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Hematologic Disorders
Jan Leynard Camposagrado, RN, USRN, MN
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- Consist of blood, bone marrow and reticuloendothelial system
- Blood is also considered an ORGAN
- Plasma is the fluid portion of theblood (55%)
Hematologic System
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Blood
- Composed of: RBC, WBC and Platelets
- 45% of blood volume
- Carries oxygen, nutrients and wastes from system
- Also carries hormones and antibodies
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Bone Marrow
- Site of hematopoiesis
- Marrow activity is limited topelvis, ribs, vertebrae and sternumin adult
- Liver and spleen resume blood production in marrow destruction
- Within the bone marrow are stem cells capable of differentiation
myeloid lymphoid
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Slide 002
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Red Blood Cells
- Disk shape provides large surface for ABSORPTION andRELEASE of oxygen molecules
- Primary function
- 120 days and removed by the reticuloendothelial cells
- Most of the iron is recycled to form new hemoglobin
- 5-day process of erythropoiesis
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White Blood Cells
- Protect the body from invasion by bacteria and other foreign entities
- Neutrophils arrive at the site within 1 hour of inflammation
- Initiate phagocytosis but short-lived
- Monocytes follow and continue phagocytosis for longer periods
- Macrophages are effective against fungi and viruses
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Lymphocytes
- Releases lymphokines for enhanced phagocytic activity
T-Lymphocytes
- Responsible for delayed allergic reaction and foreign tissue rejection
- Responsible for destruction of tumor cells
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3 Main Groups of T-Lymphocytes
1. Killer T-cells- Sensitized and stimulated in the presence of antigen- Destroy abnormal cells
2. Helper T-cells- Enhance activities of T-cells & B-cells
- Reduced in patients with HIV
3. Suppressor T-cells- Have the effect of switching off the immune system
- Controls too much T-cells
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- Releases immunoglobulin or antibodies
B-Lymphocytes
- Function in hypersensitivity reactionEosinophils & Basophils
- Eosinophils are impt in the phagocytosis of PARASITES
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T cells on the right, platelet on the center and rbc at the left
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- For clotting mechanism- Circulate freely in an inactive state in the endothelium- Normal life span of 7-10 days
Platelets
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Test Normal Range Description
Red Blood Cells M: 4.7 6.1 x 106
F: 4.2 5.4 x 106
Hemoglobin M: 13.5 17.5 g/dL
F: 11.5 15.5 g/dL
Delivers oxygen through circulation to
body tissues and returns CO2 from
tissues to lungs
Hematocrit M: 40 52%
F: 36 48%
Indicates proportion of plasma and
RBCs
Platelets 150, 000 400, 000/mm3 20, 000/mm3 serious;
10, 000/mm3 life-threatening
Diagnostic Findings
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Test Normal Range Description
White blood cells 5,000 10,000/mm3
Neutrophils 40 75% Indicates bacterial infection, inflammation,
stress and steroids.
Lymphocytes 20 50% Increased after bacterial and viral infection
Monocytes 1 10% Increased in acute and chronic infection,
and inflammation
Eosinophils 0
6% Increased in allergic states, parasites andmetastatic tumors
Basophils 0 2% Increase is very rare
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Anemia- Hemoglobin concentration is lower than normal
- Lower RBC than normallower amount of oxygen delivered
Three Broad Etiologic Categories:1. Loss of RBCdue to bleeding
2. Decreased production of RBC
3. Increased destruction of RBC
- Deficiency in co-factors- Lack of erythropoietin
- Overactive RES
- Abnormal RBC
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Classifications of Anemia
1. Hypoproliferative - marrow cannot produce adequately- due to medications
- lack of co-factors
II. Hemolytic Anemia - premature destruction of RBC
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Nursing Diagnosis
1. Activity Intolerance r/t weakness, fatigue & general malaise
2. Imbalanced Nutrition, less than body requirement r/t inadequate intake
of essential nutrients
3. Ineffective tissue perfusion r/t inadequate blood volume
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Hypoproliferative Anemia
A. Iron Deficiency Anemia- Inadequate intake of Iron resulting to low manufacture ofhgb
- Most common anemia
- Most common cause in male adult & post menopausal women is bleeding
- Most common cause in pre-menopausal women is menstruation
- Alcoholism causes GIT blood loss
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Assessment1. Pallor
2. Weakness and fatigue
3. Irritability
Diagnostic FindingBone Marrow Aspiration
- Most definitive
- Low ferritin level
- Seldom used
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Medical Management
1. Iron Preparation
- Ferrous sulfate, Ferrous gluconate6 month therapy
2. Iron Dextran
- IM for poor iron absorption
- ANST before giving the full dose
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Nursing Interventions
1. Give iron supplements between meals
