIONAL MANAGEMENT: ANEMIA
➢ Diagnoses
• Fatigue r/t inadequate oxygenation of the blood
• Imbalanced nutrition: less than body requirements r/t inadequate nutritional intake and anorexia
• Ineffective health management r/t lack of knowledge about appropriate nutrition and medication regimen
➢ Planning
• The overall goals are that the pt with anemia will:
o Assume normal activities of daily living
o Maintain adequate nutrition
o Develop no complications related to anemia.
➢ Nursing Implementation
• Acute intervention may include
o blood transfusion
o drug therapy (e.g., erythropoietin, vitamin supplements)
o RBC replacement
o O2 therapy
• Adequate nutritional intake
•
•
Safety cautions to prevent cardiopulmonary stress, falls, and injury
Food source to promote RBC maturation:
o Avocadoes, red meat (rich in vit b12)
➢ Gerontologic Considerations
• Anemia is not a normal finding
• Anemia is rt an underlying cause such as iron deficiency, bleeding, chronic disease/inflammation. Renal
insufficiency, or a hematologic cancer
• S/s:
o pallor, confusion, ataxia, fatigue, and worsening cardiovascular and resp problems
ANEMIA CAUSED BY DECREASED ERYTHROCYTE PRODUCTION
➢ Erythrocyte
• The normal life span is 120 days.
• Three alterations in erythropoiesis may occur that decrease RBC production:
o Decreased Hgb synthesis → iron deficiency anemia, thalassemia, and sideroblastic anemia.
o Defective deoxyribonucleic acid synthesis in RBCs → megaloblastic anemias
o Diminished availability of erythrocyte precursors → aplastic anemia and anemia of chronic
disease
IRON-DEFICIENCY ANEMIA
➢ Iron-deficiency anemia
• The most common nutritional disorder in the world
• The most susceptible to anemia are the very young, those on poor diets, and women in their reproductive
years.
• Normally 1mg of iron is lost daily in urine, bile, sweat, sloughing of epithelial cells from the skin and
intestinal mucosa, and minor bleeding.
➢ Etiology
• May develop because of:
o inadequate dietary intake
o malabsorption
- Occurs in GI surgery and malabsorption syndrome such as removal or bypass if the
duodenum or disease of the duodenum
- blood loss
- melena: black stool from upper GI bleed
- peptic ulcers
- gastritis
- esophagitis
- diverticula
- hemorrhoids
- neoplasia
- GU blood loss (menstruation)
- Postmenopausal bleeding
- Chronic kidney disease
- dialysis
o Hemolysis
➢ Clinical manifestations
• In early stages, there is no symptoms
• Pallor (common)
• Glossitis (inflammation of tongue)
• Cheilitis (inflammation of lips)
•
Headache, paresthesia, burning sensation of tongue
➢ Diagnostic Studies
• History and physical exam
• HCT and Hgb lvls
• RBC count, reticulocyte count
• Serum iron, ferritin, and transferrin
• Stool occult test
o Done to determine the cause of iron deficiency
• Endoscopy and colonoscopy
o used to detect GI bleeding
• Bone marrow biopsy
o may be done if other test are inconclusive
➢ Food great in iron
• Liver and muscle meats
• Dried fruit
• Legumes
• Dark green leafy vegetables
• Whole-grain and enriched bread and cereals
• Beans
➢ Interprofessional Care
•
The main goal is to treat the underlying disease that is causing the reduced intake (e.g. malnutrition,
alcoholism) or absorption of iron.
•
Drug therapy
o
Oral iron should be used whenever possible. When administering iron, consider the following
five factors:
- Iron is absorbed best from the duodenum and proximal jejunum
▪ Enteric-coated or sustained release caps, which release iron farther down the GI
tract are counterproductive and expensive
- The daily dose: 150-200 mg of elemental iron
▪ Can be ingested in three or four daily dosage which each tablet or cap of iron
preparation containing between 50-100mg of iron.
- Iron is best absorbed in an acidic environment.
▪ Should be taken about an hour before meals, when the duodenal mucosa is most
acidic.
▪ Taking iron with vit C (ascorbic acid) or orange juice, which contains
ascorbic acid, enhance iron absorption.
- Undiluted liquid iron may stain the patient’s teeth.
