blood dyscriasias

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Bone Marrow Transplant

(Hematopoietic Stem Cell transplant)- bone marrow destroyed using radiation or chemo and later replaced with healthy stem cells •Goal is to eliminate leukemic cells from the patient's body- uses a combinations of chemo with or without radiation therapy •Autologous cells- patients own cells are collected before chemo •Donor cells- match human leukocyte antigens (HLA) to reduce rejection risk •Following transplant the patient is at high risk for infection and bleeding until the transfused stem cells begin producing WBC's again(if they don't get a bone marrow transplant they can die from infection or bleeding)

A.Petechiae on the upper chest(bleeding) B.Hypotension(bleeding) C.*Cyanotic nail beds/fingertips(correct; microvascular clotting)* D.Severe headache(bleeding in brain)

A nurse is caring for a patient who has Heparin-Induced Thrombocytopenia (HIT). The nurse should notify the provider and report possible small-vessel clotting when which of the following is assessed? A.Petechiae on the upper chest B.Hypotension C.Cyanotic nail beds/fingertips D.Severe headache

A.*Provide assistance with ambulation(T)* B.*Monitor oxygen saturation(T)* C.Weigh the patient weekly(F)(doesn't affect wt) D.*Obtain stool specimen for occult blood(T)*(thrombocytopenia) E.*Schedule daily rest periods(T)*

A nurse is planning care for a patient who has a Hgb of 7.5 and a Hct of 21.5. Which of the following should the nurse include in the plan of care? (select all that apply) A.Provide assistance with ambulation B.Monitor oxygen saturation C.Weigh the patient weekly D.Obtain stool specimen for occult blood E.Schedule daily rest periods

A.*You will need a monthly injection of vitamin B12 for the rest of your life(T)* B.*Using the nasal spray form of vitamin B12 on a daily basis may be an option(T)* C.An oral supplement of vitamin B12 taken on a daily basis may be an option(F) D.You should ↑ your intake of animal proteins, legumes, and dairy to increase B12 in your diet(F)(because they cant absorb the oral b12 medicine or food high in b12) E.Add soy milk fortified with B12 to your diet to ↓ the risk of pernicious anemia(F)

A nurse is providing discharge teaching to a patient who had a gastrectomy for stomach cancer. Which of the following information should be included in the teaching? (select all that apply) A.You will need a monthly injection of vitamin B12 for the rest of your life B.Using the nasal spray form of vitamin B12 on a daily basis may be an option C.An oral supplement of vitamin B12 taken on a daily basis may be an option D.You should ↑ your intake of animal proteins, legumes, and dairy to increase B12 in your diet E.Add soy milk fortified with B12 to your diet to ↓ the risk of pernicious anemia

A.Stools will be dark red in color(green to black) B.Take with a glass of milk if GI distress occurs(decreased absorption; take on empty stomach) C.*Foods high in vitamin C will promote absorption(correct)* D.Take for 14 days(4-6 weeks)

A nurse is teaching a patient who has a new prescription for ferrous sulfate. Which of the following should be included in the teaching? A.Stools will be dark red in color B.Take with a glass of milk if GI distress occurs C.Foods high in vitamin C will promote absorption D. Take for 14 days

A.Taking the temp every 4 hours to assess for fever(no involvement with bleeding) B.Maintaining the patient on strict bed rest to prevent injury(could help with bleeding) C.*Monitoring the patient for headaches, vertigo or confusion(correct)(urgent cuz worry of brain bleed)* D.Removing the oral crusting on scabs with mouth swabs four times a day(no cuz prone to bleeding)

A patient is admitted to the hospital for evaluation and treatment of thrombocytopenia. Which action is most important for the nurse to implement? A.Taking the temp every 4 hours to assess for fever B.Maintaining the patient on strict bed rest to prevent injury C.Monitoring the patient for headaches, vertigo or confusion D.Removing the oral crusting on scabs with mouth swabs four times a day

A.*Serum potassium level of 5.8 mEq/L(correct)(lethal arrythmias)* B.Urine output less than intake by 400mL C.Inflammation & redness of the oral mucosa(can be caused by chemo) D.Ecchymosis present on the anterior trunk(low platelet count)

A patient with acute myelogenous leukemia is receiving induction-phase chemo. Which assessment finding requires the most rapid action? A.Serum potassium level of 5.8 mEq/L B.Urine output less than intake by 400mL C.Inflammation & redness of the oral mucosa D.Ecchymosis present on the anterior trunk

A.*20-year-old with possible AML who has just arrived on the medical unit(new patient; need to assess and establish a plan of care)(correct)* B.38-year-old with aplastic anemia(decreased RBC, WBC and platelet) who needs teaching about decreasing infection risk before discharge C.40-year-old with lymphedema who requests help to put on TED hose before getting OOB(out of bed); D.60-year-old with non-Hodgkin lymphoma who is refusing the prescribed chemo regimen

After you receive the change-of-shift report, which patient will you assess first? A.20-year-old with possible AML who has just arrived on the medical unit B.38-year-old with aplastic anemia who needs teaching about decreasing infection risk before discharge C.40-year-old with lymphedema who requests help to put on TED hose before getting OOB D.60-year-old with non-Hodgkin lymphoma who is refusing the prescribed chemo regimen

