Exam 3 Hematology

Question 1

Disorders of Blood Cell Amount or Volume (2)

Answer 1

Iron Deficiency Anemia

Aplastic Anemia

Question 2

Iron Deficiency Anemia =

Is it preventable/reversible?
Not a disease of?

Answer 2

= decreased amount of rbc’s, bone marrow produces smaller rbc’s bc contains less iron

Yes
Malnutrition- child can still be big

Question 3

Causes of Iron Deficiency Anemia (3)

Answer 3

• Caused by diet insufficiency in iron “white food” (pasta, milk bread)
• Vegetarian
• From formula -> cow’s milk (Ca interferes with absorption of iron, also makes you full easily)

Question 4

S/S of Iron Deficiency Anemia (3)

Answer 4

• Pallor (inside eye is pale/pink instead of dark pink/red)
• Weakness/Fatigue
• Altered CBC: Decreased Rbc’s, H/H, reticulocyte count

Question 5

Prevention/Teaching for Iron Deficiency Anemia

1) Breast feed/iron fortified milk until __ months
2) Iron fortified food intro at ___ months
3) Limit ___ milk to __-__ oz/day
4) ____ diet through _____

Answer 5

1) 6 months
2) 6 months
3) Cow’s milk, 16-24 oz/day
4) Varied, Adulthood

Question 6

Supplementation for iron deficiency anemia

• Give in ____ doses, on an ___ stomach
• Give with? why?
• Can ____ teeth, so educate to?

When do we do transfusions?

Answer 6

• divided doses bc can cause upset stomach, empty
• Vitamin C (orange juice) to help with absorption
• stain -> good oral hygiene

Only in extreme situations, or pt has cardiac compromise

Question 7

Aplastic Anemia =

It causes *

Answer 7

= Abnormality of bone marrow stem cells

Pancytopenia* = decreased wbc’s, rbc’s, platelets

Question 8

Causes of Aplastic Anemia (3)

Answer 8

1) Autoimmune Disorders
2) Infectious Processes (viral hepatitis)
3) Radiation

Question 9

S/S of Aplastic Anemia (3)

Answer 9

1) Anemia
- weakness, fatigue, pallor
2) Leukopenia
- Fever, frequent infections
3) Thrombocytopenia
- abnormal bleeding

Question 10

Aplastic Anemia Prevention and Teaching

1) _____ _____ to cause
2) Keep ____ away by (2)

Answer 10

1) Prevent exposure

2) Infections (wash hands, immunizations)

Question 11

Tx for Aplastic Anemia

• Transfusion ->
• Cure =

Answer 11

• Replaces but does not cure

- Bone marrow transplant - find donor

Question 12

Coagulopathy Abnormalities (3)

Answer 12

Immune Thrombocytopenia
Hemophilia
Von Willebrand’s Disease

Question 13

Immune Thrombocytopenia =

Answer 13

= Decreased # of platelets from decreased production, shorter lifespan dt immune mediated response -> antibodies cause platelet breakdown

Question 14

Immune Thrombocytopenia

• Tx only if?
• Is it life threatening?
• S/S (2)

Answer 14

• Only if significant bleeding
• No, around 6 months, then goes away
• petachiae, purpura (don’t blanch)

Question 15

Hemophilia =

Answer 15

= missing a clotting factor which participates in the clotting cascade -> inability to clot

Question 16

Cause of Hemophilia = *

Answer 16

Recessive, X linked (greater in males)

Question 17

Two types of Hemophilia

Answer 17

Hemophilia A = factor VIII deficiency

Hemophilia B = factor IX deficiency

Question 18

Hemophilia may be

• Mild =
• Severe =

Answer 18

• May go undiagnosed for awhile

- Bleeding in joint, can occur after trauma

Question 19

Von Willebrand’s Disease =

-Factor _____ carrier protein and ____ abnormalities cause changes in _____

Answer 19

= Genetic Disorder with missing/abnormal Von Willebrand factor (VIII)

• VIII, platelet, clotting

Question 20

Causes of Von Willebrand’s Disease (2)

Answer 20

Autosomal Dominant or Autosomal Recessive

Question 21

S/S of Decreased Clotting

1) Epistaxis =
2) _____ bleeding when (2)
3) ____ menses (1)
4) _____ pain dt -> esp in _____ (3)

Answer 21

Increased Abnormal Bleeding

1) Nose bleeds
2) Gingival bleeding when brushing teeth, dental procedure
3) Heavy, # of pads, tampons
4) Joint pain dt swelling of blood in joints (ankles, knees, elbows)

Question 22

Decreased Clotting Nursing Interventions Teaching (3)

Transfusions =

Answer 22

• Medical Alert Bracelet
• Signs of decreased platelets
• Sneeze with mouth open (less chance of blood vessels bursting)
• as needed