▪ Iron should be diluted and ingested through a straw
- GI side effects of iron administration:
▪ Black tarry stool
▪ Constipation – use stool softener or laxative if needed
o
Parental use of iron is indicated for:
- Malabsorption, intolerance of oral iron, a need for iron beyond normal limits or poor
patient adherence in taking the oral preparations of iron Parental:
▪ IM (z-track bc iron can stain tissues) or IV
➢ Nursing Management: Iron-deficiency Anemia
•
Certain groups of individuals are at an increased risk for the development of iron deficiency anemia.
These include:
o Premenopausal
o Pregnant women
o Person from a low social economic background
o Older adults
o Individuals experiencing blood loss
•
Diet teaching with an emphasis on foods with iron and ways to maximize absorption is important for
these groups.
•
To replenish the body's iron stores, the patent needs to take iron therapy for 2-3 months after the Hgb
level returns to normal.
•
Patients who require lifelong iron supplementation should be monitored for potential liver problems
related to the iron storage.
MEGALOBLASTIC ANEMIAS
➢ Megaloblastic anemia
• A group of disorders caused by impaired DNA synthesis and characterized by the presence of large RBCs
o When DNA synthesis is impaired, defective RBC maturation results.
•
The RBCs are large (macrocytic) and abnormal are referred to as megaloblasts. They are easily destroyed
due to their fragile membranes.
•
Although most result from cobalamin and folic acid deficiency, this type of RBC deformity can also
occur from DNA synthesis by drugs, inborn error f cobalamin and folic acid metabolism and
erythroleukemia (malignant blood disorder characterized by. A proliferation of erythropoietic cells in
bone marrow).
Cobalamin (vitamin B12) Deficiency
•
•
Normally a protein termed intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa if
they are not cobalamin (extrinsic factor) cannot be absorbed.
Vit B12 is needed for the myelin sheath
➢ Etiology
•
Pernicious Anemia
o Most common cause; absence of intrinsic factor (IF)
o Gastric mucosa is not secreting IF bc of either gastric mucosal atrophy or autoimmune destruction
of parietal cells
- Parietal cells secretes hydrochloric (HCI) acid, and there for is also decreased
o Affects ages 40-60 (60 most common age of diagnosis)
o Common in Northern European (esp. Scandinavians) and African Americans
➢ Other Causes of Cobalamin Deficiency
• GI surgery (gastrectomy or gastric bypass)
• Small bowel resection involving the ileum
• Crohn's disease
• Ileitis
• Celiac disease
• Dverticula of the small intestine
• Chronic atrophic gastritis
*In these cases it is because of the loss of IF-secreting gastric mucosal cells*
•
•
•
•
Excessive alcohol or hot tea ingestion
Smoking
Long-term users of H2-histamine receptor blockers and proton pump inhibitors
Strict vegetarians
➢ Clinical manifestations
•
•
•
•
•
•
•
Sore, red, beefy shiny tongue
Anorexia,
nausea
Vomiting
Abdominal pain.
Neurological manifestations:
o Weakness
o Paresthesia of feet and hands
o Reduced vibratory and position senses
o Ataxia
o Muscle weakness
o Impaired though processes (confusion to dementia)
It may take several months for these to develop (insidious disease)
➢ Diagnostic Studies
•
RBCs appear large (macrocytic) and have abnormal shapes.
•
Serum cobalamin levels are reduced.
•
If serum folate levels are normal but cobalamin is low then that suggest anemia is due to cobalamin
deficiency
•
Serum test anti-IF antibodies done for pernicious anemia
•
Upper GI endoscopy and biopsy because pernicious anemia increases risk for stomach cancer
•
Serum methylmalonic acid (MMA) is elevated in cobalamin deficiency
•
Serum homocysteine elevated in both cobalamin and folic acid deficiencies
•
Testing for serum methylmalonic acid (MMA) (elevated mainly in cobalamin deficiency) and serum
homocysteine (elevated in both cobalamin and folic acid deficiencies) helps determine the cause of this
type of anemia.
➢ Interprofessional care
•
Instruct pt on adequate dietary intake to maintain goal of good nutrition.
•
Parental vitamin B12 or intranasal cyanocobalamin is the treatment of choice. Without it these individuals
will die in 1-3 years.
•
Oral and sublingual versions are available for those whose GI absorption is intact. As along as
supplements are used anemia can be reversed.
➢ Foods that contain Vit B12
• Red meats, especially liver, eggs, enriched grain products, milk, and dairy foods, fish
Folic Acid Deficiency
➢ Folic Acid is also required for DNA synthesis and RBC formation and maturation.