Anemia- General Manifestations

As anemia becomes more severe, symptoms will be more pronounced: SKIN: pallor, *jaundice*, *pruritus* EYES: icteric(yellow color)(only in hemolytic) conjunctiva and *sclera* MOUTH: glossitis, smooth tongue CARDIAC: tachycardia, palpitations, bounding pulse, HF, MI RESPIRATORY: dyspnea, tachypnea, orthopnea, dyspnea at rest NEURO: Headache, vertigo, irritability GI: Anorexia, difficulty swallowing, sore mouth GENERAL: sensitivity to cold, weight loss, lethargy *with hemolytic anemia due to excess destruction of RBC's

Leukemia's & Lymphomas: Collaborative Care

Assessment- •Acute Leukemia- bone pain, joint swelling, enlarged liver or spleen, weight loss, fever, poor wound healing, manifestations of anemia, evidence of bleeding •Lymphoma- enlarged lymph nodes, fever, fatigue, infections Nursing Care-neutropenic precautions especially important for chemo induction and patient with bone marrow transplant (WBC< 1000) •Monitor for s/s of infection •Prevent infection- strict aseptic technique, limit visitors (mask PRN) •Private room- frequent hand hygiene •No fresh or raw fruits or vegies; no fresh flowers or plants in room •Monitor WBC, platelets •Protect from trauma (injections, venipuncture), electric razor, soft toothbrush •Alternate rest with activity WBC growth factors such as granulocyte Colony-stimulating factor (G-CSF, filgrastim) stimulate the production of WBC's (see ATI Medication Sheet) Mild if the ANC ranges from 1000-1500/microL Moderate with an ANC of 500-1000/microL Severe if the ANC is below 500/microL.

diagnostic studies CBC

CBC Chart p.626 •RBC are the major carriers of Hgb in the blood •Hgb transports O2 to the cells and CO2 from the cells •HCT is the % of RBC's in relation to total blood volume Hgb and Hct •decrease with anemia, hemorrhage, hemodilution(lot of fluids on board(IV)) •Increase with polycythemia(too many RBC) or dehydration (hemoconcentration) Platelet Count- platelets help the blood clot •normal level is 150,000-400,000 •A count below 100,000/uL is called thrombocytopenia. •Below 10,000-20,000 spontaneous hemorrhage can occur!

Aplastic

Depression of bone marrow results in the normal formation, but reduced number of RBC, WBC, & platelets (pancytopenia). 70% cause is unknown- may be secondary to radiation, chemotherapy, or infection (HIV, mono) or congenital origin If left untreated survival is 3-6 months Patient will have general manifestations of anemia along with thrombocytopenia (↓Plt =bleeding) and leukopenia (↓WBC=susceptible to infections) Treatment: Identify & remove causative agent; stem cell transplant; immunosuppressive therapy- patient will require protective isolation (neutropenic precautions) Labs: ↓H&H, ↓RBC, ↓WBC, ↓Plt, ↓reticulocyte count

Multiple Myeloma

Disease starts out with an excess production of plasma cells (B cells) which produce antibodies. •With multiple myeloma instead of producing disease fighting antibodies they produce M Proteins that do not fight infection and infiltrate the bone marrow •Patients urine will test positive for Bence-Jones Protein, because it is produced by the M Proteins •As the number of M Proteins ↑, the number of normal antibodies ↓ •The M Proteins destroy bone, making skeletal pain the major manifestation Effects men more than women and age is around 65

Folic Acid Deficiency

Folic acid is required for DNA synthesis leading to RBC formation and maturation. Cause may be decreased dietary intake (leafy green vegies, citrus fruit), and is often seen in alcoholics. May also occur with anorexia, drugs interfering with absorption (methotrexate(immune system suppressant used for rheumatoid arthristis), antiseizure drugs), or in hemodialysis patients. •Deficiency is treated with replacement therapy: usual dosage is 1mg/day. With chronic alcoholism up to 5mg/day may be required •S/S of Folic Acid Deficiency: general manifestations of anemia (coming up) •Labs: ↓H&H, ↑MCV (macrocytic), ↓serum folate, normal or low reticulocyte count

Normal Clotting Process

In normal clotting (hemostasis), the damaged vessel, platelets, and clotting factors work together to stop bleeding. 1.Vascular Response(vasoconstriction) 2.Platelet Plug forms (3-7 minutes)(platelets aggregate or clump) 3. Fibrin clot develops on the platelet plug formed by plasma clotting factors(thrombin fibrin come together to form blood clot) 4. Lysis of clot(keep blood flowing and get rid of clot(destroys clot)

A.Maintaining protective isolation B.*Promoting leg exercises and ambulation( correct; because you don't want the thick blood clotting)* C.Maintain patient on bedrest to protect from falls D.Promoting hydration with a large oral fluid intake(don't want fluid overload)

In providing care for a patient hospitalized with an acute exacerbation of polycythemia vera, the nurse gives priority to which activity? A.Maintaining protective isolation B.Promoting leg exercises and ambulation C.Maintain patient on bedrest to protect from falls D.Promoting hydration with a large oral fluid intake

Anemia due to ↓ RBC Production: Iron Deficiency

Inadequate dietary intake of iron, malabsorption of iron, blood loss or hemolysis of RBC's (pregnant patient's also at risk due to diversion of iron to fetus; blood loss during delivery; lactation) Most at risk are pre-menopausal and men and pregnant women, ↓ socioeconomic status, older adults, and patients experiencing blood loss: diet teaching with an emphasis on foods high in iron and how to maximize absorption, is important for these patients Iron deficient cuz of nutrition- children adolescents and pregnant women Iron deficient cuz of blood loss(GI bleed)-post menopausal women, and men Menstruating women can have it due to heavy menstrual flow •Labs: ↓ H &H, ↓MCV (microcytic), ↓ serum ferritin, ↑ TIBC, normal or low reticulocyte count •See General Manifestations of Anemia (coming up) •May also see chelitis (inflammation of the lips, due to lack of iron in the tissue)

