Hemeonc 10

Question 1

What is the max amt of iron the body can absorb in a day with the help of supplements? And how much iron is needed for daily function for a man? menstruating woman? Pregnant woman?

Answer 1

10mg max absorbed
Man uses 1mg/day
Menstruating women uses 2mg/day
Pregnancy needs 3mg/day

Question 2

What is a healthy amount of iron to have in the body on a given day?

Answer 2

3-4 grams

Question 3

How is the body’s iron distributed?

Answer 3

Mostly in hemoglobin
Some in storage (liver, spleen, bone marrow)
A little in myoglobin/cytochroms
very little in plasma

Question 4

What is the term for an iron overload?

Answer 4

Hemochromatosis
(Primary=hereditary
Secondary=acquired)

Question 5

What are several ways you could acquire hemochromatosis?

Answer 5

transfusions
thalassemia
increased absorption
liver dz

Question 6

What’s going on in hereditary (primary) hemochromatosis?

Answer 6

1)***Increased intestinal iron absorption
2) increase in circulatory iron
3) iron deposition in liver, spleen, heart
(autosomal recessive)

Question 7

What gene affects the Regulator protein involved in iron absorption

Answer 7

HFE gene (high Fe)

Question 8

What lab values will be red flag indicators for hemochromatosis? How would you confirm?

Answer 8

%sat >55%, and high serum ferritin

Liver bx or MRI (more common)

Question 9

What clinical signs would you look for in hemochromatosis?

Answer 9

1. Cardiac failure
2. Cirrhosis of the liver and increased risk of hepatoma
3. Endocrine abnormalities including diabetes mellitus and gonadal failure
4. Skin pigmentation (bronze)
5. Arthritis

Question 10

What treatments are available for hemochromatosis?

Answer 10

Phlebotomy, chelators

Question 11

Patients with sickle cell trait are at risk for what?

Answer 11

Renal abnormalities, mechanism is unclear. Typically SST is considered completely benign

Question 12

How does sickle cell disease manifest itself clinically?

Answer 12

Hemolysis
Occlusion of vessels and
tissue infarcts

Question 13

What lab test is performed to determine what type of hemoglobin a patient has?

Answer 13

Hemoglobin electrophoresis

Question 14

What type of hemoglobin do you have if you are:

1) normal
2) sickle cell trait
3) sickle cell disease
4) C disease
5) SC disease

Answer 14

1) A, C, F (more A, normal)
2) A, C, F, S (more A, normal)
3) C, F, S (more S, severe deficiency)
4) C (mild anemia)
5) C, F, S (equal C/S, moderate deficiency)

Question 15

Infarctions of the bones and structures of hands and feet leads to Hand-Foot syndrome, also called?

Answer 15

Dactylitis

Question 16

What organ is usually affected very early in sickle cell patients?

Answer 16

Spleen (autoinfarction)

Question 17

What treatments are available for uncomplicated sickle cell crisis (SSC)?

Answer 17

opiods
NSAIDS
hydration
oxygen

Question 18

What two factors cause cells to sickle?

Answer 18

dehydration

deoxygenation

Question 19

When would you consider treating a SSC with a transfusion?

Answer 19

acute chest syndrome
organ failure
priapism
stroke

Question 20

What is your diagnosis if your sickle cell patient was experiencing: 
Chest pain
Respiratory tract symptoms
Fever
Abnormal chest exam

How would you treat?

Answer 20

acute chest syndrome, oxygenation, transfusion

Question 21

What diagnosis is characterized by:
a tuft of vascularity that is bypassing occluded (large) vessel in brain. These patients are transfused chronically for 3-5 years keeping HgbS ,50%. If there is a second stroke, they may be transfused for life.

Answer 21

Moya moya syndrome

Question 22

If you saw:
Vaso-occlusion affecting blood supply to hips
Pain and disability
Artificial joint replacement at young ages
What would your diagnosis be?

Answer 22

Avascular necrosis of the hips

Question 23

What types of preventive measures would you take for a sickle cell patient?

Answer 23

Immunizations
annual Ophthalmology exam
if being transfused: monitor for iron overload, chelation therapy
hydroxyurea therapy (increases HgbF, hydrates cells)

Question 24

What treatment has the most ideal outcome for young children with SSD?

Answer 24

hematopoietic stem cell transplant