•
•
Common causes:
o Dietary deficiency (green leafy vegetables, citrus fruits)
Malabsorption syndromes:
o Celiac disease
o Crohn's disease
o Small bowel resections
o Drugs interfering with absorption or use of folic acid
- Methotrexate
- Antiseizure drugs
o Increased requirement (pregnancy)
o Chronic alcoholism
o Chronic hemodialysis
➢ Clinical manifestations
• Similar to those of cobalamin deficiency.
• GI disturbances included dyspepsia and a smooth, beefy red tongue.
• The absence of neurologic problem is a differentiating factor from cobalamin deficiency.
➢ Diagnostic findings
•
Serum folate is low (normal is 3-16 ng/mL [7-36 nmol/L]) and the cobalamin level is normal.
➢ Interprofessional care
•
•
•
Folic acid is treated by replacement therapy. Usual dosage id 1mg/day by mouth.
In malabsorption of alcoholism states up to 5 mg/day may be required.
Encourage pt to eat food containing large amounts of folic acid.
➢ Foods containing Folic Acid:
• Green leafy veggies, liver, meat, fish, legumes, whole grains, orange juice, permute, avocado
➢ Nursing Management: Megaloblastic Anemia
• Evaluate those with a positive family history.
• Bring signs and symptoms to attention of HCP.
• Carefully asses neurologic problems
• Pt should have frequent ad careful appropriate screening for gastric cancer.
• Protein patient of injuries, falling, burning and trauma.
• If heat therapy is used evaluate skin frequently for redness.
SICKLE CELL DISEASE
➢ Sickle cell disease (SCD)
• A group of inherited, autosomal recessive disorders characterized by an abnormal form of hgb in the
RBC.
• Usually identified during routine neonatal screening. It is an incurable disease that is often fatal by middle
age because of renal failure, infection, pulmonary failure and/or stroke.
➢ Etiology and Pathophysiology
•
•
•
•
•
•
•
In SCD the abnormal hgb S results from the substitution of valine for glutamic acid of the B-globin chain
of hgb.
Hgb S causes the erythrocyte to stiffen and elongate, takin on a sickle shape in response to low O2 levels.
Types of SCD:
o Sickle cell anemia
- Most severe
- Occurs when a person is homozygous for hemoglobin S (Hgb SS)
- Person has inherited Hgb S from both parent
o Sickle cell-thalassemia and Sickle cell Hgb C disease
- Occurs when a pt inherited Hgb S from one parent and another type of abnormal
hemoglobin (Such as thalassemia or hemoglobin C) from the other parent
o Sickle cell train
- Occur when a person is heterozygous for hemoglobin S (Hgb AS)
- Pt has inherited hemoglobin S from one parent and normal Hemoglobin (Hgb A) from the
other parent
Sickling Episodes
o Commonly triggered by low O2 tension in blood
o Hypoxia or deoxygenation of the RBCs can be caused by:
- Viral or bacterial infection (common)
- High altitude
- Emotional or physical stress
- Surgery
- Blood loss
- Dehydration
- Increased hydrogen ion concentration (acidosis)
- Increased plasma osmolality
- Decreased plasma volume
- Low body temperature
- No obvious cause
Sickled cells cannot easily pass through capillaries or other small vessels and can cause vascular
occlusion leading to acute or chronic tissue injury.
Circulating sickled cells are hemolyzed by the spleen, leading to anemia.
Sickle cell crisis:
o A severe, painful, acute exacerbation of RBC sickling, causing a vaso-occlusive crisis
- As blood flow is impaired by sickled cells, vasospasm occurs, further restricting blood
glow
- Severe capillary hypoxia causes changes in membrane permeability, leading to plasma
loss, hemoconcentration, thrombi, and further circulatory stagnation
- Tissue ischemia, infarction, and necrosis eventually occur from lack of O2
- Shock is a possibly life-threatening consequences bc of severe O2 depletion and
reduction of circulating fluid volume
- Crisis can begin suddenly and persist for days to weeks.