Polycythemia

Increased RBC's- there can be so many RBC's that there is too much blood (hypervolemia) and it is too thick (hyperviscosity) leading to congestion of organs and tissue with blood

Hemochromatosis

Iron overload- usually a genetic disorder, but can be secondary to chronic blood transfusions •Characterized by increased intestinal iron absorption → ↑tissue iron deposits •Normal range for total body iron 2-6 g; patient with hemochromatosis may exceed 50g! •Early symptoms: fatigue, arthralgia, abdominal pain, wt. loss •Late symptoms: liver enlargement, cirrhosis; then DM, skin bronzing, cardiac changes, arthritis, splenomegaly •Transferrin is going to be high •High serum iron •High total iron binding capacity(TIBC)(that was the one that reflects transferrin, which is needed to move iron into the cells for storage, so when theirs so much iron then theres gonna be a lot of movement into storage) •High ferritin which reflects the stored iron

Leukemia

Leukemia's are cancers of WBC's or of cells that develop into WBC's •In leukemia the WBC's are not functional. They invade and destroy bone marrow, and can metastasize to the liver, spleen, lymph nodes, testes and brain. •Divided into acute versus chronic disease and on the type of WBC involved: (Acute Lymphocytic Leukemia, Acute Myelogenous Leukemia, Chronic Lymphocytic Leukemia, Chronic Myelogenous Leukemia) •The goal of treatment is to eliminate all leukemic cells WBCs are being made but they don't fight off infection The overgrowth of leukemic cells prevents the growth of other blood components (platelet, RBC, WBC) •Lack of mature WBC's leads to immunosuppression & infection- leading cause of death •Lack of platelets increases the patients bleeding risk •All patient with Leukemia are predisposed to anemia, thrombocytopenia and ↓ WBC •See page 639 in Lewis for complete list of signs and symptoms

Non-Hodgkin's lymphoma-

More common in patients over 50; most commonly occurring hematologic cancer •Malignancy affecting the B cells, T cells or NK cells •Can originate outside the lymph system, spread can be unpredictable, disease often spread by time of diagnosis •Symptoms depend on spread of disease (i.e. hepatomegaly with liver involvement)- primary clinical manifestation is painless lymph node enlargement

Hodgkin's lymphoma-

Most cases involve young adults (long term survival 85%) •Reed-Sternberg cells- abnormal cells proliferate in the lymph nodes (isolated in lymph node biopsy) •Eventually spreads via the lymph system to the lungs, spleen, liver •Initial development is most often painless enlargement of cervical, axillary or inguinal lymph nodes •"B symptoms"= fever, night sweats, weight loss correlate with a worse prognosis •Staging by initial symptoms- next slide

Anemia- Collaborative Care

Nursing Care •Energy conservation measures- alternate rest and activity •Safety (i.e. dizzy, palpitations)- assist with ambulation, activity, ADL's •Neuropathy- safety measures with heat therapy and pain control(decreased level of alertness) •Encourage increased dietary intake of the deficient nutrient •Iron: Liver, eggs, red meats, whole grain cereals, leafy green vegies, dark beans •Folate: green leafy vegies, liver, meat, fish, legumes, whole grains, orange juice B12: dairy products, enriched cereal, egg yolks, some seafood When IF is not present, PO intake will not be absorbed, so parenteral B12 injections will be required for patient With malabsorption syndrome, intranasal administration may be effective

Polycythemia-Manifestations

Patient is hypertensive from ↑BV (& hyperviscosity): •h/a, vertigo, visual disturbance, tachycardia, angina, HF, CVA, PE ↑RBC production→ ↑RBC destruction •↑ uric acid produced→ possible gout •Plethora - ruddy complexion Patient can also have hemorrhagic phenomena cause by over distended and inadequate platelets. What s/s might you see if the platelets aren't working? Bleeding, petechiae, eccymosis, epitaxis, Gi bleed(can have clotting or bleeding)

Polycythemia- Collaborative Treatment

Phlebotomy- reduce blood volume until Hct returns to normal ±Generally 300-500ml removed every other day ±In primary disease may need to repeat every 2-3 months long term •Hydration- reduce blood viscosity- requires accurate I&O(don't do fluid overload) •Bone marrow suppression •Low dose aspirin- prevent clotting •Adequate activity and meds to prevent thrombus formation(ROM, ambulation)

Iron Preparations - ferrous sulfate (Feosol), iron dextran (INFeD) THERAPEUTIC USE, ADVERSE DRUG REACTIONS, INTERVENTIONS

THERAPEUTIC USE • *Iron-deficiency anemia* • Prevention of iron-deficiency anemia for clients at risk (infants, children, pregnancy, ongoing blood loss) ADVERSE DRUG REACTIONS • *Nausea, constipation, epigastric pain, diarrhea* • Metallic taste in mouth (iron dextran) • Staining of teeth (liquid form) • Fatal iron toxicity (overdose in children) • Iron dextran-Anaphylactic reactions or seizures INTERVENTIONS • *Recommend taking it with food for severe symptoms (understanding doing so reduces absorption).* • Monitor bowel patterns. • Give client hard candy to suck on or gum to chew for metallic taste in mouth • Monitor for staining. • Administer only recommended dose to children and to prevent accidental overdosage, place all lead products in childproof containers out of reach of children. Monitor for toxicity: severe gastrointestinal symptoms, shock, acidosis, and liver and heart failure. • For toxicity, administer a chelating agent, deferoxamine (Desferal), parenterally.

