Hemeonc 10 Flashcards
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?
10mg max absorbed
Man uses 1mg/day
Menstruating women uses 2mg/day
Pregnancy needs 3mg/day
What is a healthy amount of iron to have in the body on a given day?
3-4 grams
How is the body’s iron distributed?
Mostly in hemoglobin
Some in storage (liver, spleen, bone marrow)
A little in myoglobin/cytochroms
very little in plasma
What is the term for an iron overload?
Hemochromatosis
(Primary=hereditary
Secondary=acquired)
What are several ways you could acquire hemochromatosis?
transfusions
thalassemia
increased absorption
liver dz
What’s going on in hereditary (primary) hemochromatosis?
1)***Increased intestinal iron absorption
2) increase in circulatory iron
3) iron deposition in liver, spleen, heart
(autosomal recessive)
What gene affects the Regulator protein involved in iron absorption
HFE gene (high Fe)
What lab values will be red flag indicators for hemochromatosis? How would you confirm?
%sat >55%, and high serum ferritin
Liver bx or MRI (more common)
What clinical signs would you look for in hemochromatosis?
- Cardiac failure
- Cirrhosis of the liver and increased risk of hepatoma
- Endocrine abnormalities including diabetes mellitus and gonadal failure
- Skin pigmentation (bronze)
- Arthritis
What treatments are available for hemochromatosis?
Phlebotomy, chelators
Patients with sickle cell trait are at risk for what?
Renal abnormalities, mechanism is unclear. Typically SST is considered completely benign
How does sickle cell disease manifest itself clinically?
Hemolysis
Occlusion of vessels and
tissue infarcts
What lab test is performed to determine what type of hemoglobin a patient has?
Hemoglobin electrophoresis
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
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)
Infarctions of the bones and structures of hands and feet leads to Hand-Foot syndrome, also called?
Dactylitis
What organ is usually affected very early in sickle cell patients?
Spleen (autoinfarction)
What treatments are available for uncomplicated sickle cell crisis (SSC)?
opiods
NSAIDS
hydration
oxygen
What two factors cause cells to sickle?
dehydration
deoxygenation
When would you consider treating a SSC with a transfusion?
acute chest syndrome
organ failure
priapism
stroke
What is your diagnosis if your sickle cell patient was experiencing: Chest pain Respiratory tract symptoms Fever Abnormal chest exam
How would you treat?
acute chest syndrome, oxygenation, transfusion
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.
Moya moya syndrome
If you saw:
Vaso-occlusion affecting blood supply to hips
Pain and disability
Artificial joint replacement at young ages
What would your diagnosis be?
Avascular necrosis of the hips
What types of preventive measures would you take for a sickle cell patient?
Immunizations
annual Ophthalmology exam
if being transfused: monitor for iron overload, chelation therapy
hydroxyurea therapy (increases HgbF, hydrates cells)
What treatment has the most ideal outcome for young children with SSD?
hematopoietic stem cell transplant