2. Given with vit. C or multivitamins or fruit juice
3. Dont give iron with milk or antacid
4. Use a straw in liquid iron forms
5. Instruct pt. of S/E: constipation, black stool, foul aftertaste
6. Instruct high iron foods: Liver, bread and cereals, dark green leafy,meat, raisins, egg yolk, beans
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B. Aplastic Anemia- Rare disease where there is decrease or damage to marrow stem cellsFAT
- Significant neutropenia + thrombocytopenia are also seen
Causes:
1. Idiopathic2. Exposure to myelotoxic agents, autoimmune disorder
- benzene, gold compounds,chloramphenicol, arsenic, sulfonamides
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Diagnostic FindingBone Marrow Aspiration- Most definitive
- Demonstrates conversion of red bone marrow to fatty red bone marrow
Assessment1. Pancytopenia
2. Petechiae, purpura, bleeding, pallor, weakness, tachycardia, fatigue
3. Infections are common
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Medical Management1. Bone Marrow Transplantation
- Treatment of choice if a suitable donor exists
2. Immunosuppressive Therapy- Presumed that pts lymphocytes destroy the stem cell
- Antithymocyte & cyclosporine
3. Supportive Therapy
- Transfusion of RBC and platelets
- Discontinue any offending agents
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Nursing Interventions
1. Administer immunosuppressive medications
2. Administer blood transfusion as prescribed
3. Advise pt to obtain Medic-Alert bracelet
4. Assess carefully for signs of infection and bleeding
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Hemolytic Anemia
A. Sickle Cell Anemia- RBCs have a shortened lifespan
- RBC = decreased available O2 hypoxia
- Hemoglobin (hemoglobin A) is partly or completely replaced by an abnormalsickle hemoglobin (hemoglobin S)
- Happens when there is decreased O2 tension
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Spleen Primary site of sickling infarctionand phagocytosis by macrophage
infection
Lungs Infarctionpulmonary HPN Pulmonary infiltrate Chest pain, dyspnea,
CNS Infarction CVA Weakness
Kidneys Sicklingdamage to renal medulla Hematuria, renalfailure
Dehydration
Bone Increased erythroid production
(compensation)
Widening of medullary
spaces
Bone pain
Skin Infarction Skin ulcer Pain , decrease healing
Eye Infarction Scarring andhemorrhage
Decreased vision;blindness
Manifestations
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Sickle Cell Crisis
1. Vasoocclusive Crisis- Most common
- Stasis of blood with clumping of the cells in the microcirculation,ischemia and infarctionnecrosis
- s/sx: fever, pain and tissue engorgement
2. Splenic Sequestration
- Life threatening caused by pooling of blood in the spleen (autosplenectomy)
- Liver and Lungs are involved in adults
- s/sx: profound anemia, hypovolemia and shock
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3. Aplastic Crisis
- Causes production of RBC to shut down for about 10 days andlevel falls rapidly & marrow cannot compensate
- Results from infection with the human parvovirus
- s/sx: anemia and pallor
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Medical Management1. Hydroxyurea
- Chemotherapeutic agent used to decrease permanent formation of sickle cells
- S/E: a. Chronic suppression of WBC b. Potential development of malignancy
3. Transfusion Therapy
- Long term RBC transfusion- Risks include: a. Iron Overload
b. Infection and thrombosis from the sitec. Hepatitis and HIVd. Significant financial cost
2. Arginine
- Enhances nitric oxidepotent vasodilator to decrease pulmonary pressure
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3. Supportive Therapy
PAINsignificant issue
a. Lasting hours to days
b. Adequate hydration is important
c. O2 may also be required
d. Pain medication may be given (aspirin, nsaids, morphine)
e. Physiotherapy can also be used (heat, massage)f. Cognitive and behavioral intervention (distraction, relaxation)
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Nursing Interventions
1. Managing pain
- Make use of pain scale
- Relaxation and breathing exercises
2. Preventing and Managing Infection
- Monitor s/sx of infection
3. Minimizing deficient knowledge
- inform: Keep warm
Maintain hydration
Avoid stressful events
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B. G-6-PD- Glucose-6-Phospate Dehydrogenase Deficiency- G-6-PD produces an enzyme w/in the erythrocyte for membrane stability- Hemolysis only results when erythrocytes are stressed
- Commonly affected: African-American, Greek, Italian, Asian and Jewish
- X-linked defects- Medications causing hemolysis:
1. Anti-malarial2. Bactrim/Septra3. Aspirin
5. HydroDIURIL6. OHA7. Chloramphenicol4. Vit. K
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Assessment1. Asymptomatic most of the time2. Pallor, jaundice and hemoglobinuria
3. Hemolysis is often mild and self limiting
Diagnostic Test1. Screening Test (NB Screening)
2. Quantitative Assay of G-6-PD
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Medical Management1. Stop the offending medications
2. Transfusion if severe hemolysis occurs (Mediterranean Type)
Nursing Management1. Pt is educated and given a list of medications to avoid
2. Medic-Alert
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Polycythemia Vera- Uncontrolled mechanisms of the myeloid stem cellsresulting to hypercellular bone marrow activity.