Folic Acid

THERAPEUTIC USE • *Megaloblastic (macrocytic) anemia* • Folate deficiency (alcoholism) • Prevent neural tube defects in developing fetus ADVERSE DRUG REACTIONS • *Increased yellowing of urine* • Masks B12 deficiency in high doses • Increased risk of developing colorectal or prostate cancer (long-term use) INTERVENTIONS • Encourage clients to eat a diet high in folic acid • Monitor for signs of megaloblastic anemia as well as plasma folic acid levels. ADMINISTRATION • *Give orally (preferable), subcutaneously, IM, or IV* • Check vitamin B12 levels to confirm absence of B12 deficiency • *Obtain baseline serum folate, Hgb, Hct, RBC, and reticulocyte count and monitor periodically thereafter Hct should start to improve within 2 weeks* CLIENT INSTRUCTIONS • Report rash as it may indicate sensitivity • *Encourage clients to eat a diet high in folic acid* CONTRAINDICATIONS • Vitamin B12 deficiency (after initial stabilization) • Other types of anemia • Neonates PRECAUTIONS • Vitamin B12 deficiency (after initial stabilization) • Other types of anemia • Neonates INTERACTIONS • Folic acid therapy can mask the symptoms of B12 deficiency; clients who have a B12 deficiency must take adequate doses of cyanocobalamin. • Oral contraceptives, corticosteroids, or methotrexate may cause manifestations of folic acid deficiency, but will not be affected by administering folic acid

Pernicious anemias - vitamin B12 cyanocobalamin (Nascobal)

THERAPEUTIC USE • *Pernicious anemia (lack of intrinsic factor)* • *Vitamin B12 deficiency (due to either malabsorption or dietary deficiency)* ADVERSE DRUG REACTIONS • Erythema • Hypertension • Hypokalemia INTERVENTIONS • Monitor potassium levels. • Monitor for potassium deficiency (muscle weakness, cardiac dysrhythmias). ADMINISTRATION • Give orally, subcutaneously, IM, or intranasally • *Confirm gastric absorption of B12 via Schilling test (for oral dosing)* • Most clients can absorb adequate amounts of vitamin B12 if large oral doses are given • *Give oral forms with food to enhance absorption* • Give intranasally or parenterally to clients who have malabsorption syndrome • *Give intranasally 1 hr before or after clients eat hot foods to avoid removal from nasal passages before absorption* • *Obtain baseline vitamin B12 Hgb, Hct, RBC, and reticulocyte count. Monitor every 3 to 6 months thereafter* • *Expect lifelong treatment for clients who have irreversible B12 deficiencies, such as pernicious anemia, usually parenterally.* With oral forms, high doses are essential • *Encourage dietary intake of foods high in vitamin B12 (dairy products, enriched cereal, egg yolks, some seafood)* CLIENT INSTRUCTIONS • *Report muscle weakness, nausea, palpitations, or paresthesia.* CONTRAINDICATIONS • Sensitivity to vitamin B12 other cobalamins • Hereditary optic nerve atrophy • Renal dysfunction • Concurrent infections PRECAUTIONS • Cardiovascular disease • Pulmonary disease • Other types of anemia • Concurrent folic acid use INTERACTIONS • Folic acid can mask the symptoms of B12 deficiency; clients must take adequate doses of cyanocobalamin. • Chloramphenicol (Chloromycetin) decreases effectiveness. • Alcohol, cimetidine, (Tagamet), colchicine (Colcrys), and aminosalicylic acid (Paser) reduce the absorption of oral forms. • Ascorbic acid (vitamin C) alters the stability of oral forms.

Leukopoietic growth factor - filgrastim (Neupogen)

THERAPEUTIC USE • Reduction of infection risk with • bone marrow transplantation • *severe chronic neutropenia* • myelosuppressive chemotherapy ADVERSE DRUG REACTIONS • Leukocytosis • Bone pain and fever • Splenomegaly (long-term use) INTERVENTIONS • *Monitor CBC twice a week during treatment.* • *Give a lower dose or stop treatment for a WBC above 10,000/mm3.* • Monitor the degree of bone pain. • Monitor temperature. • Give acetaminophen (Tylenol) or opioid analgesics. • Monitor for spleen enlargement. ADMINISTRATION • *Obtain a baseline CBC with differential and platelet count and monitor twice weekly thereafter*. • Administer filgrastim by intermittent or continuous IV infusion or subcutaneous injection. • *Do not shake vials* or mix with other drugs. • Use each vial for one dose. • Do not administer within 24 hr of cytotoxic chemotherapy. CLIENT INSTRUCTIONS • *Report bone pain or fever.* • Report abdominal pain or fullness. CONTRAINDICATIONS • Sensitivity to Escherichia coli-derived proteins • Acute respiratory distress syndrome • Current chemotherapy, radiation PRECAUTIONS • Bone marrow cancer • Pre-existing cardia disease INTERACTIONS • Filgrastim interferes with the action of cytotoxic drugs. • Lithium potentiates release of neutrophils

A.Platelet count(no) B.Hematocrit level(no, always low in anemia) C.*Reticulocyte count(yes, bone marrow working overtime)* D.Hemoglobin level(no)

The nurse checks the lab results for a patient suspected of having hemolytic anemia, knowing that which of the following would be increased in this disease? A.Platelet count B.Hematocrit level C.Reticulocyte count D.Hemoglobin level

