hematological conditions

Question 1

anemia

Answer 1

deficiency in
- number of RBC
- quantity or quality of hemoglobin
- volume of packed RBC

Question 2

anemia causes

Answer 2

• blood loss
• hypoproliferative: impaired RBC production due to deficiency in iron, folate, vitamin b12
• hemolytic: increased destruction of RBC

Question 3

RBC function

Answer 3

transport O2 and CO2

Question 4

CM anemia

Answer 4

• body’s response to tissue hypoxia
  -Hb levels determine severity

Question 5

anemia cardiopulmonary CM

Answer 5

• additional attempts by heart and lungs to provide adequate O2 of tissues
• CO maintained by increasing HR and stroke volume

Question 6

RBC production

Answer 6

• erythropoietin is a glycoprotein primarily produced by the kidneys
• lifespan is 120 days

Question 7

alterations in erythropoiesis may decrease RBC production

Answer 7

• decreased Hgb synthesis
• defective DNA synthesis in RBC
• diminished availability of erythrocyte precursors

Question 8

sickle cell anemia

Answer 8

• hemolytic anemia from sickle Hgb gene
• autosomal recessive

Question 9

sickle cell RBC

Answer 9

• deformed, rigid shape which adheres to endothelium of vessels
• long inflexible chains
• stiff, angular

Question 10

populations at risk for sickle cell anemia

Answer 10

• african descent
• middle east
• mediterranean
• aboriginal tribes form india

Question 11

CM sickle cell disease

Answer 11

• 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

Question 12

sickle cell dx

Answer 12

• Hemoglobin electrophoresis
• peripheral blood smear
• skeletal radiographs
• MRI
• doppler studies
• chest radiography

Question 13

sickling episodes

Answer 13

• 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

Question 14

sickle cell crisis

Answer 14

• exacerbation causes vaso-occlusive crisis
• results from tissue hypoxia and necrosis due to inadequate blood supply

Question 15

complications of sickle cell disease

Answer 15

• gradual involvement of all body systems
• prone to infection
• acute chest syndrome
• pulmonary hypertension

Question 16

infections and sickle cell disease

Answer 16

• pneumonia
• due to failure of spleen to phagocytize foreign substances (usually by 2-4 years old)

Question 17

acute chest syndrome and sickle cell disease

Answer 17

• CM: fever, chest pain, cough, pulmonary infiltrates, dyspnea
• seen on chest x-ray
• etiology: chlamydia, pneumonia, parvo

Question 18

pulmonary infarction and sickle cell disease

Answer 18

• CM: pulmonary hypertension (usually cause of death), MI, HF, cor pulmonale
• dx ECG
• increased phospholipase A2 may predict impeding pulmonary hyperTSN

Question 19

nursing management sickle cell disease

Answer 19

• preventing sequelae
• alleviate symptoms
• minimize organ damage
• prompt tx
• teaching: avoid high altitudes, maintain fluid intake, tx infections
• O2
• blood transfusions
• stem cell transplant

Question 20

hydroxyurea

Answer 20

• 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

Question 21

folic acid supplementation for sickle cell disease

Answer 21

• chronic hemolysis leads to increased erythropoiesis which depletes folate stores
• replenish stores which reduces symptoms

Question 22

blood transfusions

Answer 22

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

Question 23

blood transfusion risks

Answer 23

• iron overload
• infections: hepatitis, HIV
• alloimmunity: immune response to donor cells
• increased blood viscosity with reduction in concentration of Hg S

Question 24

sickle cell disease prognosis

Answer 24

• diagnosed in childhood
• crises at 1-2 years of age
• life expectancy 42-48 years