➢ Clinical manifestations
•
•
•
•
•
Primary symptom: pain
o Pain episodes are accompanied by fever, swelling, tenderness, tachypnea, HTN, n/v
- Affect any area of the body or several site simultaneously, with back, chest, extremities
and abdomen
Pt may have chronic health problems and pain bc of organ tissue hypoxia and damage (e.g., involving the
kidneys or liver)
Pallor (check mucous membrane)
Skin may have grayish cast
Jaundice because of hemolysis and pt become more prone to gallstone (Cholelithiasis)
➢ Complications
• W/ repeated episodes of sickling, there is gradual involvement of all body systems, especially spleen,
lungs, kidney, and brain
• Infection is a major cause or morbidity and mortality
o Autosplecnectomy: spleen becomes small bc of repeated scarring
o Spleen fails to phagocytize foreign substance as it become infarcted and dysfunctional
• Acute chest syndrome:
o Used to describe acute pulmonary complication that include pneumonia, tissue infarction, and fat
embolism
- Characterized by:
▪ Fever
▪ Chest pain
▪ Cough
▪ Pulmonary infiltrates
▪ Dyspnea
• Infarction may cause pulmonary HTN, MI, HF, and cor pulmonale
• Heart may become ischemia and enlarge leading to HF
• Priapism: persistent penial erection
➢ Diagnostic studies
•
•
•
•
•
•
•
Peripheral blood smear may reveal sickled cells and abnormal reticulocytes
o The presence of Hgb S can be diagnosed by the sickling test, which uses RBCs (in vitro) and
exposes them to a deoxygenation agent.
Clinical findings include hemolysis (jaundice, elevated serum bilirubin levels) and abnoral lab test results.
Hgb electrophoresis may be done to determine the about of hgb S,
Skeletal X-rays demonstrate bone and joint deformities and flattening
MRIs may be used to dx a stroke caused by blocked cerebral vessels from sickled cells.
Doppler students used to assess for DVT,
Chest X-ray to dx infection or organ malfunction etc.
➢ Nursing and Interprofessional Management Sickle cell disease
•
•
•
•
•
•
•
•
•
•
Interprofessional care for a patient with SCD is directed toward:
o Prevention of sequealae from the disease
o Alleviating manifestation for the complication of the disease
o Minimizing end-organ damage
o Promptly treating serious sequelae, such as acute chest syndrome, that can lead to immediate
death,
Teach patients with SCD to avoid high altitudes, maintain adequate fluid intake and treat infections
promptly.
Screening for retinopathy should begin at age 10
Immunizations should be administered (such as pneumococcal, haemophilus influenzas, influenzas, and
hepatitis)
O2 may be administered to treat hypoxia and control sickling
Assess for any changes in respiratory status, rest may reduce metabolic requirements and DVT
prophylaxis.
Fluids and electrolytes are administered to reduce blood viscosity and maintain renal function.
Priapism is managed with pain meds, fluids and nifedipine (procardia).
o If not resolved call urologist who can inject corpus carvernosum eith a filute solution of
epinephrine to preserve penile function.
During an acute pain crisis, optimal pain control usually includes large dosages of continuous opioid
analgesics along with breakthrough analgesia often in the form of pain controlled analgesia (PCA).
A holistic approach should be used that addresses the pain and its impact on the patients psychologic,
social and spiritual well-being
•
•
•
Occupational and physical therapy can optimize functioning,
o Referral of social and chaplain services can assist. Infection is a frequent complication and must
be treated,
Although may sickling agents have be tried, hydroxyurea (hydrea) is the only one that has been shown to
be beneficial. This drug produces the about of fetal hgb (hgb F) and alters the adhesion of sickled RBCs
to the endothelium.
Acute chest syndrome are treated w/ broad spectrum antibiotics, O2 therapy, fluid therapy, and possible
exchange transfusion
POLYCYTHEMIA
➢ The production and presence of increased numbers of RBCs
• Increase causes increased blood viscosity (hyperviscosity) and volume (Hypervolemia)
➢ Etiology
• Primary polycythemia Vera
o A chronic myeloproliferative disorder, includes increase in RBCs, WBCs, and platelets
o 60 years old w/ slight male predominance
o Enhanced blood viscosity and blood volume w/ congestion of organs and tissues w/ blood
o Splenomegaly and hepatomegaly are common
o Hypercoagulopathies that predispose clothing problems
o Associated w/ mutations in the Janus Kinarse-2 (JAK2) gene
- Gene provides instructions for making a protein that promotes proliferation of cells,