A.Hearing loss (F) B.*Visual disturbance(T)* C.*Headache(T)* D.*Orthopnea(can breathe lying down)(T)* E.*Gout (T)(RBC die and release uric acid causing gout)* F.Weight loss(F)

The nurse is teaching a patient with polycythemia vera about potential complications from this disease. Which manifestations would the nurse include in the patient's teaching plan? Select all that apply. A.Hearing loss B.Visual disturbance C.Headache D.Orthopnea E.Gout F.Weight loss

A.Store vitamin B12 B.Digest vitamin B12 C.*Absorb vitamin B12(correct)* D.Produce vitamin B12

The primary purpose of the Schilling test is to measure the patient's ability to : A.Store vitamin B12 B.Digest vitamin B12 C.Absorb vitamin B12 D.Produce vitamin B12

Immune Thrombocytopenia Purpura (ITP)

Thrombocytopenia- Reduction of platelets below 150,000/uL •Below 50,000/uL prolonged bleeding can occur •Below 20,000/uL spontaneous, life-threatening hemorrhages can occur •Immune thrombocytopenic Purpura - ITP- (was idiopathic thrombocytopenic purpura) •Autoimmune disease in which the spleen recognizes the antibody on the platelets as foreign and destroys them •This can result in bleeding into the skin to severe hemorrhage

A.*Patient with digoxin toxicity(correct)* B.Patient with viral pneumonia(infective) C.Patient with shingles(infective) D.Patient with cellulitis(infective)

You are making a room assignment for a newly arrived patient whose laboratory test results indicate pancytopenia. Which patient will be the best roommate for the new patient? A.Patient with digoxin toxicity B.Patient with viral pneumonia C.Patient with shingles D.Patient with cellulitis

A.Philadelphia chromosome is present in the patient's blood smear(expected) B.Glucose level is elevated as a result of prednisone therapy(expected) C.There has been a 20-lb weight loss over the last year D.*The patient's chemo has resulted in neutropenia(correct)(because they are goin to a LTC, don't want them in a room with someone with an infection)*

You are transferring a patient with newly-diagnosed CML to a LTC facility. Which information is most important to communicate to the LTC charge nurse before transferring the patient? A.Philadelphia chromosome is present in the patient's blood smear B.Glucose level is elevated as a result of prednisone therapy C.There has been a 20-lb weight loss over the last year D.The patient's chemo has resulted in neutropenia

A.The patient reports chronic bone pain B.The blood calcium level is very elevated C.The 24-hour urine test shows Bence Jones proteins D.*The patient reports new-onset leg numbness(correct)*

You obtain the following data about a patient admitted with multiple myeloma. Which information has the most immediate implications for the patient's care? A.The patient reports chronic bone pain B.The blood calcium level is very elevated C.The 24-hour urine test shows Bence Jones proteins D.The patient reports new-onset leg numbness

Anemia due to RBC Destruction: Hemolytic Sickle Cell Disease

a group of inherited, autosomal recessive disorders characterized by an abnormal form of Hgb in the RBC (usually identified in infancy or early childhood). Often fatal by middle age due to renal failure, infection, pulmonary failure or stroke. •Sickling episodes are most commonly triggered by low oxygen tension in the blood- the malformed RBC's obstruct capillaries in joints and organs (spleen, lungs, kidneys, and brain) •Jaundice secondary to hemolysis of RBC's is common •Primary symptom associated with sickling is pain •Collaborative care→ alleviate s/s (pain and hypoxia) from disease complications and minimize end target organ damage The patient with hemolytic anemia will have the general manifestations of anemia along with some specific manifestations. •Sickle cell disease (SCD) inherited autosomal recessive disorder in which the Hgb forms long inflexible chains & alters the shape of the RBC. (see Genetics p. 645 Lewis) •The sickled RBC can become stuck in the capillaries and occlude blood flow causing tissue hypoxia Sickling episodes precipitated by hypoxia 2° to infection, stress, surgery, dehydration, etc. Sickle cell crisis occurs when the sickle shaped cells occlude BF causing painful vasospasm, tissue ischemia with necrosis and shock as possible outcomes (severe pain with tissue hypoxia/ischemia!) Labs: ↓H&H, Normal MCV (normocytic), ↑reticulocyte count

Cobalamin Deficiency Also called pernicious anemia or Vitamin B12 deficiency-

due to deficiency of intrinsic factor (IF), produced by gastric mucosa and necessary for absorption of vitamin B12 from the GI tract •Begins with insidious onset. The cause can be gastric mucosa atrophy, autoimmune (no IF), Crohn's, diverticuli, etc. (↓IF or malabsorption) •When IF is missing from the GI system, PO replacement will not be absorbed. Patient will require monthly parenteral (IM or SQ) replacement for life. Usual dose is 1000mcg/day IM for 2 weeks, then weekly until Hgb returns to normal then monthly for life •Signs & Symptoms of B12 Deficiency: •Labs: ↓ H&H, ↑MCV (macrocytic)(large cells but fragile), ↓serum B12, normal or low reticulocyte count •See General Manifestations of Anemia (coming up) •May also see neuro symptoms: weakness, paresthesia, ataxia(unsteady gait), impaired thought processes (confusion to dementia)

Chronic Myelogenous Leukemia (CML)

most cases in young adults; ↑ incidence ˃ 55 •Excess neoplastic granulocytes move from the bone marrow to the peripheral blood→ liver/spleen Philadelphia Chromosome in 90% of patients •Starts out chronic and eventually moves to an accelerated phase

