Hemotologic Disorders - Faculty Sites

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Hemotologic Disorders
By Diana Blum RN MSN
Metropolitan Community College
NURS 2140
Should be subjective
 Should be objective
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Assessment
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Blood is a type of connective tissue
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Immunological aspects also important
◦ Transports nutrition
◦ Transports oxygen
◦ Transports hormones
◦ Critical to body defense
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Maintains temperature
Controls PH
Removes toxins
Regulates electrolytes
Anatomy
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Hematopoiesis:
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Bone Marrow:
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Stem Cell:
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Plasma:
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Leukocyte:
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Reticulocyte:
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WBC:
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RBC:
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Erythrocyte:
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Platelet:
Cell Types (page 1991-94)
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Responsible for removing excess fluid
protein and large particles from cells
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Main functions:
◦ fluid balance maintenance
◦ Defense
◦ Fat absorber
Lymphatic system
Stores 20-40 ml blood
 Removes old cells
 Filters and destroys antigens before they
enter system
 Stores platelets (1/3 of body’s store)
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Spleen
Stopping of blood flow
 2 phases:
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◦ Primary
◦ Secondary
Hemostasis (page 1995)
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Be able to explain tests to pts
Be able to explain why tests being done
Know normal from abnormal
Be good communicator with physician
Be able to consider anticipated actions
◦ Ex. When blood transfusion needed
Critical thinking here
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Hemoglobin is low
Oxygen is low
Not a disease but an underlying condition
2 types: hypoproliferative and hemolytic
◦ Hypoproliferative: marrow can’t produce
adequate erythrocytes
 Lack of factors, chemicals, marrow damage
◦ Hemolytic: premature destruction of
erythrocytes. Leads to tissue hypoxia
 Abnormality like sickle cell, direct injury
Anemia (page 2008)
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Stimulation of RBCs by erythropoietin
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Erythropoietin: hormone produced by
kidneys during periods of hypoxia to
ensure growing tissues will have sufficient
oxygen.
Erythropoiesis
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Based on:
◦ Marrow ability to respond to decreased
erythrocytes
◦ Manner of maturity for young erythrocytes
◦ The presence or absence of end products of
erythrocyte destruction within the circulation
Determination between types
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Rapidity with the anemia has developed
Duration of anemia
Metabolic requirements of patient
Other disorders
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Cardio: tachycardia, hypoxia, MI symptoms
Respiratory: SOB in varying degrees
Neuro: headache, poor concentration,
vertigo, irritibility, confusion
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Compensation also may occur
Clinical manifestations (pg 2010)
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Prioritize activities
Plan rest periods
Eats healthy foods rich in iron, vitamins, and
proteins
Use nutrition supplements as needed
Report palpitations, dizziness, dyspnea
Monitor vitals
Monitor edema
Monitor neuro status
Safety techniques
Education
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Low RBC
Low HGB andHCT
Low serum Iron level
Low ferritin level
High TIBC level
Results from diet low in iron
Results from body not absorbing enough iron from GI tract
◦ Not enough hemoglobin made as result
s/s: sore tongue, brittle nails, fatigue, pallor, orthostatic
changes (in severe cases)
Dx: bone marrow aspiration, lab work
Tx: iron supplements, iron rich foods (liver, oysters, red meat,
fish, dried fruits, legumes, dark green veggies, whole grain
breads and cereal)---orange juice or other vitamin C product
will enhance iron absorption
◦ What is the major side effect of IV iron?
