Diseases caused by developmental disorders of both T and B cells or lack of cell-to-cell interactions

More common in newborns and infants, common clinical features

In all forms of SCID, T cells are deficient (T-)

Reduced or absent Ig levels in the body, reduced or absent B cells in peripheral blood

T cells normal

Recurrent purulent bacterial infections, increased susceptibility to certain viruses

X-linked agammaglobulinemia (XLA)/Bruton's disease

Common variant immunodeficiency (CVID)/adult-onset or delayed-onset hypogammaglobulinemia

Mostly X-linked, male

Mutation of NADPH oxidase gene in phagocytes → unable to kill intracellular bacteria, continued chronic infection → accumulation of phagocytes and formation of granulomas

symptom

Abnormal function of the IL-12/IL-23/IFNy signaling circuit → weakens the killing effect of macrophages and T cells on intracellular bacteria

Caused by defects in any of the intrinsic components of complement, regulatory proteins or receptors

include

Eczema-infection-thrombocytopenia triad syndrome

Sex-linked recessive genetic disease, mostly male patients

The WAS gene mutation is located on the X chromosome (Xp11.2-11.23), and its encoded protein is responsible for regulating the cytoskeleton and forming the immune synapse.

WAS gene mutations lead to abnormal function of platelets and immune cells

AICD-related gene defects lead to immune disorders

NEMO, a key regulator of the NF-kB signaling pathway, is mutated, mostly in males.

No or little sweating, sparse hair, no or few teeth

recurrent purulent bacterial infections

In blood, semen, vaginal secretions, breast milk, saliva, cerebrospinal fluid

Sexual contact transmission

Bloodborne

mother-to-child vertical transmission

Nosocomial infections, occupational exposure, etc.

HIV-1—95%

HIV-2—weak pathogenic ability

shell

core

target cells

Killing mechanism

other cells

HIV epitopes frequently mutate

DC-SIGN on the surface of DC is an HIV receptor, which enables DC to phagocytose viral particles and become a "shelter" for the HIV virus.

After HIV invades cells, it enters a latent state. It does not express viral proteins at this time and will replicate when the time is ripe.

neutralizing antibodies

anti-p24 shell protein antibody

Anti-gp120 and gp41 antibodies

CD8 T cells

CD4 T cells

There are no obvious symptoms 3-6 weeks after infection, and CD4 T cells recover quickly after being reduced.

window period

Six months to several years after recovery from the acute phase, there are no symptoms or mild infection, but the immune system has gradually become exhausted and damaged.

AIDS symptoms appear, CD4 T cells continue to decline, and immune function is extremely weakened

Typical AIDS onset stage

Extremely elevated viral load

Publicity and education

Cut off transmission routes

Prevent hospital cross-infection

No effective vaccine yet

HAART (Highly Active Antiretroviral Therapy)

A combination of three or more antiviral drugs to treat HIV

Immune preparations and enzyme replacement therapy

immune reconstitution

Gene therapy