Flashcards in Chapter 40 Hematologic Power Point Deck (82):
Describe Sickle Cell Disease (Anemia)
-Genetic disorder with autosomal recessive pattern
-Formation of abnormal hemoglobin
--Healthy adults with normal hemoglobin have two alpha and two beta chains
--Sickle cell disease hemoglobin contains abnormal beta chains
-Most common in African Americans
What is the primary symptom of Sickle Cell Disease?
What are some other symptoms of Sickle Cell Disease?
Hart murmurs (S3 heart sound)
--Cyanosis around lips
--Roof of the mouth and sclera (African Americans)
Ulcers on legs
Damage to spleen and liver
--Enlarged lumpy liver
What are some other symptoms of Sickle Cell Disease?
Decreased urine output
--Chronic kidney disease
--due to vessel occlusions.
--Look for joint swelling and temp change
--Low grade fever
--Seizures or stroke symptoms (due to frequent hypoxia
What are some interventions you can provide for Sickle Cell Disease? (Chart 40-2)
Pain-hospitalizations/ opioids(no dependency problems)
Hydroxyurea (Droxia) increases # of HbF (may lead to leukemia)
Warm room(because they have problems with O2, they are colder)
What are some more interventions we can provide for Sickle Cell Disease? (Chart 40-3)
Prevent sepsis and multi organ dysfunction
-Hand washing and aseptic technique
-Flu shot and pneumonia shot
-Respiratory problems –wear a mask
-Assessment q 8hrs: skin, mucous membranes, lungs (crackles, wheezes), urine output, antibiotics
-HSCT (Hematopoietic Stem Cell Transfusion aka bone marrow transfusion-costly, many side effects)
Describe Glucose-6-Phosphate Dehydrogenase Deficiency Anemia.
-X link recessive disorder
-Missing enzyme which leads to destruction of RBCs
-Triggered by infection or drugs (aspirin, diuretics, high doses of vit C)
-Symptoms: Dark urine, enlarged spleen, pallor, SOB
How can you prevent G6PD deficiency?
-Prevention: Genetic testing
--Prevent infection (triggering agents)
-Hydrate: Prevent hemoglobin collection in kidneys and Acute kidney injury
--Mannitol- osmotic diuretic helps prevent AKI
Describe Immunohemolytic Anemia
-Abnormal immunity that results in destruction of RBC membranes
-May be due to trauma, infections, drug exposure
-Warm antibody- excess of IgG
-Cold antibody-IgM protein fixation
--Arteries in hands and feet constrict in response to cold or stress
--Therapy: Steroids; Splenectomy; Plasma exchange
Describe an Anemia due to an Iron deficiency.
-May result from blood loss, poor GI absorption or inadequate diet
-Evaluate for abnormal bleeding
-Increase Iron rich food, Iron supplement, Parenteral supplement
What are some manifestations related to an iron deficiency anemia?
-Manifestations: pallor, weakness, fatigue, fissures in corners of mouth, exercise intolerance
Describe a b12 deficiency
Failure to absorb Vitamin B12
Slow onset (vegan diets- lack of dairy products)
What are some manifestations of B12 deficiency?
Manifestations: pallor, jaundice, glossitis, fatigue, weight loss, paresthesia of feet and hands
What are some interventions for B12 deficiency?
Increase dietary intake
Vitamin supplements : B12 shots
B 12 shots are given once a week at first and once a month later as a maintenance ( therapy for life)
What is a Folic Acid Deficiency?
Caused by poor nutrition, drugs, malabsorption, Crohns’ disease
Management: diet and scheduled replacements
Describe Aplastic Anemia?
Bone Marrow failure to produce enough RBCs
Unknown cause ( exposure to toxic agents, viral infections)
What are some interventions for Aplastic Anemia?
-Bone marrow biopsy-replacement of cell forming marrow with fat
---Hematopoietic stem cell transplant
What is polycythemia vera?
-Cancer of RBCs
--Massive production of RBCs
--Massive production of Leucocytes
--Massive production of platelets
What is the assessment you do for polycythemia vera?
Assessment : cyanotic, flushed, itching,
Poor perfusion (blood flow may actually stop)
Increased demand on heart (hypertension)
What are some interventions for polycythemia vera?
Phlebotomy with apheresis
Increase hydration and promote venous return (chart 40-4 p805)
Describe hereditary hemochromatosis.
Autosomal recessive disorder
Excessive absorption of Iron leading to organ failure
What are some manifestations of hereditary hemochromatosis?
Manifestations: abd pain, enlarged liver, hyperglycemia, darkening of the skin
Later signs: diabetes, liver cirrhosis, endocrine gland failure, heart disease & death
What is an intervention for hereditary hemochromatosis?
What is Myelodysplastic Syndrome (MDS)?
Abnormal cell formation in the bone marrow
Cells have short life (pancytopenia)
Unknown cause (risk factors: smoking, aging, radiation/chemo therapy)
What si the therapy for Myelodysplastic Syndrome (MDS)?