Chronic Lymphocytic Leukemia (CLL)-

most common adult leukemia- slow progression •Functionally inactive lymphocytes infiltrate the bone marrow, spleen, liver and lymph system (↑WBC- not effective) •Pressure on nerves from enlarged lymph nodes can cause pain, paralysis, pulmonary symptoms

Acute Lymphocytic Leukemia (ALL)-

most common childhood leukemia; less than 50% of adults can be cured •Immature small lymphocytes multiply in the bone marrow •Fever at time of diagnosis; bleeding, weakness, fatigue, bone and or joint pain

Bone Marrow Aspiration-

specimen can be evaluated for ability to make blood cells (hematopoiesis- how well is the bone making blood cells), as well as pathology and chromosomal abnormalities 1.Informed Consent 2.Position depends on site (side-lying, prone, supine) 3.Local anesthesia- patient will feel pressure and then brief pain 4.Mild sedative may be prescribed 5.Apply sterile dressing- pressure to site if ↑ bleeding risk 6.Monitor the site for infection or bleeding Sternum or iliac crest are other sites used

Heparin Induced Thrombocytopenia (HIT)

•An immune mediated response to Heparin in which platelet-fibrin thrombi are formed and cause venous or arterial thrombosis - most common complications are DVT or PE; can also cause microvascular clotting, skin necrosis, stroke, organ damage (i.e. kidney) •Body makes antibodies to Plt/Hep complex→ removed from circulation= thrombocytopenia and platelet/fibrin thrombi §Develops 5-10 days after the onset of Heparin therapy (IV, SQ, or LMWH) §Incidence is ↑ with higher dose and longer duration of therapy §Suspect if platelet count falls by more than 50% or falls below 150,000/uL §After 5 days of IV Heparin, HIT occurs in 3% of patients §Approximately 20% of patients with HIT have a thrombotic event(DVT, PE, microvascular clotting)! §Limb loss occurs at 20-30%; mortality as high as 30% !!!

thrombocytopenia nursing care

•Avoid IM injections- if necessary, use smallest needle possible •For SQ injection- hold pressure for 5-10 minutes after injection; ice PRN •Monitor lab results- blood counts and coagulation studies •Monitor for signs of organ failure or intracranial bleeding (oliguria(low urine output), change in LOC)

Multiple Myeloma-Signs/Symptoms

•Development is slow and insidious. No symptoms until disease is advanced. •Skeletal pain major manifestation. Osteolytic lesions- seen on x-ray. •Results in diffuse osteoporosis (pathological fractures) •Hypercalcemia- calcium lost from bone to blood stream •Anemia, thrombocytopenia, neutropenia- related to the replacement of normal bone marrow with plasma cells •X-rays may also show bone fractures, especially vertebral or ribs •Patient may experience spinal cord compression from vertebral fractures- s/s: numbness and tingling below level of compression

Thrombocytopenia- Collaborative Care(HIT)

•Discontinue Heparin when HIT first recognized (SAFETY- check Platelet count when administering Heparin) •In severe cases patient may require plasmapheresis •Mark patient record- no more Heparin •No platelet transfusions if at all possible because it will make the problem worse •To maintain anticoagulation thrombin inhibitor will be started

There are three phases of chemotherapy used to treat leukemia

•Induction Therapy- intensive combination therapy lasts 4-6 weeks; goal is to induce remission •Post induction Therapy (consolidation therapy)- same meds as induction, but at lower dose and different combination; lasts about 6 months; goal is to eradicate any residual leukemic cells •Maintenance Therapy- lower doses of oral or IV chemo over 2-3 years to prevent relapse •SAFETY- Patient should report manifestations of infection or illness immediately to MD •Chemo may also be used to treat Non-Hodgkin's lymphoma •Radiation Therapy is the primary form of treatment for Hodgkin's lymphoma. May be used as part of treatment for Non-Hodgkin's lymphoma along with chemotherapy.

Anemia-Causes

•Low number of RBC or oxygen carrying capacity of RBC (Hgb, Hct)- indicates an underlying disease or disorder. •Results in ↓ O2 to tissues and organs •Hgb levels often used to determine severity of anemia •Mild (Hgb10-12) may have no symptoms or compensation with exercise (dyspnea, palpitation) •Moderate (Hgb 6-10) cardiopulmonary symptoms increase and occur at rest •Sever (Hgb < 6) dyspnea, ↑HR,↓BP, MI, HF (complete list Lewis p 608) Feel like they are not getting enough O2 even if they are getting lots of O2 in theres not enough hemoglobin to transport it around(like a fish out of water)

thrombocytopenia discharge

•Notify MD of any bleeding- what specifically would you tell the patient? (black tarry stools or frank blood, black bloody vomit, or sputum, bruising or small red or purple spots on the skin, bleeding from the mouth or anywhere on the body; any spontaneous bleeding, any headache or change in vision, any difficulty talking sudden weakness or confusion •Precautions for blowing nose; care for nosebleed •Prevent constipation- no suppository or enema •Soft toothbrush, electric razor, no tattoos or piercings •Avoid ASA(aspirin), herbal products- will decrease platelet aggregation •Keep track of # of pads if menstruating Check with MD before any invasive procedure

subjective assessment

•Past history: anemia, bleeding disorders, liver, kidney or spleen disorders, problems with clotting, exposure to radiation or chemicals •Medications: herbal products (can interfere with clotting)(garlic vit E, ginkgo, fish oil)(<- can increase bleeding risk) OTC meds, antineoplastics, long term anticoagulant therapy •Surgical history: What might you ask about here? Prostetic heart valve(on blood thinners), splenectomy, duodenal surgery(iron absorbed here), gastric bypass surgery, any trouble with wound healing, any problems with kidneys, problems with bleeding, any transplants •Family history: genetic links with Sickle Cell & hemochromatosis •Nutrition: balanced diet with foods high in iron and B12 •Increased alc use can cause GI bleeds and affect nutrition as wel

