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Flashcards in Chapter 40 Hematologic Power Point Deck (82)
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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?

Pain everywhere


What are some other symptoms of Sickle Cell Disease?

Hart murmurs (S3 heart sound)
--Cyanosis around lips
Cool temperature
Diminished pulses
--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
Decreased ROM
--due to vessel occlusions.
--Look for joint swelling and temp change
CNS effects:
--Low grade fever
--Seizures or stroke symptoms (due to frequent hypoxia
--Pronator drift
--Hand grasp
--Gain coordination


What are some interventions you can provide for Sickle Cell Disease? (Chart 40-2)

Administer oxygen
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?

Slow onset
Pallor, fatigue
Caused by poor nutrition, drugs, malabsorption, Crohns’ disease
High risk-elderly
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
---Immunosuppressive therapy


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)
Thrombus formation


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?

therapy: phlebotomy


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


Describe Leukemia.

White blood cell disorder
Uncontrolled production of immature white blood cells (WBC)
Lymphocytic (Lymphoblastic)
Myelocytic (Myeloblastic)
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?

Low H&H
Low Platelets
Abnormal WBC
Prolonged PTT


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).