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Flashcards in Hemeonc 10 Deck (24)
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1
Q

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?

A

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

2
Q

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

A

3-4 grams

3
Q

How is the body’s iron distributed?

A

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

4
Q

What is the term for an iron overload?

A

Hemochromatosis
(Primary=hereditary
Secondary=acquired)

5
Q

What are several ways you could acquire hemochromatosis?

A

transfusions
thalassemia
increased absorption
liver dz

6
Q

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

A

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

7
Q

What gene affects the Regulator protein involved in iron absorption

A

HFE gene (high Fe)

8
Q

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

A

%sat >55%, and high serum ferritin

Liver bx or MRI (more common)

9
Q

What clinical signs would you look for in hemochromatosis?

A
  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
10
Q

What treatments are available for hemochromatosis?

A

Phlebotomy, chelators

11
Q

Patients with sickle cell trait are at risk for what?

A

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

12
Q

How does sickle cell disease manifest itself clinically?

A

Hemolysis
Occlusion of vessels and
tissue infarcts

13
Q

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

A

Hemoglobin electrophoresis

14
Q

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

A

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)

15
Q

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

A

Dactylitis

16
Q

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

A

Spleen (autoinfarction)

17
Q

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

A

opiods
NSAIDS
hydration
oxygen

18
Q

What two factors cause cells to sickle?

A

dehydration

deoxygenation

19
Q

When would you consider treating a SSC with a transfusion?

A

acute chest syndrome
organ failure
priapism
stroke

20
Q
What is your diagnosis if your sickle cell patient was experiencing: 
Chest pain
Respiratory tract symptoms
Fever
Abnormal chest exam

How would you treat?

A

acute chest syndrome, oxygenation, transfusion

21
Q

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.

A

Moya moya syndrome

22
Q

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

A

Avascular necrosis of the hips

23
Q

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

A

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

24
Q

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

A

hematopoietic stem cell transplant