- erythrocyte, leukocyte and platelet counts are elevated
Manifestations:
a. Increase blood volume - headache, dizziness, tinnitus, fatigue, paresthesiaand blurred vision.
b. Increase blood viscosity - angina, claudication, dyspnea, thrombophlebitis
c. Increase BP
d. Generalized pruritus
e. Splenomegaly
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Diagnostic Test1. Elevated erythrocyte count
2. Increased leukocyte and platelet
3. Elevated hematocrit (60%)
Complications1. Thromboses
- MI- CVA
2. Bleeding - Very large platelets but dysfunctional
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Medical Management1. Phlebotomy - to diminish blood viscosity
2. Anagrelide (Agrylin)inhibits platelet aggregation
3. Interferon Alpha-2b (Intron-A)for pruritus- anti-histamines are not effective
4. Chemotherapeutic Agents (Hydroxyurea)
- Suppress bone marrow but increases risk for leukemia
- Lowers incidence of thrombolytic complications than phlebotomy
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Nursing ManagementNurses role is primarily an EDUCATOR
1. Avoid smoking, obesity, poorly controlled hypertension
2. Avoid tight or restrictive clothing (stockings)3. Avoid crossing legs
4. Avoid aspirin
5. Minimize alcohol intake (GI bleeding)
6. Avoid Iron Preparations
7. Bathe in tepid or cool water or sodium bicarbonate
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Hemophilia
Hemophilia A
- Deficient or defective factor VIII
Hemophilia B- Deficient or defective factor IX
- Both hemophilias are inherited as X-linked traits
- Females are carriers and are asymptomatic
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Manifestations
1. Joint bleeding (hemarthrosis)75% of all bleeding
- knees, elbows, ankles, hips, wrist and shoulders
- Recurrent joint hemorrhages can result to ankylosis
2. Hematoma can occur without known trauma in severe factor deficiency
3. Damage sensation of the peripherals, weakness and atrophy
4. Spontaneous hematuria and GI bleeding
5. Most dangerous site of bleeding is the head
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Medical Management1. Infusion of Factor VIII and IX
- Given during active bleeding or as preventive measure before procedures
- Patient and family are instructed how to administer by IV at home
- Patient sometimes develop antibodies to the concentrate- If so, we give Recombinant Factor VIIa
2. Aminocaproic Acid (Amicar)
- Used to treat mucosal bleeding
3. Demopressin (DDAVP)
- Used for pt with mild forms of Hemophilia A
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Nursing Management
A. Extensive teaching about activity restrictions and self care measures
1. Avoid aspirin, NSAIDs, herbs and alcohol2. OTC cold remedies are avoided
3. Dental hygiene to avoid dental extractions4. Nasal packing is avoided5. Splint joints for signs of bleeding6. Avoid all injections if possible
7. Invasive procedures should be minimized or performed afteradministration of factor replacements
8. Frequently assess surgical sites post surgery
9. Wear Medic-Alert bracelet
10. Frequent v/s monitoring
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B. Analgesicsfor pain associated with hematoma and hemorrhage to joints
C. Warm bath promotes relaxation
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Procuring blood and blood productsDonors should be in good health and without any of the ff:
1. History of viral hepatitis
2. History of blood transfusions
3. History of untreated syphilis or malaria
4. History of drug abuse of IV drug use5. Skin infections
6. Recent history of asthma, urticaria or allergy to medications
7. Pregnant
8. History of untreated exposure to infectious diseases
9. Recent tattoo
10. Cancer
11. Aspirin usage within 48-72
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All donors are expected to meet the ffminimal requirements:
1. Body weight should exceed 50 kg (110lbs) for 450 mL donation
2. 17 y.o are disqualified
3. Oral temp should not exceed 37.5C
4. PR should be regular between 50-100 bpm
5. BP of 90/60180/100
6. Hgb should be at least: women - 12.5g/dL
men - 13.5g/dL
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Complications of Blood Transfusion
A. Febrile Non-Hemolytic Reaction- Reaction of pts antibodies to donor WBC
- s/sx: chills and fever
- Antipyretics for fever
B. Acute Hemolytic Reaction- Pt antibodies combine with donor RBC and start hemolysis
- Incompatibility of blood (hemolysis)- Errors in blood component labeling and patient identification- s/sx: fever, chills, low-back pain
- n/v, chest tightness, dyspnea- hypotension, bronchospasm
- Most dangerous and life threatening
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Management:
- Discontinue immediately- Meticulous attention to labels- Accurate identification of recipient- Maintain blood volume
C. Allergic Reaction- Due to hypersensitivity to allergens found in the transfused blood
- s/sx: urticaria, itching, flushing
Management:- anti-histamines for mild reactions
- Epinephrine and corticosteroid
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D. Circulatory overload- Too much blood infused- s/sx: dyspnea, orthopnea, tachycardia, sudden anxiety
- Jugular vein distention, increased BP- Crackles at the base of the lungs
- if noted, transfusion is discontinued
- Notify physician
- KVO with NSS
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E. Bacterial Contamination- Incidence is very low
- Usually due to organisms on the donors skin
- Most bacteria cannot survive in cold temperature used to storeRBCplatelets are at greater risk
s/sx:
- Fever, chills & hypotension
- Transfusion is discontinued
- IV with NSS- Notify physician and blood bank is informed