hematological conditions Flashcards
anemia
deficiency in
- number of RBC
- quantity or quality of hemoglobin
- volume of packed RBC
anemia causes
- blood loss
- hypoproliferative: impaired RBC production due to deficiency in iron, folate, vitamin b12
- hemolytic: increased destruction of RBC
RBC function
transport O2 and CO2
CM anemia
- body’s response to tissue hypoxia
-Hb levels determine severity
anemia cardiopulmonary CM
- additional attempts by heart and lungs to provide adequate O2 of tissues
- CO maintained by increasing HR and stroke volume
RBC production
- erythropoietin is a glycoprotein primarily produced by the kidneys
- lifespan is 120 days
alterations in erythropoiesis may decrease RBC production
- decreased Hgb synthesis
- defective DNA synthesis in RBC
- diminished availability of erythrocyte precursors
sickle cell anemia
- hemolytic anemia from sickle Hgb gene
- autosomal recessive
sickle cell RBC
- deformed, rigid shape which adheres to endothelium of vessels
- long inflexible chains
- stiff, angular
populations at risk for sickle cell anemia
- african descent
- middle east
- mediterranean
- aboriginal tribes form india
CM sickle cell disease
- asymptomatic except during sickling episodes
- cells rapidly hemolyzed: short span of 10-12 days
- jaundice in sclera
- enlargement of bones in face and skull: bone marrow expands as compensatory effort
- tachycardia
- cardiac murmurs
- enlarged heart
- HF
sickle cell dx
- Hemoglobin electrophoresis
- peripheral blood smear
- skeletal radiographs
- MRI
- doppler studies
- chest radiography
sickling episodes
- most commonly effects back, chest, extremities, abdomen
- triggered by low O2 tension in the blood
- precipitating factors: infection, dehydration, acidosis, plasma osmolality
- pain episodes accompanied by fever, swelling, tenderness, tachypnea, hypertension
sickle cell crisis
- exacerbation causes vaso-occlusive crisis
- results from tissue hypoxia and necrosis due to inadequate blood supply
complications of sickle cell disease
- gradual involvement of all body systems
- prone to infection
- acute chest syndrome
- pulmonary hypertension
infections and sickle cell disease
- pneumonia
- due to failure of spleen to phagocytize foreign substances (usually by 2-4 years old)
acute chest syndrome and sickle cell disease
- CM: fever, chest pain, cough, pulmonary infiltrates, dyspnea
- seen on chest x-ray
- etiology: chlamydia, pneumonia, parvo
pulmonary infarction and sickle cell disease
- CM: pulmonary hypertension (usually cause of death), MI, HF, cor pulmonale
- dx ECG
- increased phospholipase A2 may predict impeding pulmonary hyperTSN
nursing management sickle cell disease
- preventing sequelae
- alleviate symptoms
- minimize organ damage
- prompt tx
- teaching: avoid high altitudes, maintain fluid intake, tx infections
- O2
- blood transfusions
- stem cell transplant
hydroxyurea
- chemo agents that decreases formation of sickled cells
- increases Hgb F production
- increases RBC volume and hydration
- alters adhesion of RBC to endothelium
- side effects: chronic suppression of leukocyte formation, teratogenesis
folic acid supplementation for sickle cell disease
- chronic hemolysis leads to increased erythropoiesis which depletes folate stores
- replenish stores which reduces symptoms
blood transfusions
effective in
- exacerbations of anemia
- prevention of complications from anesthesia and surgery
- improving response to infection
- severe cases of acute chest syndrome
- preventing/managing complications form sickle cell disease
blood transfusion risks
- iron overload
- infections: hepatitis, HIV
- alloimmunity: immune response to donor cells
- increased blood viscosity with reduction in concentration of Hg S
sickle cell disease prognosis
- diagnosed in childhood
- crises at 1-2 years of age
- life expectancy 42-48 years
