Type 1 hypersensitivity disorders

1. Anaphylaxis

2. allergies

3.Eczema

4. astma

HIP GEAR GOOD Boy's Prefer More

1. hemolytic anemia

2. ITP

3. Pernicious anemia

4. Graves'

5. Erythroblastosis fetalis

6. Acute hemolytic transfusion reactions

7. Rheumatic fever

8. Goodpasture's

9. Bullous pemphigoid

10. Pemphigus vulgaris

11. Myasthenia gravis

SSHARPP

1. SLE

2. Serum Sickness

3. Hypersensitivity pneumonitis

4. Arthus rxn

5. Rheumatoid arthritis

6. PAN

7. Poststreptococcoal glomermulonephritis

1.Type 1 DM

2.MS

3.GBS

4.Hashimoto's

5.GVD

6.PPD

7.Contact dermatitis

SLE, Sjogern's, Rheumatoid arthritis

Anti-dsDNA

Anti-Smith

Anti-Scl-70

(anti-DNA-topoisomerase I)

Antigliadin

Antiendomysial

antimicrosomal

antithyroglobulin

Anti-SS-A (anti-Ro)

Anti-SS-B (anti-La)

microscopic polyangitis

Churg-Straus syndrome

polymyalgia rheumatica

temporal arteritis

rheumatoid arthritis

infection, inflammation, malignancy

very nonspecific, not diagnositc

XR, increased in boys

defect in BTK, a tyr kinase gene

blocks B-cell differentiation, maturation

recurrent bacterial infections after 6 months due to opsonization defect

decreased number of B-cells and immunoglobulins of all classes

defective CD40L on helper T cells thus no class switching

severe pyogenic infections early in life

increased IgM

defect in isotype switching

IgA deficiency most common

sinus and lung infections, milk allergies and diarrhea

Anaphylaxis on exposure to blood products with IgA

CVID

(common variable immunodeficiency)

defect in B-cell maturation

acquired in 20s and 30s

inc risk of autoimmune dz, lymphoma, sinopulmonary infections

normal number of B cells but decreased plasma cells and immunoglobulins

22q11 deletion

failure to develop 3rd and 4th brachial pouches

tetany, hypocalcemia

recurrent viral and fungal infections

congenital heart and great vessel defects

thymus and parathyroids fail to develop thus decreased T cells and decreased PTH leading to decreased Ca

no thymic shadow on CXR

decreased TH1 response thus disseminated mycobacterial infections

decreased IFN-gamma

Thelper cells fail to produce IFN-gamma thus neutrophils do not respond to chemotactic stimuli

inc eosinophils

FATED - coarse Facies, cold Abscesses, retained primary Teeth, increased IgE, Derm prob (eczema)

T-cell dysfunction

tx = ketoconazole

XR --> defective IL-2 receptor (most common - decreased Tcell activation)

AR --> ADA deficiency (inc adenine toxic to B and T cells)

failure to syn MHC II Ag

recurrent viral, bacterial, fungal, and protozoal infections due to T and B-cell deficiency

Tx = BMT (no allograft rejection)

inc alpha-feto protein after 8 months of age

poor smooth pursuit

avg age of death = 25 y/o

defect in DNA repair enz

increased cancer risk so avoid x-rays

Triad: Ataxia, spider Angiomas, IgA deficiency

XR

progressive deletion of B and T cells

decreased IgM but increased IgE and IgA

Triad = Thrombocytopenic purpura, infections, eczema (truncal)

WAITER = Wiskott Aldrich Immunodeficiency, Thrombocytopenia, Eczema, Recurrent pyogenic infections

defect in LFA-1 integrin (CD18) on phagocytes

recurrent bacterial infections, absent pus formation, delayed separation of umbilicus

neutrophilia

AR

defect in microtubular function w/ decreased phagocytosis

recurrent pyogenic infections --> Staph and strep

partial albinism

peripheral neuropathy

Lack of NADPH oxidase thus decreased ROX and absent respiratory bursts in neutrophils

inc susceptibility to catalase (+) organisms (Staph aureus, E. coli, Aspergillus, Klebsiella, Candidiasis)

(-) Nitroblue tetrzolium dye reduction test