-Therapy: hematopoetic stem cell transplant
Medication takes 3-6 months to work so supportive therapy is necessary- transfusion and erythropoiesis stimulating medications
White blood cell disorder
Uncontrolled production of immature white blood cells (WBC)
Acute or Chronic
What are some patient considerations of leukemia? (Chart 40-5)
-Cardiovascular: increased heart rate, low BP, murmurs, bruits, slow capillary refill
-Respiratory: increased respiration rate
-Skin: pale & cool, petechiae
-GI : weight loss, nausea, anorexia, decreased bowel sounds, constipation, abdominal pain and enlarged liver
What are some labs you can perform with Leukemia?
What is an intervention for leukemia?
Prevent infection: frequent thorough hand washing, Aseptic technique, respiratory mask, personal hygiene, fever, urine, VS, (q8hrs)-inspect mouth, chest
What are some other interventions for leukemia?
-Minimize injury: Acute mylogeneous leukemia- risk for bleeding: assess for signs
-Conserve energy: patients are fatigued due to low RBC. Small frequent meals( High in protein and carb).
What is a phamacologic intervention for leukemia?
Medication therapy: Erythropeiesis stimulating agents (only if Hemoglobin is
Describe what is the first phase of chemo therapy?
-Induction: started at time of diagnosis, lasts 7 days.
-Intense chemo therapy
-Goals is to suppress bone marrow and cause neutropenia. Pt susceptible to infections.
-Hospitalization is necessary up to 3 weeks
-Nausea, vomiting, diarrhea, stomatitis, alopecia, liver and cardiac toxicity, sepsis, DIC (disseminated intravascular coagulation)
What is the 2nd phase of chemo therapy?
Consolidation-Early remission (may consider transplant at this time)
What is the 3rd phase of chemotherapy?
Maintenance- : months and years later
Goals is to maintain remission
Describe interventions for chronic leukemia
Imatinib mesylate (Gleevec)- 1st choice chemo drug (Philadelphia chromosome positive)
Oral- well tolerated
Chronic lymphocytic leukemia –most prevalent
Genetic testing- chromosome 13- good prognosis/ 11&17 bad prognosis
Chemo remission but no cure
Stem cell transplant – patient risk.
What are some home care interventions for patients with leukemia?
-Patients on bleeding and infection precautions
-Therapy at home
-No live vaccines for up to 2 years post transplant
What are the common symptoms of leukemia?
weight loss, fever, frequent infections, SOB, weakness, pain or tenderness, fatigue, loss of appetite, swelling of the lymph nodes, enlargement of spleen or liver, night sweats, easy bleeding and bruising, purplish patches or spots
Describe bone marrow transplant.
Hematopoietic Stem Cell --Transplant (bone marrow)
-Eradicate bone marrow
-Therapy is lethal- patients are hospitalized on isolation- high risk procedure
-Bone Marrow Harvest- same day surgery. Donor must match.
-Site of collection- hip
-500-1000 ml is harvested. Donor regrows in few weeks
-Donor: fluid loss, infection, transfusion (RBC), pain- non aspirin analgesics
Describe Peripheral Blood Stem Cell for a bone marrow transplant.
-Stem cells in blood stream ( low concentration but artificially increased)
-Mobilization- increase the number of stem cells and WBC in blood
-Allogenic collection- hematopoietic growth factor
-Autologous collection- chemo/ hematopoietic growth factor
-Apheresis-1 to 5 collections
What are some patient considerations for the peripheral blood stem cell bone marrow transplant?
Patient Considerations: catheter clotting, hypocalcemia (tingling, numbness, muscle cramping), hypotension ( administer NS, Ca)
Describe Cord Blood Stem Cell in a bone marrow transplant.
-Collection from umbilical cord before placenta detaches
-Stored at the cord blood bank
-Used for individual it self of unrelated patient
What is the patient preparation for bone marrow transplant?
Patient undergoes treatment to destroy his/her own bone marrow (chemo/radiation higher than normal or non-myeloblastive)
What are some patient considerations for getting a patient ready for a bone marrow transplant?
-Patient considerations: nausea/vomiting, hair loss, mucous membranes sores.
-High risk for infection- limit visitors, private rooms, hygiene, dedicate equipment, strict aseptic technique
What is the assessment and what are some side effects for a bone marrow transplant?
-Assessment: q4hrs- VS, skin/mucous membranes
-Side effects 3-10 days post transplant
What is a T-0 transfusion and side effects?
– transfusion through the central line ( do not use regular IV catheter)
-Side effects: fever, hypertension (preservatives)
Acetaminophen, Benadryl, Hydrocortisone
Diuretics, hypertension medication
What is an engraftment?
Presence of “chimerism”
High presence= successful transplant
What does it mean to "Failure to Engraft"?
Allogenic and autologous transplant
What does it mean to be GVHD?
Allogenic- donor cells attach the host
What does Veno-Occulusive Disease mean?
Blockage of liver vessels by clotting & infection
Within 30 days- jaundice, URQpain, enlarged liver- supportive therapy
Assess- weight gain, fluid retention, hepatomegaly and increased girth.
What are the 3 complications for a bone marrow transplant?
-Failure to graft
-Veno- Occulsive Disease
Describe Hodgkin's lymphoma.