especially blood cells from hematopoietic stem cells
- Begins w/ one or more DNA mutations of a single hematopoietic stem cells
• Secondary polycythemia
o Hypoxia driven secondary polycythemia, hypoxia stimulates erythropoietin (EPO) production in
the kidney, which in turn stimulate RBC
- The need for increase O2 results from high attitudes, pulmonary disease, cardiovascular
disease, alveolar hypoventilation, defective O2 transport, or tissue hypoxia
o Hypoxia independent secondary polycythemia, EPO is produced by a malignant or benign tumor
tissue
- Serum EPO remains elevated
o Splenomegaly does not accompany secondary polycythemia
➢ Clinical manifestation and Complications
• Occur because if HTN caused by hyperviscosity and hypervolemia
• First manifestations:
o Headache, vertigo dizziness, tinnitus, visual disturbances
• Generalized pruritus’ (often exacerbated by a hot bath) rt histamine release from an increased number of
basophils
• Paresthesia and erythromelalgia (painful burning and redness of ands and feet)
• Angina, FH, intermittent claudication, thrombophlebitis, which may be complicated by embolization
o Caused by blood vessel distention, impaired blood flow, circulatory stasis thrombosis, tissue
hypoxia caused by hypervolemia and hyperviscosity
• Stroke secondary thrombosis
• Hemorrhagic caused by either vessel ruptures from overdistention or inadequate platelet function may
result in petchia, ecchymoses, epistaxis, or GI bleeding
• Hepatomegaly and splenomegaly from organ engorgement may contribute to pt complaints of satiety and
fullness
• Plethora: ruddy complexion
• Hyperuricemia caused by the increase RBC destruction that accompanies excessive RBC production
o Uric acid is a product of cell destruction
➢ Diagnostic Studies
•
o (1) Elevated hemoglobin and RBC count w/ microcytosis
o (2) Low to normal EPO level (secondary polycythemia has high EPO Level)
o (3) Elevated WBC count w/ basophilia
o (4) Elevated platelet count (thrombocytosis) and platelet dysfunction
o (5) Elevated leukocyte alkaline phosphatase, uric acid, and cobalamin levels
o (6) Elevated histamine levels
Bone marrow examination shows hypercellularity or RBCs, WBCs, and platelets
➢ Interprofessional Care
• Treatment is directed toward reducing blood volume and viscosity and bone marrow activity
• Phlebotomy is to reduce hematocrit and keep it less than 45% in men and 42% in women
• Hydration therapy is used to reduce the blood’s viscosity
• Ruxolitnib (Jakafi), which inhibits the expression of JAK2 mutation, is used for pts who have not
responded o hydroxyurea
• Low-dose heparin used to prevent clotting
• Anagrelide may be used to reduce the platelet cunt and inhibit platelet aggregation.
➢ Nursing Management
• Primary is not preventable
• Evaluate hydration for overhydration and underdehydration
• Myelosuppressive agents used
• Assess pt nutritional status, since inadequate food intake can result in HI symptoms of fullness, pin,
dyspepsia
PROBLEMS OF HEMOSTASIS
➢ Hemostasis involves:
• Endothelium, platelets, and coagulation factors, which normally function together to stop hemorrhage and
repair vascular injury
o Disruption of any of these components could result in bleeding or thrombotic disorders
• Three major disorders:
o Thrombocytopenia (low platelet count)
o Hemophilia and von Willebrand disease (inherited disorders of specific clotting factors)
o Disseminated Intravascular Coagulation (DIC) (process of clotting and unclotting)
THROMBOCYTOPENIA
➢ Etiology and Pathophysiology
• A reduction of platelets below 150,000
o Acute, severe, or prolonged decreases from this normal range can result in abnormal hemostasis
that manifests are prolonged bleeding from minor trauma or spontaneous bleeding without injury
• Inherited (e.g., Wiskott-Aldrich syndrome)
• Acquired
o Drugs
- Chemotherapy drugs
- Aspirin
o Quinine
o Infections
- Viral infection (hep C virus, HIV, cytomegalovirus)
- Bacterial infections
o Hematologic malignancy Hematologic malignancy (Leukemia, lymphoma, myeloma)
o Disseminated Intravascular Coagulation (DIC)
o Immune thrombocytopenic purpura (ITP)
- Most common cause of acquired
- Syndrome of abnormal destruction of circulating platelets
-
Primarily an autoimmune disease; platelets are coated w/ antibodies
▪ Function normally, when they reach the spleen the antibody coated platelets are
recognized as foreign and are destroyed by macrophages
▪ Decreased platelet production contribute to this
o Thrombotic thrombocytopenic purpura (TTP)
- Uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurologic
abnormalities, fever (in absence of infection), and renal abnormalities not all feature are
present in all pts
- Almost always associated w/ hemolytic-uremia syndrome (HUS); often referred to as
TTP-HUS
- Enhanced aggregation of platelets, which formation thrombi that deposit in arterioles and
capillaries
- Deficiency of a plasma enzyme (ADAMS13) that usually break down the von Willebrand
clotting factory (vWF) into normal size
▪ W/o enzyme, usually large amounts of vWF attach to activated platelets, thereby
promoting platelet aggregation
- Predominate in female
o Heparin-induced thrombocytopenia (HIT)
- Also called heparin-induced thrombocytopenia and thrombosis syndrome (HITTS)
- Pts develop 5-10 days after the onset of heparin therapy
- Should never be given heparin
- Major clinical problem:
▪ Venous and arterial thrombosis
▪ Arterial vascular infarcts resulting in skin necrosis, stoke, and end-organ damage
- Platelet factor 4 (PF4) (protein made and release by platelets) binds to heparin
▪ Then binds to platelet surface, leading to further platelet activation and release of
more PF5 thus creating a positive feedback
- Management therapies:
▪ Heparin may be discontinued
▪ To maintain anticoagulation pt should be started on other anticoagulant
▪ If severe clotting, protamine sulfate, thrombolytic agent, and surgery removal of
clots
➢ Clinical Manifestation
• Many are asymptomatic
• Bleeding is the most common symptoms
o Petechia – small red/purple marks on the skin
o Ecchymoses – discoloration of the skin resulting from bleeding underneath
• Hemorrhage
• Confusion, headache, seizures, and coma due to TTP-related thrombosis may be seen
➢ Diagnostic Studies
• Thrombocytopenia: platelet <150,000
• Prolong bleeding doesn’t occur till platelets <50,000
• <20,000 spontaneous life-threatening bleeding
• ITP antigen specific assay, platelet action/function assay, PF4 heparin complex for HIT
➢ Nursing Management
• Acute care
o Goal is to prevent or control hemorrhage
o If subcut injection are unavoidable the use of small-gauge needle and application of direct
pressure for 10-15 minutes
o In a woman, menstrual bleeding may exceed the usual amount and duration
- Counting sanitation napkins is helpful
- Blood loss of 50ml will completely soak napkin
- Suppression of menses w/ hormonal agents may be indication w/ predictable periods
• Pt / caregiver teaching
o
o
o
o
o
o
o
o
o
o
o
o
Notify HCP for manifestation of bleed (Stool, vomit, bruising, headaches)
Ask HCP for restriction on activities
Do not blow nose forcefully
Do not bed down w/ head lower than your waist
Prevent constipation by drinking plenty of fluids
Shave only w/ electric razor
Do not tweeze eyebrows
Do not puncture skin (tattoos, piercing)
Avoid meds that prolong bleeding
Use soft bristly toothbrushes
Women menstruation should keep tract of flow
Ask HCP before any invasive procedures
NEUTROPENIA
•
•
•
Leukopenia refers to decrease in total WBC count
o Granulocytpenia- include neutrophils, eosinophils, basophils
o Neutrophilic granulocytes
- play a major role in phagocyting pathogenic microbes, closely monitor indicating
infection
Neutropenia - reduction in neutrophils
o Absolute neutrophil count (ANC)
- Determined by multiplying the total WBC count by the percentage of neutrophils
o Neutropenia defined as ANC less than 1000
o Severe neutropenia <500
Important to know where the decrease in neutrophil count was gradual or rapid, the degree of neutropenia,
and duration
o Faster drop, the longer the duration, the most susceptible to infection, sepsis, and death
➢ Clinical Manifestation
•
•
•
•
A low-grade fever is of great significance because it may indicate infection and lead to septic shock and
death
Neutropenic fever (>100.4 and neutrophil count <500) is an emergency
Blood culture should be drawn STAT and antibiotics started within 1 hour
Complaints of sore throat and dysphagia, ulcerative lesion of the pharyngeal and buccal mucosa, diarrhea,
rectal tenderness, vaginal itching or discharge, SOB, and nonproductive cough
➢ Diagnostic Studies
•
•
•
•
•
Peripheral WBC count
Bone marrow aspiration
Biopsy
Differential WBC count
Peripheral blood smear used to assess immature form of WBCs
➢ Nursing Management
•
•
•
•
•
•
(1) Determine the cause of neutropenia
(2) instituting antibiotic therapy promptly
(3) Identifying the offending organism if an infection has developed
(4) Administering hematopoietic growth factors prophylactically after chemotherapy