Polycythemia-Types

•Primary (Polycythemia vera)- increase production of RBC, WBC and platelets (so clotting problems too!) •Secondary Polycythemia •Hypoxic Driven- ↓O2 in the blood stimulates the kidney to release erythropoietin (EPO) which stimulates the production of more RBC's •Hypoxia independent polycythemia- EPO produced by a malignant or benign tumor

Iron Studies and b12 schilling test

•Serum iron= iron in the blood •Serum ferritin= iron stored in the body •Serum transferrin- needed to move iron from serum to storage [reflected in the total iron-binging capacity=TIBC] •↓ serum iron and ↑TIBC indicate iron deficiency anemia. (Transferrin increases in an attempt to maximize the use of the little iron that is available) Schilling test- tests for Pernicious Anemia (B12 deficiency) 1) Patient is given B12 24-hour urine for B12- if no B12 in urine(then that means where was no absorption of iron into circulation) absorption problem 2) Patient is given B12 + intrinsic factor (IF) 24-hour urine for B12. B12 in urine proves problem with absorption without IF

Thrombocytopenia

•When your patient has a low platelet count, they are prone to bleeding. What s/s would the nurse assess for? •Skin: petechiae, purpura, and ecchymosis •Procedure: prolonged bleeding after venipuncture or IM injection •Internal Hemorrhage: pale skin , weakness, fatigue, dizziness, elevated HR, decreased BP •What would you watch for in the patient with thrombocytopenia? Lower h/h, bleed into brain(confusion leading to seizure or coma), pg 624

A.*Hemochromatosis; deferoxamine(correct)* B.ITP; corticosteroids C.Anemia; ferrous sulfate D.Polycythemia; phlebotomy

A 45-year-old patient has symptoms including arthralgia, impotence, weight loss, and liver enlargement. His laboratory results include an elevated serum iron, TIBC, and serum ferritin levels. Which disorder does this describe and which treatment will be used? A.Hemochromatosis; deferoxamine B.ITP; corticosteroids C.Anemia; ferrous sulfate D. Polycythemia; phlebotomy

iron Preparations - ferrous sulfate (Feosol), iron dextran (INFeD) ADMINISTRATION, CLIENT INSTRUCTIONS, CONTRAINDICATIONS, PRECAUTIONS, INTERACTIONS,

ADMINISTRATION Ferrous sulfate (Feosol) • *Liquid preparations • dilute in another compatible liquid • give through a straw • rinse with water* • Spread doses evenly across waking hours to maximize the production of RBCs • *Give the drug on an empty stomach for best absorption* • Make sure clients do not crush or chew the sustained release forms • *Expect to discontinue the drug when anemia resolves (usually 1 to 2 months).* • *Recommend foods high in iron (liver, egg, yolks, muscle meats, wholegrain cereals, leafy green vegetables)* • Ensure dose of selected preparation is adequate in regard to providing needed amount of elemental iron • *Monitor Hgb, Hct, and reticulocyte count Iron dextran (INFeD)* • Give test dose first - follow with prescribed dose 1 hour later due to possibility of anaphylaxis • Have epinephrine available for hypersensitivity reaction. • *Administer using 2- to 3-inch-long needle using Z-track technique.* • Give prescribed IV doses no faster than 50 mg/min or dilute the dose for intermittent infusion in 200 to 1,000 milliliters of 0.9% NaCl (normal saline) (NS) and infuse over *1 to 6 hr*, depending upon concentration. Monitor blood pressure closely due to risk for hypotension • Do not take oral form of iron when receiving iron dextran • Monitor Hgb, Hct, and reticulocyte count CLIENT INSTRUCTIONS • *Take it with food to minimize gastrointestinal effects but understand that this reduces absorption.* • These effects diminish as drug therapy continues. • *Report persistent gastric irritation*. • *Expect dark green or black stools (harmless discoloration)*. • *For constipation, increase exercise and fluid and fiber intake.* • Recommend client suck on hard candy or chew gum for metallic taste in mouth • Dilute liquid forms with water and drink it through a straw; use a dropper to give it to infants. • Check the label; some liquid forms are compatible with milk and juice; some are not. • Rinse the mouth after taking it. • Store iron preparations in childproof containers and away from children's reach. Use a locked cabinet if necessary. CONTRAINDICATIONS • Hemolytic anemia • Severe liver disease, alcoholism, severe renal impairment • Peptic ulcer disease, ulcerative colitis, regional enteritis • Hemochromatosis Assessment Technologies Institute: Pharmacology Made Easy 4.0 3 PRECAUTIONS • Liver disease • Gastrointestinal disorders INTERACTIONS • Antacids reduce absorption. • Vitamin C increases absorption, but also increases the risk of gastrointestinal effects. • Absorption of tetracyclines, fluoroquinolones, bisphosphonates and penicillamine, will decrease when administered with iron supplements. • Concurrent use with ACE Inhibitors may increase the risk of anaphylactic reaction with iron dextran

