Chapter 24: Hematologic & Immunologic Dysfunction Flashcards
What are the main 3 hematologic differences in pediatric patients?
- hematopoiesis: formation of blood that begins before birth
- WBC count is highest at birth
- platelet levels are lower in newborns
What is anemia?
3 aspects
- 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
How does anemia affect the circulatory system?
4 effects
- 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
What are some treatments for anemia?
5 treatments
- transfusion
- nutritional intervention for deficiency anemias
- IV fluids to replace blood volume
- oxygen
- rest
What is iron-deficiency anemia?
2 aspects
- decreased oxygen-carrying capacity of blood d/t inadequate supply of iron
- generally preventable
What are some possible causes of iron-deficiency anemia based on the patient’s age group?
3 age groups
- 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
What are some treatments for iron-deficiency anemia?
3 treatments
- infants – no cow’s milk, iron-fortified foods/formula
- dietary iron
- iron supplements
What are 3 education topics when giving PO iron?
- may stain teeth – drink with straw
- take vitamin C with iron – facilitates absorption of iron
- dark tarry (green/black) stools
What is sickle-cell anemia?
3 aspects
- 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
Which population more commonly experiences sickle-cell anemia?
African Americans
What kind of hereditary pattern does sickle-cell anemia follow?
autosomal recessive
What is one advantage of sickle-cell anemia when compared to non-sickle-celled RBCs?
in areas where malaria is common, people with sickle-cell anemia tend to have a survival advantage
What are some treatments for sickle-cell anemia?
3 treatments
- bone marrow transplants
- transfusions
- hydration – can prevent sickling
What is a sickle cell crisis?
pain that results from sickling of RBC d/t obstruction of blood flow
What are some factors that can precipitate sickle cell crisis?
6 factors
- anything that increases the body’s need for oxygen
- anything that alters transport of oxygen
- trauma
- fever
- infection
- physical or emotional stress
What are the 3 types of sickle cell crisis?
- vaso-occlusive (VOC) thrombotic episode
- splenic sequestration
- aplastic crisis
What is a vaso-occlusive thrombotic episode?
4 aspects
- most common type of crisis
- very painful
- stasis of blood with clumping of cells –> ischemia –> infarction
- s/s:
– fever
– pain
– tissue engorgement
What is splenic sequestration?
4 aspects
- 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
What is aplastic crisis?
3 aspects
- diminished production of RBCs with increased destruction of RBCs
- triggered by viral infection or depletion of folic acid
- s/s:
– profound anemia
– pallor
What are some treatments for sickle-cell anemia?
6 treatments
- 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
What is thalassemia? What is the difference between the 2 types?
- 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.
What are the s/s of thalassemia?
11 s/s
- chronic hypoxia
- h/a
- irritability
- precordial and bone pain
- exercise intolerance
- anorexia
- epistaxis
- pallor
- FTT
- hepatosplenomegaly
- severe anemia (Hgb < 6 g/dL)
How is thalassemia treated?
2 treatments
- transfusions
- iron-chelating drugs (Desferal) which bind excess iron for excretion – use for hemosiderosis (iron overload)
What is hemophilia?
3 aspects
- 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)
Differentiate between the 3 types of hemophilia?
- 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
What are some s/s of hemophilia?
10 s/s
- 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
What are some treatments for hemophilia?
8 treatments
- 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