(5) implementing protect practice (hand washing)
Teaching
o Wash hands frequently
•
•
•
•
•
•
•
•
Notify HCP:
o Fever >100.4
o Chills or felling hot
o Redness, swelling, discharge, or new pain on or in your body
o Changes in urination or bowel movements
o Cough, sore throat, mouth sores or blister
If at home take temp as directed
Avoid crowds and people w/ flu, or infection
o If in public place wear mask, use hand sanitizer frequently
Avoid uncooked meats, seafood or eggs, and unwashed fruits and veggies
Bathe or shower daily and use moisturizer to avoid cracking skin
Maintain some daily activity
Brush teeth w/ soft brush 4 times a day
Do not perform garden or clean up after pets
LEUKEMIA
•
•
A group of malignant disorder affecting the blood and blood-forming tissues of the bone marrow, lymph
system, and spleen
Accumulation of dysfunctional cells because of loss of regulation in cell division
➢ Etiology and Pathophysiology
•
•
All cancer begins w/ mutation of DNA of certain cells
Chemical agents, chemo, viruses, radiation, and immunologic deficiencies have been associated w/
development
➢ Classifications
• Acute
o Characterized by the clonal proliferation of immature hematopoietic cells
•
o
Acute Myelogenous Leukemia (AML)
- Adults
- Uncontrolled proliferation of myeloblastic (macrophages..), the precursor of granulocytes
- Hyperplasia of bone marrow
- Strongly linked to toxins, congenital, and hematologic disorders
- infiltration of malignant cells in skin, gums, and other soft tissue
- S/s:
▪ fatigue, weakness, headache, mouth sores, anemia, bleeding, fever, infection,
sternal tenderness, gingival hyperplasia, milk hepatosplenomegaly
o
Acute Lymphocytic Leukemia (ALL)
- Children mostly
- Immature small lymphocytes (T and B cells, NK cells) proliferation in bone marrow;
most are B cell origin
- Generalized lymphadenopathy, infections, weight loss, hepatosplenomegaly, headache,
mouth sores
- Increased intracranial pressure (n/v, lethargy, cranial nerve dysfunction) secondary to
meningeal infiltration
- S/S:
▪ Fever, pallor, bleeding, anorexia, fatigue, weakness, bone, joint, and ab pain
Chronic
o Involve more mature form of WBC, and the disease onset is more gradual
o
Chronic Myelogenous Leukemia (CML)
- Excessive development of mature neoplastic granulocytes in bone marrow
- Infiltrate liver and spleen
- Blastic phase: more acute, aggressive phase
- Philadelphia chromosome originates from the translocation b/w the BCR gene on
chromosome 22 and ABL gene on chromosome 9 creating BCR-ABL
- No symptoms in early stages
- S/S:
▪ Fatigues and weakness, fever, sternal tenderness, weight loss, joint pain, bone
pain, massive splenomegaly, increase in sweating
o
Chronic Lymphocytic anemia (CLL)
- Most common in adults
- Production and accumulation of functionally inactive but long-lived small matureappearing lymphocytes
- Infiltrate bone marrow spleen and liver
- Frequently no symptoms
➢ Diagnostic Studies
•
Peripheral blood evaluation and bone marrow exam diagnose and classify types
➢ Interprofessional Care
•
Attaining remission or disease control is a realistic option for majority of pts
o Complete remission:
- no evidence of overt disease on physical examination, and bone marrow and peripheral
blood appear normal
o Partial remission:
- lesser state of control; lack of symptoms and a normal peripheral blood smear but still
evidence of disease in bone marrow
- Minimal residual disease tumor cells that cannot be detected by morphologic examination
but can be identified by molecular testing
o Molecular remission:
all molecule studies are negative for residual leukemia
• Stages of chemo
o Induction therapy
- Aggressive seeks to destroy leukemic cells in the tissues, peripheral blood, and bone
marrow to eventually restore normal hematopoiesis on bone marrow recovery
o Postinduction or postremission therapy
- Intensification therapy (high-dose) therapy, may be given immediately after induction
therapy for several months
- Consolidation therapy:
▪ Started after remission is achieved
▪ Purpose is to eliminate remaining leukemia cells that may not clinically or
pathological evident
o Maintenance therapy
- Treatment w/ lower doses of some drugs used in induction given every 3 to 4 weeks for
prolonged period
- Goal is to keep body free of leukemic cells
➢ Nursing Management
• Acute care:
o Maximizing the pt physical function
o Teaching pts that acute side effects of treatment are usually temporary
o Encouraging pts to discuss their quality of life issues
LYMPOMAS
•