Question 23

Acute Injury Nursing Interventions (4)

Answer 23

• Apply constant pressure
• elevate extremity above heart ( to prevent pooling)
• cool compress (to vasoconstrict to stop bleeding)
• avoid moving injured area

Question 24

Tx for Von Willebrand’s Disease (2)*

Answer 24

• Factor VIII or IV administration

- Desmopressin (synthetic vasopressin) = stimulates release of Von Willebrand factor

Question 25

Disorders of Blood Cell Function or Anatomy (3)

Answer 25

Thalassemia Hereditary Spherocytosis Sickle Cell Disease

Question 26

Thalassemia = A ____ illness Highest in what races?

Answer 26

= anemia of varying severity d/t abnormally formed hemoglobin Hereditary Asian Americans, Mediterranean

Question 27

Minor (_____) Thalassemia = | S/S (2)

Answer 27

= (Dominant) one copy of thalassemia gene and one normal gene - May have no signs - May resemble iron deficiency anemia

Question 28

Major (____) Thalassemia = S/S - diagnosis around _ months

Answer 28

= (minor) two copies of thalassemia gene PIGH - Pallor - Irritability - Growth Retardation - may have bone expansion to compensate to produce more rbc's - Hepatosplenomegaly - spleen works hard to remove damaged blood 6

Question 29

Preventing infection for all coagulopathy abnormalities (3)

Answer 29

- Wash Hands - Penicillin - Vaccinations

Question 30

Tx for Thalassemia (3)

Answer 30

1) Transfusions 2) Splenectomy 3) Iron Chelation Therapy

Question 31

Transfusions for Thalassemia - every __-__ weeks with __-__ units of blood for how long?*

Answer 31

3-5 wk, 1-3 units, lifelong*

Question 32

Thalassemia 1) Splenectomy = ____ old blood and if its is overrun may cause _____ 2) Why Iron chelation?

Answer 32

1) = filters, splenomegaly | 2) bc so much transfusion, iron is very toxic to organs

Question 33

Sickle Cell Disease = *

Answer 33

= Change in genetic make-up causing sickling of RBC's , hemoglobin molecule is altered with one change to amino acid 6*

Question 34

Cause of Sickle Cell Disease* Highest in what ethnicities?

Answer 34

Autosomal Recessive* African American, Mediterranean

Question 35

S/S of Sickle Cell Disease (3)*

Answer 35

1) Acute Sequestration Crisis 2) Vaso-occlusive Crisis 3) Aplastic Crisis

Question 36

Acute Sequestration Crisis = - damaged/sickle cells ____ easily in spleen

Answer 36

= blood pools in the spleen causing blood volume loss and shock - pool

Question 37

Vaso-occlusive Crisis = - ____ d/t abnormal shape -> ____ of vasculature -> altered peripheral _____, tissue ____ - Severe ____**! ____ in hands/ft, _____ disturbances, damage to _____

Answer 37

= stressors cause RBC's to sickle - clumping, blockage, perfusion, hypoxia, - PAIN*, Swelling, Visual, organs Note: pain triggered by stressors (fatigue, stress) -> typicall on PCA pumps to manage pain

Question 38

Aplastic Crisis =

Answer 38

= Fighting an infection can cause profound anemia; ABRUPT AND LIFE THREATENING!

Question 39

Sickle Cell Teaching | How to increase RBC's (3)*

Answer 39

1) Folic Acid daily 2) Hydroxuria = chemo drug that activates fetal hemoglobin production, so they can be dependent on that rather than sickled RBC's 3) Water!*

Question 40

Treatment for Vaso-occlusive crisis in Sickle Cell Disease 1) treat ____ that led to crisis 2) Administer _____ _____ 3) ____ as ordered -> 4) ___ meds, as ordered, 5) _____ for patient

Answer 40

1) infection 2) IV fluids* 3) Oxygen -> prevents further sickling 4) Pain 5) Advocate

Question 41

Tx for Sickle Cell Disease (2)

Answer 41

- Transfusions | - Splenectomy

Question 42

Hereditary Spherocytosis = Cause = ____ illness, autosomal _____ Spleen ___ RBC's ->

Answer 42

= RBC's become sphere shaped dt loss of RBC membrane protein, unable to bind to oxygen correctly Hereditary, Dominant Destroys -> splenomegaly d/t difficulty RBCs passing through spleen

Question 43

S/S of Hereditary Spherocytosis (3)

Answer 43

1) Anemia 2) Jaundice - d/t hemolytic anemia process, bilirubin (broken down rbc's) released 3) Splenomegaly

Question 44

Tx for Spherocytosis (2)

Answer 44

1) Transfusion w severe cases | 2) Splenectomy