Chapter 24: Hematologic & Immunologic Dysfunction

Question 1

What are the main 3 hematologic differences in pediatric patients?

Answer 1

1. hematopoiesis: formation of blood that begins before birth
2. WBC count is highest at birth
3. platelet levels are lower in newborns

Question 2

What is anemia?

3 aspects

Answer 2

• most common hematologic disorder of childhood
• decrease in the number of RBCs/Hgb – Hgb < 10 or 11 g/dL –> decreased oxygen-carrying capacity of blood
• 2 classifications:
  1. etiology – RBC/Hgb depletion
  2. morphology – RBC characteristics that alter function

Question 3

How does anemia affect the circulatory system?

4 effects

Answer 3

• hemodilution: decreased concentration of RBCs
• decreased peripheral resistance –> more blood returns to the heart
• increased cardiac circulation and turbulence –> murmur or HF d/t increased workload
• slowed growth d/t decreased cellular metabolism

Question 4

What are some treatments for anemia?

5 treatments

Answer 4

• transfusion
• nutritional intervention for deficiency anemias
• IV fluids to replace blood volume
• oxygen
• rest

Question 5

What is iron-deficiency anemia?

2 aspects

Answer 5

• decreased oxygen-carrying capacity of blood d/t inadequate supply of iron
• generally preventable

Question 6

What are some possible causes of iron-deficiency anemia based on the patient’s age group?

3 age groups

Answer 6

• premature infants – low fetal supply of iron
• 12 - 36 months – excessive cow’s milk (poor iron source and interferes with iron absorption)
• adolescents – rapid growth, poor eating habits, or menses

Question 7

What are some treatments for iron-deficiency anemia?

3 treatments

Answer 7

• infants – no cow’s milk, iron-fortified foods/formula
• dietary iron
• iron supplements

Question 8

What are 3 education topics when giving PO iron?

Answer 8

• may stain teeth – drink with straw
• take vitamin C with iron – facilitates absorption of iron
• dark tarry (green/black) stools

Question 9

What is sickle-cell anemia?

3 aspects

Answer 9

• partial or complete replacement of normal Hgb with abnormal Hgb (Hgb S) that is in a sickle shape
• sickle-cell shaped RBCs
• these obstruct blood flow –> engorgement and tissue ischemia –> hypoxia occurs –> damage to and impaired function of tissues

Question 10

Which population more commonly experiences sickle-cell anemia?

Answer 10

African Americans

Question 11

What kind of hereditary pattern does sickle-cell anemia follow?

Answer 11

autosomal recessive

Question 12

What is one advantage of sickle-cell anemia when compared to non-sickle-celled RBCs?

Answer 12

in areas where malaria is common, people with sickle-cell anemia tend to have a survival advantage

Question 13

What are some treatments for sickle-cell anemia?

3 treatments

Answer 13

• bone marrow transplants
• transfusions
• hydration – can prevent sickling

Question 14

What is a sickle cell crisis?

Answer 14

pain that results from sickling of RBC d/t obstruction of blood flow

Question 15

What are some factors that can precipitate sickle cell crisis?

6 factors

Answer 15

• anything that increases the body’s need for oxygen
• anything that alters transport of oxygen
• trauma
• fever
• infection
• physical or emotional stress

Question 16

What are the 3 types of sickle cell crisis?

Answer 16

1. vaso-occlusive (VOC) thrombotic episode
2. splenic sequestration
3. aplastic crisis

Question 17

What is a vaso-occlusive thrombotic episode?

4 aspects

Answer 17

• most common type of crisis
• very painful
• stasis of blood with clumping of cells –> ischemia –> infarction
• s/s:
  – fever
  – pain
  – tissue engorgement

Question 18

What is splenic sequestration?

4 aspects

Answer 18

• life-threatening type of crisis – death can occur within hours
• large volume of blood pools in spleen –> decreases blood volume –> shock
• s/s:
  – profound anemia
  – hypovolemia
  – shock
• treatment – splenectomy

Question 19

What is aplastic crisis?

3 aspects

Answer 19

• diminished production of RBCs with increased destruction of RBCs
• triggered by viral infection or depletion of folic acid
• s/s:
  – profound anemia
  – pallor

Question 20

What are some treatments for sickle-cell anemia?

6 treatments

Answer 20

• prevent sickling – rest, hydration, electrolytes, pain meds, transfusions, antibiotics
• encourage childhood vaccines –> decreases infection
• treat infections
• prophylactic antibiotics for children 2 months - 5 y.o.
• diet – iron and hydration
• splenectomy

Question 21

What is thalassemia? What is the difference between the 2 types?

Answer 21

• anemia that results from defective synthesis of Hgb, structurally-impaired RBCs, and a shortened life of RBCs
• 2 types:
  1. alpha – alpha chains affected; more common in Asian and African pop.
  2. beta – most common; 4 forms; more common in Greek, Italian, and Syrian pop.

Question 22

What are the s/s of thalassemia?

11 s/s

Answer 22

• chronic hypoxia
• h/a
• irritability
• precordial and bone pain
• exercise intolerance
• anorexia
• epistaxis
• pallor
• FTT
• hepatosplenomegaly
• severe anemia (Hgb < 6 g/dL)

Question 23

How is thalassemia treated?

2 treatments

Answer 23

• transfusions
• iron-chelating drugs (Desferal) which bind excess iron for excretion – use for hemosiderosis (iron overload)

Question 24

What is hemophilia?

3 aspects

Answer 24

• group of hereditary bleeding disorders that result from deficiencies of clotting factors
• X-linked recessive
• 3 types:
  – hemophilia A
  – hemophilia B
  – von Willebrand disease (vWD)

Question 25

Differentiate between the 3 types of hemophilia?

Answer 25

* hemophilia A: -- classic hemophilia -- deficiency of factor VIII * hemophilia B: -- Christmas disease -- deficiency of factor IX * von Willebrand disease (vWD): -- deficiency, abnormality, or absence of vWF -- deficiency of factor VIII -- affects both males and females

Question 26

What are some s/s of hemophilia? | 10 s/s

Answer 26

* bleeding into joint spaces --> impaired mobility --> bony changes --> disability * warmth * pain * bruising * decreased movement * epistasis * bleeding in GI tract * excessive bleeding after procedures * hemorrhaging * bleeding in spinal cord --> paralysis

Question 27

What are some treatments for hemophilia? | 8 treatments

Answer 27

* replacement of missing clotting factors * desmopressin (DDAVP) -- increases factor VIII * transfusions * meds -- steroids, NSAIDs * exercise * physical therapy * advise to shave only with electric razors * apply pressure and ice for 15 minutes for superficial bleeding