Iron Deficiency Anemia
Pts don’t become severely anemic til
creatinine levels exceed 3mg/100ml
 Hematocrit decreases
 Causes:
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◦ shorted RBC lifespan
◦ Deficiency of erythropoietin
◦ Long term hemodialysis
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Tx: monitor serum levels of iron,
hematocrit, and vitals
Anemia and Renal Disease
Only inflammation, infection, and
malignancy cause
 It is nonprogressive
 Develops over 6-8 weeks
 Many don’t require treatment
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Anemia and chronic disease
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Most cases are idiopathic
Infection and pregnancy, medication, chemicals or
radiation can trigger
◦ Airplane glue, arsenic, pestisides
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Complete failure of the bone marrow
Low RBC count
Low WBC
Low Platelet cts
S/S: pallor, fatigue, tachycardia, sob, hypotension,
prolonged/spontaneous bleed, frequent infections, purpura,
retinal hemorrhages are common
Tx: transfusion of RBC and Platelet, antibiotics,
corticosteroids, bone marrow transplant, ICU setting,
monitor for signs and symptoms of infection and bleeding,
neutropenic precautions
Aplastic Anemia
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Pt does not absorb vitamin B12 from stomach
◦ Higher incidence of gastric cancer
Pt may lack intrinsic factor-essential for b12
absorption
 Assess hx: gastrectomy, crohn’s, family history,etc.
 s/s: weakness, sore tongue, numbness of hand and
feet
 Tx: B12 injections monthly, endoscopy q1-2 years---if
untreated can develop heart failure and lead to death
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◦ Monitor oral cavity, and skin. Monitor for jaundice, monitor
gait, provide small frequent bland diet
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Dx: Obtain shilling test
Pernicious Anemia
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Most frequent in SE Asian, African, and
Mediterranean descent
Thalassemia
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Folate is in green leafy veggies and liver
Alcohol increases need for folic acid
S/S same as pernicious anemia except no
neuro symptoms
Dx: folate level
Tx: replacements
Folic Acid Deficiency
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RBCs normally disc shaped
In sickle cell they are sickle shaped
Easily rupture
Obstruct blood flow
Genetic in nature
Most common in African Americans
Sickle cell is recessive (inherit from mom
and dad)
Sickle cell anemia
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3 types:
◦ sickle crisis: most common---obstructive
◦ aplasic crisis---from infection from parvovirus
◦ sequestrian crisis---result from other organs
pooling sickled cells
 Spleen is most common
 Also in liver and lungs
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S/S: tachycardia, fever, decreased HGB,
infiltrates on chest x-ray
Pulomary HTN is a symptom that is not
usually detected until damage is irreversible
Monitor x-rays, CT scan, Echo, HGB level
Tx: treat symptoms, Bone marrow
transplant, transfusions, Hydroxyurea,
Arginine
Sickle Cell Crisis
This is a deficiency in the gene that is essential
for membrane stability
 Hemolysis only occurs with stressful situations
 Mostly affects african americans, greek or italian
decent as well as asians and jewish origins
 Inherited on x chromosome
 S/S: asymptomatic, or jaundice, pallor,
hemoglobinuria
 Dx: serum G-6-PD
 Tx: stop offending med, transfusion, educate
about meds to avoid, instruct to wear medic alert
bracelet
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Glucose -6-Phospate
Dehydrogenase Deficiency
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Enough RBCs made but they are destroyed
once they are released into circulation
Causes: infection, drug reaction, cancer
s/s: pallor, fatigue, tachycardia, sob,
hypotension, jaundice, high bilirubin levels
Positive direct coombs antiglobulin test
Tx: blood transfusions, corticosteroids,
spleenectomy
Recovery in few days to weeks
Autoimmune Hemolytic Anemia
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Too many RBCs produced
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s/s: headache, dizziness, ringing in the ears, blurred
vision, ruddy complexion, HTN, pruritis, gout
problems, burning fingers and toes
DX: o2 sat, CBC with DIFF
Complications: CVA, MI, BLEED issues
Tx: hydroxyurea, interferon, plavix, ASA
Treat symptoms
Teach pt: don’t cross legs, quit smoking, stay active,
minimal alcohol, avoid excess iron
phlebotomize blood
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◦ Blood more viscous
 Use 18 gauge needle
 In antecubital vein and remove I unit of blood
Polycythemia Vera
Defect in the WBC stem cell
 Abnormal reproductions
 Unknown cause, may be from chemical or
