PD8.5: Patient met adaptieve immuunsysteem Flashcards
adaptive immunity is inheritable
defects in human B-cell differentiation
defect in preBCR signaling
defect in preBCR signaling
- agammaglobuminemia
- by BTK defect
X-linked agammaglobunimia
spectrum of disease
- most patients with XLA develop recurrent bacterial infections, particularly otitis, sinusitis and pneumonia, in the first two years of life
- the most common organisms are S.pneumonia and H. influenzae
- the serum IgG is usually less than 0.2 g/l and the IgG and IgA are generally less than 0.02 g/l
common variable immunodeficiency (CVID)
- dysregulation syndrome
- granulomatous disease
- unexplained polyclonal lymphoproliferation
- affected familty member with antibody deficiency
common variable immunodeficiency (CVI)
- peak incidence 3rd decade
- familial clustering in 20%
- time from symptoms to diagnosis 4-6 years
- 20 year survival 64-67%
CVID
- enteroviral
- acquired
- Bcell precursors in peripheral blood
- Tcell (laboratory!!!!!!!!!) dysfunction
- increased risk malignant lymphoma, gastric carcinoma (atrophic gastritis)
- granulomatous disease
=accompanied with splenomegaly/lymphadenopathy
=noncaseating granulomata
=steroid therapy in case of vital organs
clinical complications of CVID
- recurrent respiratory tract infections
- bronchiectasis
- auto-immune disease
- granulomatous disease
- malignancy
- gastrointestinal pathology
IgA deficiency
- pt >4y who has a serum IgA of < 0.07g/L but normal serum IgG and IgM
- other causes of hypogammaglobulinemia have been excluded
- these pt have a normal IgG antibody response to vaccination
- risk of transfusion reaction caused by antibodies to IgA
IgG subclass deficiency
- <10% of normal level or impaired vaccination response
- disease spectrum: recurrent airway infections
- severe IgG1 deficiency relatied to other subclass deficiencies
- IgG2 related to IgA deficiency
- subclass deficiency may develop intro CVID
- IgG4 is not an immunodeficiency
selective antibody deficiency with normal immunoglobulins (SADNI)
- high risk for pulmonary infections and bronchiectasia
X-linked hyper IgM (XHIM)
recurrent bacterial/oppertunistic infections:
- pneumocystis carinii pneumonia
- chronic or intermittent neutropenia with oral ulcers
- IgM may be normal or elevated
DD hypogammaglobulinemia
- malignancy
=thymoma
=Bcell malignancy - systemic disorders
=immunodeficiency caused by excessive loss of Ig (diarree, nefrose) - drug induced
=glucocorticoiden
voorbeeldvraag
in diA
in case of immunodeficiency
no live vaccines
- rotavirus vaccines
- oral poliovirus vaccine
- measles-mumps-rubella vaccine
- chickenpox vaccine
- BCG vaccine
PID therapies
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