Malignant neoplasm originating in the bone marrow and lymphatic structure resulting in proliferation of
lymphocytes
HODGKIN’S LYMPHOMA
•
•
Reed-Sternberg cells, located in lymph nodes
Occurs b/w 15 and 30 and above 55
➢ Etiology
•
•
•
Infection of Epstein Barr viruses (EBV), genetic predisposition and exposure to occupational toxins
Increased w/ HIV infection
Normal structure of lymph nodes is destroyed by hyperplasia of monocytes and macrophages
➢ Clinical Manifestations
•
•
•
•
•
•
Initially development is most often enlargement of cervical (80%), mediastinal, axillary, or inguinal
lymph nodes
Nodes remain moveable and nontender
S/S:
o Weight loss, fatigues, weakness, fever, chills, tachycardia, or night sweats
Initial findings include fever (>100,4), drenching night sweats, weight loss termed B symptoms
Alcohol induced pain
hepatomegaly, splenomegaly, anemia
➢ Diagnostic and Staging Studies
•
•
•
•
•
•
Peripheral blood analysis
Excisional lymph node biopsy
Bone marrow examination
Radiologic evaluation can help define all sites and determine the clinical stage of disease
Staging:
o Stage 1: involvement of single lymph node
o Stage 2: involvement of 2 or more on one side of diaphragm
o Stage 3: lymph node involvement above and below diaphragm
o Stage 4: involvement outside diagram (e.g., liver, bone marrow)
o Stages followed by A (Absent) or B (presence) to indicated systemic symptoms
Dx: Undergo a liver function test
➢ Interprofessional Management
•
•
Standard chemo that is the ABVD regimen
o Doxorubicin (Adriamycin)
o Bleomycin
o Vinblastine
o Dacarbazine
Advanced stages use BEACOPP
o Brentuximab vedotin (Adcetris), bleomycin, etoposide, doxorubicin (Adriamycin),
cyclophosphamide, vincristine (Oncovin), procarbazine and prednisone
NON-HODGKIN’S LYMPHOMA (NHLs)
•
Heterogenous group of malignant neoplasm of primary B-, T-, and NK cells organ affecting all ages
➢ Etiology and Pathophysiology
•
May result from:
o Chromosol translocation
o Infections
o Environmental infections
o Immunodeficiency states
➢ Clinical Manifestation
•
•
•
•
•
Can originate outside the lymph nodes the method of spread is unpredictable
Primary: painless lymph node enlargement
Manifest in nonspecific ways such as airway obstruction, hyperuricemia, and renal failure from tumor
lysis syndrome, pericardial tamponade, and HI complaints
B symptoms
Pt undergoing chemotherapy is at risk for developing tumor lysis syndrome
➢ Diagnostic and Staging Studies
•
Treatment is guided by the cell type, cytogenetic studies, and clinical behavior: indolent (low grade),
aggressive (high grade), highly aggressive (very high grade)
MULTIPLE MYELOMA*
•
•
Or plasma cell myeloma, condition in which neoplastic plasma cells infiltrate the bone marrow and
destroy bone
More common in men and African Americans
➢ Etiology and Pathophysiology
•
•
Instead of plasma cells producing antibodies to fight different infection, myeloma tumors produce
monoclonal (M proteins) (they are all one of a kind) antibodies
o They don’t fight infection and infiltrate bone marrow
o M protein are made up of 2 light chains and 2 heavy chains
- Bence jones protein are the light chain part
o Show up in urine
Plasma cell production of excessive and abnormal number of cytokines also play a role in pathologic
process of bone destruction
➢ Clinical Manifestations
•
•
•
•
•
Pain in pelvis, spine, and ribs common and trigged by movement
Diffuse osteoporosis develops as bone is destroyed
Osteolytic lesions are seen in skill, vertebrae, and ribs
Hypercalcemia causing GI, renal, or neurological manifestations such as polyuria, anorexia, confusion,
seizures, coma, cardiac problems
Serum hypervelocity symptoms leading to central, pulmonary, renal, and organ dysfunction can occur in
some pts
➢ Interprofessional Care
•
Hydration and ambulation treat hypercalcemia, dehydration, and potential renal damage
•
•
•
•
•
•
Weight bearing helps reabsorb some calcium and fluids dilute calcium and prevent protein precipitates
from causing renal tubular obstruction
Control pain and preventions of pathology fracture
Analgesic orthopedic supports and local radiation help reduce skeletal pain
Kyphoplasty control spine vertebral disease by injecting cement to stabilize vertebrae
Bisphosphonates inhibit bone breakdown and used for treatment of skeletal pain and hypercalcemia
o Inhibit bone reabsorption without inhibiting born formation and mineralization
Chemotherapy reduced plasma cells
➢ Nursing Management
•
•
Use caution when moving pt bc of pathological fractures
Hydration