immunodeficiency Flashcards
When should you suspect immunodeficiency?
1-When infections are: —-
2- — as Known immunodeficiency,
excess infections,
unexplained deaths,
history-associated features
3- — risk factors as — history , — , and – use
4-Failure to — (children) / — (adults)
5- Lymphopenia in – (<2.8 x 109/L)
6- Reactions to —
7- Associated — malformations
8-Lymphoid —
Serious
Persistent
Unusual organism/site
Recurrent
family history
HIV risk factors
sexual , transfusion , IV drug use
thrive
weight lodd
infant
live vaccines
congenital
malgiancy
6 warnings signs for adults investigates if 2 or more present :
* 4 or more infections requiring – within 1 year (otitis,bronchitis, sinusitis, pneumonia)
* — infections or infection requiring— therapy
* Two or more severe — (osteomyelitis,
meningitis, septicemia, cellulitis)
* Two or more radiologically proven — within 3 years
* Infection with unusual — or unusual —
* PID — history
antibiotics
recurring injections
prolonged antibiotic therapy
bacterial infection
penumonia
localisation
pathogen
family
classification of immunodeficny :
* —
* —
( – , or — cause)
* —
(Secondary to something we — about)
physiological
primary
genetic
no known
secondary
we know ab
type of infection gives clues to immune detect :
1- T cell clinical features will be —>
2- b cell clinical features —>
3- neutrophil clinical features —>
4- complement clinical features — >
1- Viral & protozoal infections
Fungal infections – eg. Pneumocystis jirovecii pneumonia
2- Bacterial infections (esp. extracellular)
3- Boils & abscesses; Staph. aureus
4-Recurrent meningitis
Lupus-like disease
prevalence of primary immunodeficiencies:— deficiency - 1:300
Chromosome 22q — (DiGeorge) -1:3,000
Specific — deficiency - Uncertain — Variable Immunodeficiency - 1:10,000 approx.
Individually –
Collectively – —-
- whole secondary immunodeficiency has — growth
igA
deletion
antibody
variable
rare
not uncommon
rapid
to detect humoral immunity :
- Physiological
- Primary
- Secondary
( check slide12)
detect humoral immunity :
Primary ID
* — agammaglobulinaemia (XLA)
* —- immunodeficiency (CVID)
* — deficiency
* — subclass deficiency
* Defective – production
Secondary ID
* — directed therapies (e.g Rituximab)
* —
* Lymphoma/B cell malignancy/Myeloma
* – therapy eg cyclophosphamide, mycophenolate
* – splenectomy
* (Immunoglobulin Loss)
x liniked
common variable I
IgA
iGG
antibody
b ce;;
thymoma
drug
post
common variable immunodeficiency CVID:
* Low — (> – S.D. below mean for age)
* Low — or –
* Known causes of – excluded
Features:
* Onset – years old…can be insidious
* Diagnosis often – …bronchiectasis may already have set in
* Poor response to –
* Risk factor for granulomatous disease, autoimmune disease, malignancies
(esp. lymphoproliferative disease)
low igG
>2
igA or IgM
hypogammaglobulinaemia
2-80
delayed
vaccines
managing a patient w humoral immune defects:
1- minor by — and —
2- significant hypogammaglobulinaemia:
- by — replacement through IV; subcutaneous; facilitated subcutaneous
- aggressive management of —
- — , — , —
- management of other complications
antibiotics n vaccination
immunologbin replacmeent
infections
Physiotherapy/ bronchodilators/ antibiotics
( check slide 19,20)
fucntions of the spleen:
- is a — lymphoid organ where immune cells encounter —
- acts as — which removed abnormal particles from — as pathogens, abnormal blood cells, immune complexes
- causes of hyposplesim:
2dary
antigen
filter
bloodstream
causes:
Post-splenectomy
Congenital asplenia
Sickle cell anaemia
Coeliac disease
Note:
(Ultrasound imaging may be normal;Diagnosis based on detection of Howell-Jolly Bodies on blood film.)
Overwhelming post-splenectomy infection
(OPSI):
-High risk of infection by —
(eg. Strep. pneumoniae, Haemophilus influenza, Neisseria meningitides)
Also E. coli, malaria, others
Timing: - 65% within 1-2 yrs, 30% ~5 yrs, some >20 yrs
— - — mortality
- managed by:
encapsulated bacteria
50-70%
managed by:
Vaccination – Pneumovax, HiB, Meningococcal
Antibiotic prophylaxis
Advice re travel, animal bites
Medic Alert
immunodeficiencies associated w neutrophil dysfunction:
- Primary ID
—- (very rare):
—- (CGD)
—- defect
—- syndrome
- Secondary ID (Common)
Drugs eg: —
- —
- —-
genetic
chronic granulomatous
leukocyte adhesion
hyper igE
steroids
diabetes mellitus
myelodsyplsia
( check slide 26,29)
Managing a patient with primary neutrophil defects:
Only cure is —
- management of CGD:
* — – cotrimoxazole
* — – itraconazole
*—- for inflammatory complications
*— therapy is in clinical trials
HSC transplantion
antibiotic
anti fungal
steroids
gene
complement deficiency :
primary id: genetic by – levels or — protein
secondary id: —-
- management by:
low
dysfunctional
autoantibodies
no cure but we can use:
* Vaccination
* Prophylactic antibiotics
* Patient education
T cell / combined immunodeficiency:
— ID
* Severe combined immunodeficiency (SCID)
ADA deficiency
X-linked SCID & many more
* CD40 ligand deficiency (Hyper-IgM syndrome)
— ID
* HIV
* Chemotherapy
* Radiotherapy
* Transplant recipients
* Treatment for severe autoimmune disease
primary
2ndary
chrck slide 32 33