radiation exposure
 Symptom onset is abrupt
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Leukemia
Defective stem cell
Incidence rises with age 60+
Prognosis variable
Younger pts may survive 5 years
S/S: fever, infection, weakness, fatigue, bleed
tendency, pain
 Dx: no warning, CBC
 Complications: bleeding (petechiae, ecchymosis),
infection, DIC
 TX: chemo, transfusions, infection treatment,
growth factor agents, bone marrow transplant,
palliative care
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AMLAcute Myeloid Leukemia
Uncommon in pts under 20
 Life expectancy 3-5 years
 s/s: asymptomatic, dyspnea, mild
confusion, enlarged liver, wt loss, anorexia
 Tx: tyrosine kinase inhibitor, avoid
antacids and grapefruit juice, interferon,
cytosine, leukopheresis, bone marrow
transplant
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CML
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Most common in young kids 4-14
Uncommon
s/s: decreased cbc counts, pain, enlarged
liver, enlarged spleen, headache, vomiting
Tx: bone marrow transplant, chemo,
corticosteroids
Complications: infection
Acute Lymphocytic LeukemiaALL
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Common in older adults
Most common form in US and Europe
More frequent in men
s/s: asymptomatic, lymphadenopathy,
pain, hepatomegaly, spleenomegaly,
anemia, fevers, drenching sweats, wt
loss, infections
Tx: treat symptoms, chemo, monoclonal
antibodies
CLL
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Characterized by reed- sternberg cells in
the lymph nodes
Highest occurance is in 20s and50s
Men are more likely than women to have
Tx: radiation, chemo, bone marrow
transplant, stem cell transplant
Survival rates vary
5 yr survival rate is 82%
Hodgkins Disease
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6th most common type of cancer and cancer
death in US
◦ Avg age of dx is 60
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Stages
◦ Low grade
◦ Intermediate grade
◦ High grade
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The higher the grade the more aggressive
Tx: chemo, radiation, bone marrow
transplant, stem cell transplant
5 year survival rate is 52%
Non Hodgkins Lymphoma
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Cancer of the plasma cells
Most common over the age of 60
No known cause
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s/s: bone pain, hyperuricemia (kidneys),
anemia, hypercalcemia, fractures, spinal cord
compression, renal failure
Diagnosis: radiographs, serum and urine
protein electrophoresis, bone marrow biopsy
No known cure
Tx: chemo and radiation to treat symptoms,
vertebroplasty, antiemetics, pain
management
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◦ Genetics and radiation exposure play a part
Multiple Myeloma
Bleeding disorders
http://www.mhhe.com/biosci/esp/2002_general/Esp/folder_structure
/tr/m1/s7/trm1s7_3.htm
Clotting Cascade
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Too few platelets
◦ Causes: cancer treatment
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Too many platelets being destroyed
◦ Causes: idiopathic thrombocytopenic purpura,
thrombic thrombocytopenic purpura
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Dx: bone marrow bx, CBC
s/s: petechiae, purpura, gingival bleed,
epistaxis, prolonged bleeding
Tx: transfusions, spleenectomy, chemo, stop
med that is causing, stop ETOH, treat
infection
Thrombocytopenia
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Most common among kids and young girls
Usually 1-6 wks after viral infection
Cause unknown, h. pylori may play role
s/s: asymptomatic, low platelet count,
petechiae, hemoptysis
Dx: bone marrow bx, platelet ct
Tx: stop med, immune globulin, chemo,
Nsg: assess lifestyle, teach symptoms, avoid
constipation, avoid valsalva, avoid teeth
flossing, use only electric razors, use only
soft bristled tooth brush, monitor for
osteoporosis
Immune thrombocytopenic
purpura
Example of drug induced immune
mediated thrombocytopenia
 Rare
 Related to heparin therapy
 Tx: observation, assessment, monitor lab
values
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HIT
Most common inherited bleeding disorder
 1-2% international population
 s/s: Mucosal and cutaneous bleeding,
porlonged bleed times
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Tx: DDAVP, FFP, Cryoprecipitate, bleed
precautions
Von Willebrand’s Disease
Thrombic Thrombocytopenic
Purpura
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Genetic
Lack of blood clotting factor
1-2 cases per 20,000 persons
Types
◦ A: factor VIII is missing-higher incidence
◦ B: factor IX is missing
Trait is carried on x chromosome
Rare for women to have
s/s: uncontrolled bleed especially in joints, skin, GI tract
Tx: no cure. Transfusions, pain treatment (IV morphine
is common)..monitor for addiction to opiods.