--Cancer of a single lymph node (chain)
-Large/painless lymph nodes
--Fever, weight loss and night sweats- poor prognosis
--Infection, nausea, vomiting, skin problems, constipation, diarrhea, sterility, development of secondary CA
What is non-hodgkin's lymphoma?
All other lymphomas that do not have Reed-Sternberg cells
What is the therapy for non-hodgkin's lymphomas
Dependent on tissue involvement
Monitor patient for nausea/vomiting, infection, skin problems…
What is multiple myeloma?
WBC cancer involving B-lymphocytes.
Low RBC & Platelet- clothing difficulty
What are some symptoms of multiple myeloma?
-Fatigue; Dysfunctional kidneys
---Paraprotein in urine and blood –premalignant condition
->10% bone marrow infiltrated with plasma cells
---Monoclonial protein in urine
---Osteolytic bone lesion
What are some interventions for multiple myeloma?
Peripheral neuropathy, thrombolytic event, nausea/vomiting, constipation/diarrhea
What are some medications for multiple myeloma?
Stem cell (autologous)
What is the Autoimmune Thrombocytopenia purpuria (idiopathic).
normal production but reduced ciruclation- not enough platelets
What is an assessment for Autoimmune thrombocytopenia purpuria (idiopathic)
Bruises, petechial rash on arms, legs, upper chest, neck, IC bleeding, anemia, low H&H
What are some interventions for Autoimmune thrombocytopenia purpuria (idiopathic).
IV immunoglobulins (prevent destruction of coated platelets )
Describe thrombotic thrombocytopenic purpuria.
Platelets clump together in capillaries
Tissue ischemia- kidney failure, MI, Stroke
Plasma removal and FFP transfusion
Aspirin- to thin the blood
Immunosuppressive therapy- high risk for infection!!!!
Hereditary bleeding disorder
Hemophilia A- Factor VIII deficiency
Hemophilia B-Factor IX deficiency
Prolonged bleeding time after minor cuts, joint and muscle pain, hemorrhages
Infusion of synthetic factor VIII
What are some labs for hemophilia?
Labs- prolonged aPTT and PT time
Describe heparin induced thrombocytopenia.
Autoimmune disorder induced by Heparin (unfractionated)
VTE & PE
Describe thrombin inhibitor therapy for heparin induced thrombocytopenia.
Argatrobin ( Argatroban)
Describe tranfusion therapy (table 40-6 &40-12)
-Must have MD order
-Must have signed patient consent
-Explain procedure to patient
-Blood specimen to be cross- match with donor
-Use 0.9% normal saline to administer blood
-2 licensed RNs (one of whom must be an RN administering blood)
-Order, consent, ABO & Rh, expiration, visually inspect for cloudiness, bubbles, discoloration
-Establish baseline VS ( immediately prior to transfusion)
What do you do after your blood transfusion therapy?
Remain with patient for the first 15- 30 min (organization specific)
Reassess VS after 15 min of beginning
Follow facility policy for rechecking VS
Look for: SOB, chills, hives itching
Hyperkalemia – due to cell damage
If a patient experiences adverse reactions,what do you do?
Stop the transfusion immediately and call the physician or rapid response team.
Cannot throw away the tubing and blood
Cannot flush the tubing for IV access
What is RBC-250mL?
Donor and recipient must be carefully matched
ABO type is inherited
Rh negative (missing Rh antigen) should not receive Rh positive blood
What is the platelets: 300 mL transfusion?
Only transfused if lab value is
Describe a plasma transfusion.
Given fresh to replace blood volume or clotting factors
FFP- given immediately after thawing
While plasma factors are still active
Infused over 30-60 min
Describe granulocyte (WBC) transfusion.
Not commonly transfused
Strict patient monitoring (MD presence)
Reactions more common
Describe autologous blood collection.
Patients own blood collected and stored up to 40 days (frozen 10 years)
Circulatory overload and bacterial contamination
Surgical blood collection- collected, filtered and transfused with in 6 hours
What is a febrile transfusion reaction?
Can occur after multiple transfusions
Chills, tachycardia, fever, hypotension, tachypnea
Prevention: use WBC filters, single donor transfusion
What is a hemolytic transfusion reaction?
Blood type incompatibility
Fever, chills, apprehension, headache, chest pain, hypotension, DIC
What is an anaphylactic reaction for transfusion?
Urticaria, itching, bronchospasm
May onset with in 24 hrs after transfusion
Describe a bacterial transfusion reaction.
Tachycardia, fever, chills, hypotension, shock
Describe circulatory overload.
Blood infused too quickly
Hypertension, bounding pulse, distended jugular veins, dyspnea confusion
Transfuse slow, diuretics, monitor I&O
Describe Graft vs. Host Disease( GVHD).
Rare and life threatening
Irradiated blood may prevent this (Destroyed T cells)
Describe transfusion related acute lung injury.
May occur with in 6 hours of transfusion
Provide a list of Acute Transfusion reaction.
-febrile transfusion reaction
-hemolytic transfusion reaction
-bacterial transfusion reaction
-Graft vs host disease (GVHD)
-Transfusion related acute lung injury