Acute Phase Cytokines

Secreted by monocytes and macrophages

Pyrogens

Released by TH2 CD4 cells

Inhibits macrophages

Inhibits TH1 CD4 cells

Secreted by TH1 CD4 cells

Stimulates macrophages

Secreted by TH1 Cells

Stimulates TH1 and TH2 cell proliferation, differentiation, activation

Target of immunosuppressants:

Tacrolimus and cyclosporine

On T cell

Binds B7 on APC cell- second signal

On CD4 T cells

Binds CD40 on B Cells- second signal

B cells

21 is EBV receptor

NK Cells

Macrophages

Binds Fc 

DAF- anti complement

all WBC, RBC and PLTs

deficiency= Paroxysmal Nocturnal Hemoglobinuria

iron deficiency anemia, thrombosis, hemolysis +Ham test

Histiocytes

Langerhans cells

Dendritic Cells

Bone marrow growth

Similar to GM CSF

IgE and IgG production

(TH2 cells-B cell switching)

Stimulates IgA production

(TH2 cells- B cell switching)

TH2 cells

Inhibits activated T cells and TH1

Regulatory T cells secrete

Modulates immune response

Secreted by virally infected cells

Neighboring cells synthesis ribonuclease

Degrade viral mRNA/proteins

Drains lower body

Drains left upper body

Junction of left subclavian and internal jugular veins

Positive (cortex) and Negative (medulla) selection of T cells

Double positive in cortex (CD 4 and 8)

Single positive in medulla (CD 4 or 8)

Classic pathway of complement

Antigen-antibody complex activates

C1 esterase deficiency causes angioedema

Alternative pathway of complement

Initiated by microbial surfaces (nonspecific)

Membrane attack complex

Deficiency of any one- susceptible to neisseria

Anaphylaxis

neutrophil chemotaxis

(w/ IL8 and LTB4)

decay accelerating factor

CD55 and 59

Prevents complement activation

All RBC, WBC and PLTs

missing: paroxysmal nocturnal hemoglobinuria

Reed Sternberg Cells

Hodgkin Lymphoma

Stimulates NK cells

IL 12 from macrophages

IFNs from viral infected cells

Secreted by macrophages

septic shock

anti-TNFα drugs used for RA, AS, Crohn's, psoriasis

non-specific lupus

scleroderma

RA

dematomyositis

polymyositis

sjogren's syndrome

mixed CT dz

Churg Strauss vasculitis

MPA vasculitis

X linked

Defect in B TK (tyrosine kinase)

Reucurrent Bacterial infections

Boys

Defective CD40 L

High IgM

X Linked or AR or NEMO deficient

Severe infections early in life

Eczema

Recurrent pyogenic infections

Purpura

Low IgM

Thymic aplasia

3rd and 4th branchial pouches aplastic

Hypocalcemia and tetany

No T cells

Viral, fungal, protozoan infection

22q11 deletion

Adenosine Deaminase Deficiency

Defect early in stem cell line of lymphocytes

Triad:

severe recurrent infections

chronic diarrhea

failure to thrive

no thymus on CXR

IgA deficient

Cerebellar Ataxia

Poor Smooth Pursuit

Telangectasias after 5 yeras

Lymphomas and Leukemias

Radiation sensitive

Increased AFP after 8 months

Sinus and lung infections

Asthma, atopy

Anaphylaxis with blood transfusions

Lack NADPH oxidase

Can't make H2O2-PMNs ineffective

Susceptible to catalase + (staph aureus, klebsiella, aspergililus, candida)

No blue on Nitro-blue tetrazolium

recurrent infection

prophylactic Bactrim

Chediak Higashi

Defective transport of enzymes to lysosome

LYST gene

Giant cytoplasmic granules in PMNs

Albinisim, Respiratory and Skin infection, Neuro disorders

Hyper IgE

Deficient IFNγ

PMNs don't come

Exzema, recurrent staph abscesses that are cold

Course facial features, doughy skin

2 rows of teeth