Hematological System Review

Bone Marrow Produces: (all done through negative feedback) •RBC (erythrocytes)- oxygen transport •WBC (leukocytes)- protect against infection •Platelets (thrombocytes)- promote blood coagulation (clotting) -Iron is obtained from food or supplements and either bound to HGB or stored as ferritin in the bone marrow, liver, spleen or macrophages. -Liver, spleen and lymph system function in filtration of blood. When the tissue becomes hypoxic, then the kidneys release a substance called erythropoietin which causes stem cells inside the bone marrow to increase their RBC production to help increase O2 transport Dialysis patients have low RBCs cuz ertyhtopoetin is affected

Leukemia-Manifestations

Bone marrow failure: •Bone marrow over-crowded by abnormal cells •Inadequate production of normal marrow elements •Patient symptoms- anemia, thrombocytopenia, ↓WBC's •Often s/s fever, fatigue, wt. loss, infection, headache, mouth sores, bone pain

lymph node biopsy and chromosome testing

Lymph Node Biopsy •Needle or incisional biopsy •Obtain lymph tissue for histological exam Chromosome testing- can also be performed on tissue samples from lymph or bone marrow biopsy

Sickle Cell nursing care and discharge

Nursing •Oxygen- treat hypoxia and control sickling •IVF- hydration ↓ sickling •Pain control- PCA preferred •Treat infections •Only medication available: hydroxyurea- chemotherapy drug- decreases hemolysis, sickling & pain Discharge Teaching •Avoid infection (hypoxia triggers sickling) •Avoid dehydration •Pain control •Support groups

anemia symptoms

Physical Symptoms: lumps, easy bruising, bleeding, fever, night sweats, dyspnea, tachycardia, fatigue Tiredness, weakness, pale skin, irregular heartbeat, SOB, chest pain, dizziness, cold hands and feet, headache Values-Beliefs: OK with blood or bone marrow transfusions?

Multiple Myeloma- Collaborative Care

Seldom cured. Treatment can relieve symptoms, produce remission, and prolong life. Medications •Chemotherapy with corticosteroids •Bisphosphonates- inhibit bone breakdown, treat pain and hypercalcemia •Stem cell transplant (HSCT) •Localized radiation to reduce skeletal pain *Maintain fluid intake 3-4L/day to keep UO 1.5-2L/day- minimize damage from hypercalcemia (may require dialysis at end stage) *Activity helps to keep Ca in bone; use caution when moving or ambulating patient "brittle bones" *Pain management- NSAID's in combination with opioids- be aware of potential for constipation *Braces for immobilization to help with pain control *Prompt treatment of infections *Psychosocial support

Hodgkin's Lymphoma- Staging

Stage I, involvement of single lymph node. Stage II, involvement of 2 or more nodes on 1 side of the diaphragm. Stage III- lymph node involvement above and below the diaphragm. Stage IV- involvement outside the diaphragm (liver, spleen). Followed by A (absence) or B (presence) to indicate systemic symptoms (fever, night sweats, wt. loss)

A.An elevated hemoglobin level(no) B.An increased reticulocyte count(low because they do no have enough iron; no) C.An elevated RBC count(no) D.*Red blood cells that are microcytic(small) and hypochromic(pale)(yes)*

The nurse reviews a patient's laboratory results, knowing that which of the following results would indicate iron deficiency anemia? A.An elevated hemoglobin level B.An increased reticulocyte count C.An elevated RBC count D.Red blood cells that are microcytic and hypochromic

Hemochromatosis-Treatment

To remove iron, blood is removed (phlebotomy) at the rate of 500mL per week for 2-3 years, then less frequently to keep iron levels WNL Iron chelating agents form a complex with iron & promote excretion (PO, IV, SQ). Example is deferoxamine (Desferal) Dietary modifications- avoid Vitamin C & iron (liver, egg, meats, whole grain cereals, leafy greens)

A.*Patients with platelet deficiencies can have internal or external hemorrhage.(T)* B.All patients with ITP will be treated with platelet transfusions(F)( because they can make more antibodies; c them as foreign and destroy them) C.*ITP is characterized by increased platelet destruction by the spleen(T)* D.*A patient diagnosed with HIT should never receive Heparin again(T)* E.*A classic manifestation of thrombocytopenia that the nurse would expect to find on physical exam is petechia(T)*

Which statements accurately describe thrombocytopenia? (select all that apply) A.Patients with platelet deficiencies can have internal or external hemorrhage. B.All patients with ITP will be treated with platelet transfusions C.ITP is characterized by increased platelet destruction by the spleen D.A patient diagnosed with HIT should never receive Heparin again E.A classic manifestation of thrombocytopenia that the nurse would expect to find on physical exam is petechia

Lymphoma

are cancers of lymphocytes (type of WBC) & lymph nodes- can metastasize

Acute myelogenous leukemia (AML)-

poor prognosis •Normal cells in the bone marrow (& to a smaller extent other tissues) are replaced by leukemic myeloblasts •Myeloblasts will be present in bone marrow and WBC's. Excess production of myeloblasts will ↓ production of RBC's & Platelets •May have serious infection and abnormal bleeding from the onset of the disease

Thrombocytopenia- Collaborative Care(ITP)

•Asymptomatic patient- may not begin therapy unless platelet count < 30,000 /uL •Corticosteroids or IVIG to decrease the immune response (autoimmune dx.) •Splenectomy- decreases production of antiplatelet antibodies, ↓ phagocytic action •Platelet transfusion- platelet count < 10,000/uL (only do if life threatening hemmorhage, cuz they don't want them to make the antibodies again)

objective data

•Review assessment abnormalities on page 595 & 596 •Skin assessment for bleeding is important. In general, skin and mucosal bleeding indicates a platelet disorder. Enlarged Cervical Lymph Node Purpura (bleeding under skin) Petechiae


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