אימונולוגיה

Question 1

which autoimunne disease is in a/w deff in complement C1,C2,C4

Answer 1

SLE

Question 2

What is the first test to do in suspicion of defficency in complement?

Answer 2

CH50

Question 3

DRESS syndrome

מחלה הנובעת מתגובה לתרופות

אילו תרופות גורמות לתסמונת ומהי ההליניקה

Answer 3

Clinical menefistation (2-6 month after strat of Tx)
triad of
* fever
* rash
* hepatitis
also:
face edema
cervical LAP
sore throat

medications
Carbamazepine
Phentoine
Phenobarbital
Lamotrigine
Alloperinoul

Question 4

Which blood cancer have indication for BM transplant?

Answer 4

ALL
AML
Lymphomas

Question 5

Which hematologic condistion can be healed with BM transplant?

Answer 5

• Sick sell
• B-Thalasemia major
• Fanconi anemia
• Aplastic anemia

Question 6

What is the main problem in phagocyte in Chronic granulomatous disease

Answer 6

Can’t kill catalase positive due to defect production of ROS

Question 7

A kid with reccurent pneumonias, lymphadenitis, skin abcess has come for the clinic.

you send him to NTB test and the NTB didn’t turn blue.

what is most likely the Dgx?

Answer 7

CGD

Chronic grnaulomatous disease

X-linked reseccive

ALL fungal and many bacteria (mainly SA) ARE CATALSE POSITIVE

Question 8

How to dgx CGD?

Answer 8

DihydroRHODAMINE FACS&raquo_space; if theres superoxide the dihydrorhodamine turn to rhodamine = green fluroscent compund

Question 9

נשירה מאוחרת של חבל הטבור עם לויקוציטוזיס משמעותי וזיהומים חדייקים חוזרים יכול להעיד על איזו מחלה?

Answer 9

Leukocyte adhesion deficiency

AR

Tx- BM transplant

Question 10

Which syndrome is a/w lesion in chemotaxis and opsonization of granalocytes?

Answer 10

JOB syndrome (hyper IgE syndrome)

AD- lesion in STAT3 gene

Th17 re defficient

Question 11

Tx for JOBS syndrome?

Answer 11

ppx for SA
somtimes IVIG

Question 12

Clinical presentation of JOBS syndrome

Answer 12

Cold skin abscesses + absent of fever during systemic infection

coarse facies
eosinophils might be eleveted

lack of PMN chemotaxis > absent inflammatory response

Question 13

which immunology disease is a/w abscent of lymphatic tissue?

when we start to see presentation? what we will se in the lab and what is the Tx?

Answer 13

Agammaglobulinemia

Presentation after 6 month = when maternal Abs disappear

lab- low to abscent B cells
Tx- monthly IVIG