immunodefeciency disorders

Immunodefeciency Immunodefeciency disordersdisorders

DR.FATMA ZAHRANIDR.FATMA ZAHRANI

Immunodef. Immunodef. (con,d)(con,d)

Objectives:Objectives: By the end of this lecture you should :By the end of this lecture you should :

1.know the anatomy& physiology of immune 1.know the anatomy& physiology of immune system.system.

2.know when to suspect immunodef.2.know when to suspect immunodef.

3.know the classification of immunodef.3.know the classification of immunodef.

4.give example of each type.4.give example of each type.

5 know the lab.tests to diagnose.5 know the lab.tests to diagnose.

6.outline the treatment of different types. 6.outline the treatment of different types.

ImmunodefImmunodef..

Organs of immune system:Organs of immune system:

primaryprimary Bone marrow Bone marrow

ThymusThymus

SeconderySecondery Lymphoid tissues Lymphoid tissues

SpleenSpleen

ImmunodefImmunodef..

Cells of immune systemCells of immune system::

1. - B&T cells.(bone marrow&thymus)1. - B&T cells.(bone marrow&thymus)

- Natural killer cells- Natural killer cells

2.Mononuclear phagocyte (RES).2.Mononuclear phagocyte (RES).

3.Denteritic cells ( Langerhans cell ).3.Denteritic cells ( Langerhans cell ).

4.Graulocytes (nutrophils,esinophil –4.Graulocytes (nutrophils,esinophil –etc) etc)

ImmunodefImmunodef..

Immune response:Immune response:Bacteria----complement+phagocyteBacteria----complement+phagocyte ↓↓ denderiticdenderitic lymph node T cell arealymph node T cell area killed,diff.killed,diff. B cell areaB cell area plasma,memoryplasma,memory

ImmunodefImmunodef..

When to suspect immunodef.When to suspect immunodef.

Recurrent infection.Recurrent infection.

Delayed response to anti Delayed response to anti microbial.microbial.

Opportunestic infection.Opportunestic infection.

Failure to thrive.Failure to thrive.

Dysmorphic features.Dysmorphic features.

+ve family history.+ve family history.

ImmunodefImmunodef..

ClassificationClassification : :

PrimaryPrimary::

1.B cell defect : age 6months+1.B cell defect : age 6months+

encapsulated bacteria.encapsulated bacteria.

sinopulmonary disease.sinopulmonary disease.

eg: X-linked agammaglobulinemia.eg: X-linked agammaglobulinemia.

selective IgA defselective IgA def

ImmunodefImmunodef..

Primary:Primary:

2. T-cell defect: from early life.2. T-cell defect: from early life.

opportunestic bacteria.opportunestic bacteria.

Mycobacteria.Mycobacteria.

viruses.viruses.

chronic diarrhea.chronic diarrhea.

malabsorptionmalabsorption

dysmorphismdysmorphism

ImmunodefImmunodef..

T cell defT cell def..

Digeorge syndromeDigeorge syndrome

Thymus aplasia.Thymus aplasia.

parathyroid aplasia parathyroid aplasia

vertebral anomalies.vertebral anomalies.

cardiac anomalies.cardiac anomalies.

dysmorphismdysmorphism

ImmunodefImmunodef..

3.SCID.3.SCID.

4.Phagocytic4.Phagocytic

killing( CGD).killing( CGD).

chemotaxis.chemotaxis.

5.complement.5.complement.

ImmunodefImmunodef..

Secondry :Secondry :

HIV.HIV.

Malignency.Malignency.

medication.medication.

ImmunodefImmunodef..

Diagnosis:Diagnosis: B.cell defect:B.cell defect: Total IgG &subclasses.Total IgG &subclasses. specific ab.specific ab. T cell defect:T cell defect: wbc.wbc. T cell subset.T cell subset. skin testskin test

ImmunodefImmunodef..

Diagnosis:Diagnosis:

Nbt.Nbt.

Phage test.Phage test.

CH50CH50

Complement .Complement .

ImmunodefImmunodef..

Treatment:Treatment:- Aggressive parenteral anti microbial. Aggressive parenteral anti microbial. - Nutretional support.Nutretional support.- IVIG.IVIG.- G-CSF.G-CSF.- BMT.BMT.- Antiviral . Antiviral .