Hemophilia
Not a disease
Triggers: sepsis, trauma, cancer, shock, abruptio
placentae, allergic reactions
 Life threatening condition
 Initially coag time is normal
 S/S: are manifested in organs with either clots or
bleeding. Bleeding from all orifaces(tear ducts,
gums, IV sites, rectum, urethra, etc.)
 Dx: PT, PTT, D-Dimer
 Tx: treat the cause, replace fluids, o2, fix
electrolyte imbalances, administering
vasopressors are vital, cryoprecipitate to replace
factor 5 and 7, FFP, heparin
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DIC
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Universal donor= O◦ Does not contain A, B, or Rh antigens
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Universal recipients= AB+
◦ Blood contains A, B, and RH antigens
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Usually blood banks exactly match the pt
blood
Blood typing for transfusion
Pt needs 18 or 20 gauge IV needle so cells are
not lysed (destroyed)
 Prior to administration, blood needs to be
checked by 2 licensed nurses. Check the
expiration date, name, medical record number,
type of blood, blood band id, pt birthday
◦ Check vitals prior to administration
 **blood must be initiated with in 30 minutes of
arrival from lab to floor
 Use blood tubing for administration
 Monitor for blood reactions
 Monitor vitals continuously during administration
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adminstration on blood
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Hemolytic: fever, chills, nausea, dyspnea, chest pain, back pain, hypotension
◦ Antigen/antibody rx to transfusion
◦ Happens shortly after initiation
◦ Tx: stop the transfusion, call md, supportive therapy to maintain HR and BP
Anaphylactic: urticartia, wheezing, dyspnea, hypotension
◦ Type 1 hypersensitivity rx to plasma proteins
◦ Occurs within 30 minutes of initiation
◦ Tx: stop transfusion, call md, be ready for epi and steroids
Febrile: fever, chills
◦ Recipients antibodies rx to donor leukocyte
◦ Occurs within 30-90minutes of initiation
◦ Tx: stop infusion, call md
Circulatory overload: cough, frothy sputum, cyanosis, decreased BP
◦ cardio system is unable to manage the additional fluid load
◦ Occurs anytime during transfusion and up to several hours after completion
◦ Tx: stop infusion, call for help, be prepared for code, be prepared to administer oxygen and Lasix
Blood reactions
Minimize the # of invasive procedure
Avoid prolonged tourniquet use
Avoid IM injections
Instruct the client to use soft bristled
tooth brush
 No strait edge razor shaving only use
electric razor
 Avoid NSAIDS
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Bleeding precautions
Nrsg diagnosis
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Risk for injury r/t bleed. Goal: cessation of bleeding aeb no
visual signs of bleeding and stable vs
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Acute pain r/t bleeding into closed spaces(creating pressure
on nerves. Goal: pain relief aeb: patient states pain is
relieved and appears to be in relaxed manner.
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Ineffective therapeutic regimen management r/t lack of
knowledge about dx process and self care. Goal: effective
management of condition aeb patient accurately describes
condition and demonstrates self care measures.
ANY QUESTIONS???
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