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Question 1

What are the lymph node structures?

Answer 1

Follicle
Medulla
Paracotex

Question 2

What is the function of the Follicle of the LN?

Answer 2

Site of B cell localization and prolif

• in out cortex
• 1’ follicles are dense and dormant
• 2’ follicles have pale central germinal centers and are active

Question 3

What is the function of the medulla of the LN?

Answer 3

Consists of medullar cords and medullar sinuses

- medullary sinus comm with efferent lymphatics and contain reticular cells (Fibroblasts) and macs

Question 4

What is the medullar cord?

Answer 4

Closely packed lymphocytes and plasma cells

Question 5

What is the function of paracortex of the LN?

Answer 5

Houses T Cells

• Region of cortex between follicles and medulla
• contains high endothelial venules through which T and B cells enter from blood
• Not well dev in pt with DeGeorge
• enlarges in extreme cellular immune response like viral infection

Question 6

what is contained in the marginal zones of the spleen?

Answer 6

Macs and specialized B cells and where the APC’s capture blood borne Ags for recognition

Question 7

What in terms of immune system is deficient in a pt with a splenectomy?

Answer 7

Decreased IgM
Decreased Complement
Decreased C3b
Increased susceptibility of encapsulated organisms

Question 8

What are the encapsulated organisms we worry about with a person with a splenectomy?

Answer 8

Please SHINE my SKiS
Pseudomonas
Strep p
Haemophilis Influenzae
Neisseria m
E coli
Salmonella**
Klebsiella p
Group B strep

Question 9

What is isoniazid?

Answer 9

It is a TB antibiotic

- INH Injures Neurons and Hepatocytes

Question 10

What process of synthesis can it interfere with?

Answer 10

heme synthesis by B6 deficiency

• one taking Isoniazid should be put on a B6 supplement in the pyridoxine form
• CNS effects, anemia, and peripheral neuropathy
• sideroblastic anemia

Question 11

Wha is B6 needed for?

Answer 11

Cysthationine
heme
niacin
histamine
and 5ht, Epi, NE, DA and GABA

Question 12

What are the main suspects if you have elevated conjugate (direct)bilirubin?

Answer 12

Biliary obstruction

tumor of liver

Question 13

What would you suspect if you elevated unconjugated bilirubin?

Answer 13

Haemolysis- the liver can keep up with the degradation of RBC’s and thus starts to secrete unconjugated
Gilberts syndrom

Question 14

What is prophyria?

Answer 14

group of disorders resulting from deficiencies in enzymes in the pathway for heme biosynthesis

Question 15

What is the classical presentation of acute porphyria?

Answer 15

Abdominal pain + neuro disturbances

- must have very high suspicion to Dx it

Question 16

What is porphyria cutanea tarda?

Answer 16

Deficiency in hepatic Uroporphyrinogin Decarboxylase

• cant make Coproporphyinogen III from Uroporphyrinogen III
• More commonly acquired by rare homozygous inheritance
• Risk factors: Increased iron stores, Hep C, estrogens, HFE hemochromatosis, African iron overload

Question 17

What are the symptoms for Porphyria Cutanea Tarda?

Answer 17

Bullous dermatosis- blistering skin lesions

• Scarring
• Hyperpigmentation
• Hypertrichosis- abnormal Excessive hair

Question 18

How do you Dx a person who you think has Porphyria Cutanea Tarda?

Answer 18

Lesions

- elevated Urine prophyrins with uroporphyrin..coproporphy

Question 19

What is the treatment for Porphyria Cutaea Tarda?

Answer 19

Avoid alcohol
Phlebotomy 500ml/week
Iron chelation

Question 20

What are the 2 forms of dietary iron?

Answer 20

1. Heme iron- best absorbed from hemoglobin and myoglobin of chicken beef and fish
2. Non-heme iron- cereal and vegetables. Taken up less avidly and vegetarians needs to be careful since must be converted from Fe3 to Fe2+

Question 21

what are the common lab findings for a person with iron deficient anemia?

Answer 21

low serum iron
Low serum ferritin- less than 10 
Low transferrin iron saturation
Increase in total iron binding capacity 
         Transferrin production is increased but low transferrin iron saturation

Question 22

45 year old man who is iron deficient, low iron and ferritin, increased total iron binding capacity. What is the next test?

Answer 22

Colonoscopy searching for a GI bleed

Question 23

what are some chronic non-hematolitic conditions associated with Anemia of Chronic Disease?

Answer 23

Infectious like TB, endocarditis and AIDs
Malignant like Hodkin’s, NHL, Metastatic Dz
Immunologic like RA, SLE and vasculitis
Ischemic heart disease
Traumatic

Question 24

how does one dx anemia of a chronic disease?

Answer 24

Low serum iron
Increase or normal serum ferritin (iron in cell)
Low transferrin

Question 25

what is the number one way to tell the difference between iron deficient anemia and anemia of a chronic disease?

Answer 25

increased or normal ferritin

-this happens because iron is in the cell

Question 26

what is hereditary hemochromatosis?

Answer 26

Primary iron overload 
autosomal inherited (recessive)

Question 27

What is type I hereditary hemochromatosis?

Answer 27

Cysteine to Tyrosine substitution at amino cid 282 or aspartate to histdine substitution at amino acid 63

Question 28

What are some other factos about type I?

Answer 28

HFE mutation so dont make hepcidine with high iron levles

• absorb to much iron
• low iron you should have HFE interacting with Transferrin receptor 1
• high iron you have have HFE interaction with Transferrin receptor 2 to induce hepcidin
• both of which dont happen

Question 29

How do you dx type I HH?

Answer 29

-High Serum iron
-High serum ferritin
-Liver biopsy with 1000+ ferritin
Biopsy is gold standard**
-High Transferrin saturation

Question 30

How do you treat type I HH?

Answer 30

take blood from them- phlebotomy treatment