ImmunodefImmunodef..

Classification:Classification: PrimaryPrimary

Antibodies defAntibodies def . . Cell mediated defCell mediated def . . Combined defCombined def . .

Phagocytic dysfunctionPhagocytic dysfunction.. Complement def. Complement def. SeconderySecondery .Infection.Infection MalignencyMalignency Medication Medication

ImmunodefImmunodef..

Primary antibodies defPrimary antibodies def.. S&SS&S:: age :after 6 months of age.age :after 6 months of age. +ve family history.+ve family history. male (Bruton,s disease)male (Bruton,s disease) sinopulmonary disease.sinopulmonary disease. more frequentmore frequent prolonged even with treatementprolonged even with treatement hepatosplenomegalyhepatosplenomegaly +&- lymphadenopathy.+&- lymphadenopathy.

ImmunodefImmunodef..

Bruton disease:Bruton disease:

ImmunodefImmunodef..

Transient infantile Transient infantile hypogammaglobulinemia:hypogammaglobulinemia:

ImmunodefImmunodef..

Dignosis:Dignosis:

1. Total immunoglobulins—less than o.5g/dl1. Total immunoglobulins—less than o.5g/dl

IgG (total &subclasses)IgG (total &subclasses)

IgM,IgA &? IgE.IgM,IgA &? IgE.

2.B-cell markers (CD2.B-cell markers (CD

3.Isoheamagglutenins.3.Isoheamagglutenins.

4.4.

ImmunodefImmunodef..

Treatement:Treatement:

Regular IVIG. Q4weeks.Regular IVIG. Q4weeks.

Aggressive parenteral Aggressive parenteral antimicrobial.antimicrobial.

Nutritional supports.Nutritional supports.

BMTBMT

ImmunodefImmunodef..

Cell mediated immunodef.Cell mediated immunodef. S&SS&S:: age from neonatal periodage from neonatal period.candidasis.candidasis no sex prediliction.no sex prediliction. ? Family history (Mucocutaneous candidiasis? Family history (Mucocutaneous candidiasis early deathes.early deathes. ?dysmorphism (DiGeorge)?dysmorphism (DiGeorge) allopecia,nail dystrophy,FTTallopecia,nail dystrophy,FTT no BCG scarno BCG scar

ImmunodefImmunodef..

Digeorge syndromeDigeorge syndrome:: Sporadic,3Sporadic,3rdrd&4&4thth arches disease. arches disease. DysmorphicDysmorphic cardiac,vertebral anomaliescardiac,vertebral anomalies sever hypocalcemia(parathyroid sever hypocalcemia(parathyroid

aplasia)aplasia) convulsionconvulsion thmic aplasiathmic aplasia

ImmunodefImmunodef..

Dignosis;Dignosis; cbc lymphopenia.cbc lymphopenia. no reaction to intradermal test.no reaction to intradermal test. CD markersCD markers CXRCXR

ImmunodefImmunodef..

Treatement:Treatement:

aggressive antimicrobial aggressive antimicrobial therapy.therapy.

thymus transplantaionthymus transplantaion prognosis poorprognosis poor

ImmunodefImmunodef..

Sever combind immundef.:Sever combind immundef.:

ImmunodefImmunodef..

Phagocytic def.Phagocytic def. S&S:S&S: age any age.age any age. +ve family history (CGD)+ve family history (CGD) recurrent suppurativ infectionrecurrent suppurativ infection (skin,bone,sinopulmonary).(skin,bone,sinopulmonary). lymphadenopathy.lymphadenopathy. hepatosplenomegaly.hepatosplenomegaly.

ImmunodefImmunodef..

Diagnosis:Diagnosis: cbc wbc(high OR low)cbc wbc(high OR low) NBTNBT phagotestphagotest other other

ImmunodefImmunodef..

TreatmentTreatment:: agrressive antimicrobial .agrressive antimicrobial . WBC transfusionWBC transfusion colony stimulation factors.colony stimulation factors. BMTBMT

ImmunodefImmunodef..

Complement def.Complement def. S&S:S&S: rarerare recurrent neisseria recurrent neisseria

infectioninfection Dignosis:Dignosis: CH50CH50 complement assay.complement assay.