Transcript Immunodef.

Immunodefeciency
disorders
DR.FATMA ZAHRANI
Immunodef.
(con,d)

Objectives:
By the end of this lecture you should :
1.know the anatomy& physiology of immune system.
2.know when to suspect immunodef.
3.know the classification of immunodef.
4.give example of each type.
5 know the lab.tests to diagnose.
6.outline the treatment of different types.
Immunodef.
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Organs of immune system:
primary Bone marrow
Thymus
Secondery Lymphoid tissues
Spleen
Immunodef.
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Cells of immune system:
1. - B&T cells.(bone marrow&thymus)
- Natural killer cells
2.Mononuclear phagocyte (RES).
3.Denteritic cells ( Langerhans cell ).
4.Graulocytes (nutrophils,esinophil –etc)
Immunodef.
Immune response:
Bacteria----complement+phagocyte
↓
denderitic
lymph node T cell area
killed,diff.
B cell area
plasma,memory
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Immunodef.
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When to suspect immunodef.
Recurrent infection.
Delayed response to anti microbial.
Opportunestic infection.
Failure to thrive.
Dysmorphic features.
+ve family history.
Immunodef.
Classification :
Primary:
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1.B cell defect : age 6months+
encapsulated bacteria.
sinopulmonary disease.
eg: X-linked agammaglobulinemia.
selective IgA def
Immunodef.
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Primary:
2. T-cell defect: from early life.
opportunestic bacteria.
Mycobacteria.
viruses.
chronic diarrhea.
malabsorption
dysmorphism
Immunodef.
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T cell def.
Digeorge syndrome
Thymus aplasia.
parathyroid aplasia
vertebral anomalies.
cardiac anomalies.
dysmorphism
Immunodef.
3.SCID.
4.Phagocytic
killing( CGD).
chemotaxis.
5.complement.
Immunodef.
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Secondry :
HIV.
Malignency.
medication.
Immunodef.
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Diagnosis:
B.cell defect:
Total IgG &subclasses.
specific ab.
T cell defect:
wbc.
T cell subset.
skin test
Immunodef.
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Diagnosis:
Nbt.
Phage test.
CH50
Complement .
Immunodef.
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Treatment:
Aggressive parenteral anti microbial.
Nutretional support.
IVIG.
G-CSF.
BMT.
Antiviral .
Immunodef.
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Classification:
Primary
Antibodies def.
Cell mediated def.
Combined def.
Phagocytic dysfunction.
Complement def.
Secondery
.Infection
Malignency
Medication
Immunodef.
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Primary antibodies def.
S&S:
age :after 6 months of age.
+ve family history.
male (Bruton,s disease)
sinopulmonary disease.
more frequent
prolonged even with treatement
hepatosplenomegaly
+&- lymphadenopathy.
Immunodef.
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Bruton disease:
Immunodef.
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Transient infantile hypogammaglobulinemia:
Immunodef.
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Dignosis:
1. Total immunoglobulins—less than o.5g/dl
IgG (total &subclasses)
IgM,IgA &? IgE.
2.B-cell markers (CD
3.Isoheamagglutenins.
4.
Immunodef.
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Treatement:
Regular IVIG. Q4weeks.
Aggressive parenteral antimicrobial.
Nutritional supports.
BMT
Immunodef.
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Cell mediated immunodef.
S&S:
age from neonatal period.candidasis
no sex prediliction.
? Family history (Mucocutaneous candidiasis
early deathes.
?dysmorphism (DiGeorge)
allopecia,nail dystrophy,FTT
no BCG scar
Immunodef.
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Digeorge syndrome:
aplasia)
Sporadic,3rd&4th arches disease.
Dysmorphic
cardiac,vertebral anomalies
sever hypocalcemia(parathyroid
convulsion
thmic aplasia
Immunodef.
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Dignosis;
cbc lymphopenia.
no reaction to intradermal test.
CD markers
CXR
Immunodef.
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Treatement:
aggressive antimicrobial therapy.
thymus transplantaion
prognosis poor
Immunodef.
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Sever combind immundef.:
Immunodef.
Phagocytic def.
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S&S:
age any age.
+ve family history (CGD)
recurrent suppurativ infection
(skin,bone,sinopulmonary).
lymphadenopathy.
hepatosplenomegaly.
Immunodef.
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Diagnosis:
cbc wbc(high OR low)
NBT
phagotest
other
Immunodef.
Treatment:
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agrressive antimicrobial .
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WBC transfusion
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colony stimulation factors.
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BMT
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Immunodef.
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Complement def.
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S&S:
rare
recurrent neisseria infection
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Dignosis:
CH50
complement assay.