Immunodefeciency disorders
description
Transcript of Immunodefeciency disorders
![Page 1: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/1.jpg)
Immunodefeciency Immunodefeciency disordersdisorders
DR.FATMA ZAHRANIDR.FATMA ZAHRANI
![Page 2: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/2.jpg)
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.
![Page 3: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/3.jpg)
ImmunodefImmunodef..
Organs of immune system:Organs of immune system:
primaryprimary Bone marrow Bone marrow
ThymusThymus
SeconderySecondery Lymphoid tissues Lymphoid tissues
SpleenSpleen
![Page 4: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/4.jpg)
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)
![Page 5: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/5.jpg)
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
![Page 6: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/6.jpg)
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.
![Page 7: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/7.jpg)
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
![Page 8: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/8.jpg)
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
![Page 9: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/9.jpg)
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
![Page 10: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/10.jpg)
ImmunodefImmunodef..
3.SCID.3.SCID.
4.Phagocytic4.Phagocytic
killing( CGD).killing( CGD).
chemotaxis.chemotaxis.
5.complement.5.complement.
![Page 11: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/11.jpg)
ImmunodefImmunodef..
Secondry :Secondry :
HIV.HIV.
Malignency.Malignency.
medication.medication.
![Page 12: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/12.jpg)
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
![Page 13: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/13.jpg)
ImmunodefImmunodef..
Diagnosis:Diagnosis:
Nbt.Nbt.
Phage test.Phage test.
CH50CH50
Complement .Complement .
![Page 14: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/14.jpg)
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 .
![Page 15: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/15.jpg)
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
![Page 16: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/16.jpg)
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.
![Page 17: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/17.jpg)
ImmunodefImmunodef..
Bruton disease:Bruton disease:
![Page 18: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/18.jpg)
ImmunodefImmunodef..
Transient infantile Transient infantile hypogammaglobulinemia:hypogammaglobulinemia:
![Page 19: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/19.jpg)
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.
![Page 20: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/20.jpg)
ImmunodefImmunodef..
Treatement:Treatement:
Regular IVIG. Q4weeks.Regular IVIG. Q4weeks.
Aggressive parenteral Aggressive parenteral antimicrobial.antimicrobial.
Nutritional supports.Nutritional supports.
BMTBMT
![Page 21: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/21.jpg)
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
![Page 22: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/22.jpg)
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
![Page 23: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/23.jpg)
ImmunodefImmunodef..
Dignosis;Dignosis; cbc lymphopenia.cbc lymphopenia. no reaction to intradermal test.no reaction to intradermal test. CD markersCD markers CXRCXR
![Page 24: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/24.jpg)
ImmunodefImmunodef..
Treatement:Treatement:
aggressive antimicrobial aggressive antimicrobial therapy.therapy.
thymus transplantaionthymus transplantaion prognosis poorprognosis poor
![Page 25: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/25.jpg)
ImmunodefImmunodef..
Sever combind immundef.:Sever combind immundef.:
![Page 26: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/26.jpg)
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.
![Page 27: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/27.jpg)
ImmunodefImmunodef..
Diagnosis:Diagnosis: cbc wbc(high OR low)cbc wbc(high OR low) NBTNBT phagotestphagotest other other
![Page 28: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/28.jpg)
ImmunodefImmunodef..
TreatmentTreatment:: agrressive antimicrobial .agrressive antimicrobial . WBC transfusionWBC transfusion colony stimulation factors.colony stimulation factors. BMTBMT
![Page 29: Immunodefeciency disorders](https://reader036.fdocuments.us/reader036/viewer/2022070404/56813adb550346895da32527/html5/thumbnails/29.jpg)
ImmunodefImmunodef..
Complement def.Complement def. S&S:S&S: rarerare recurrent neisseria recurrent neisseria
infectioninfection Dignosis:Dignosis: CH50CH50 complement assay.complement assay.