IMMUNODEFICIENCY

Randanan Bandaso Department of Pathology Anatomy Faculty of Medicine Hasanuddin University

I R

D E F I C I E N T

PRIMER

SEKUNDER

IMMUNODEFICIENCY DISEASE

CONGENITAL (PRIMARY): AGAMMAGLOBINEMIA OF BRUTON. DIGEORGES SYNDROME SEVERE COMBINED IMMUNODEFICIENCY DISEASE (SCID) DEFICIENCY B,T,STEM CELL, COMPLEMENT. ACQUIRED (SECONDARY): AIDS, MALNUTRITION, AGING, IMMUNOSUPRESSIVE-RADIATION

PRIMARY IMMUNODEFICIENCY
X LINKED AGAMMA GLOBINAEMIA OF BRUTON ISOLATED IgA DEFICIENCY DI GEORGE SYNDROME SWISS TYPE AGAMMAGLOBINAEMIA SEVERE COMBINED ID (SCID) WISKOT ALDRICH SYNDROME GENETIC DEFICIENCY OF THE COMPLEMENT SYSTEM.

CAUSES PRIMARY IMMUNODEFICIENCY

EMBRYOLOGIC ABNORMALITY Eg DI GEORGE SYNDROME ENYMATIC DEFECT Eg CHRONIC GRANULOMATOUS UNKNOWN Eg WISKOT ALDRICH SYNDROME

BRUTON DISEASE:

NO B CELLS IN THE BLOOD AND LYMPHOID TISSUE X LINKED AGAMMAGLOBINAEMIA AFFECTING MALES LYMPH NODES VERY SMALL, NO TONSILS THEIR SERUM CONTAINS NO IgA, IgM, IgD OR IgE. IgG ONLY SMALL AMOUNT IN THE 6-12 MONTHS PROTECTED FROM INFECTION BY MATERNAL IgG AFTER MATERNAL IgG EXHAUSTED RECURRENT PYOGENIC INFECTION. INFUSED I.V. LARGE DOSES OF GAMMA GLOBULIN REMAIN HEALTHY

DI GEORGES SYNDROME

T CELL DEFICIENCY THYMUS AND PARATHYROID NOT DEVELOP LOST CELLULAR IMMUNITY EASY INFECTION BY FUNGUS AND VIRUS TETANY T CELL DEFICIENCY VARIABLE HOW BADLY THE THYMUS GLAND IS AFFECTED

SWISS TYPE A GAMMAGLOBINAEMIA

SEVERE COMBINED IMMUNODEFIENCY (SCID) RECURRENT INFECTION EARLY IN LIFE: CANDIDA, PNEMOCYTIS CARINI, PSEUDOMONAS, VIRUS AND BACTERI. VACCINATED WITH LIVE ORGANISMS eg VIRUS AND BCG DIE OF PROGRESSIVE INFECTION DIE IN THE FIRST OR TWO YEARS UNLESS RESCUED BY TRANSPLANTS OF BONE MARROW

WISKOTT-ALDRICH SYNDOME (WAS)

LOW THROMBOYT THROMBOCYTOPENIA AFFECTED MALES DEVELOP SEVERE ECZEMA AS WELL AS PYOGENIC AND OPPORTUNISTIC INFECTIONS. IN THE SERUM IgA AND Ig E INCREASED, IgG NORMAL, IgM DECREASED T CELLS DEFECTED IN FUNCTION. THE CAUSE UNKNOWN

SECONDARY IMMUNODEFICENCY DISEASES

CAUSES

RESULT FROM EXTRINSIC OR ENVIROMENTAL MOST DRUGS IN CANCER CHEMOTHERAPY CYTOTOXIC FOR T CELLS PROTEIN MALNUTRITION T CELLS DEFICIENCY LOSS OF IMMUNOGLOBULIN KIDNEY DISEASE, INFLAMMATORY BOWEL DISEASE, BURN INFECTIONS AIDS

AIDS
A CQUIRED I MMUNO D EFICIENCY S YNDROME

AIDS

RECOGNIZED AS EARLY 1979 INCREASED DRAMATICALLY EACH YEAR 1982 CENTER FOR DISEASE CONTROL (CDC) DECLARED AIDS A NEW EPIDEMIC 1987 40.000 CASES 1993 300.000 SERO POSITIF ICE MOUNTAIN PHENOMENA MOSTLY IN AFRICA INDONESIA MOSTLY IN PAPUA

EPIDEMIOLOGY
1994 ----> 163 STATES FIVE HIGH RISK GROUPS:

HOMOSEXUAL AND BISEXUAL MAN INTRAVENOUS DRUG ABUSER HEMOPHILIAC RECIPIENT OF BLD AND B.CMPNENT. HETEROSEXUAL CONTACT WITH OTHER HIGH RISK GROUP.

ETIOLOGY

A HUMAN RETROVIRUS FELINE IV, SIMIAN IV, VISNA VIRUS OF SHEEP. LONG INCUBATION PERIODE --> FATAL OUTCOME TROPISM FOR HAEMOTOPOETIC AND NERVOUS SYSTEM ABILITY TO CAUSE IMMUNOSUPRESSION CYTOPHATIC EFFECT IN VITRO

MAJOR IMMUNOLOGIC FEATURES

REDUCED HELPER/SUPRESSOR T RATIOS REDUCED PHERIPHERAL BLOOD LYMPHOCYTE RESPONSE TO MITOGENS AND ANTIGENS ELEVATED IMMUNOGLOBULINS LEVEL REDUCED TO ABSENT ANTIBODY RESPONSE FOLLOWING IMMUNIZATION INCREASED CIRCULATING IMMUNE COMPLEXES REDUCED NK CELL ACTIVITY REDUCED INTERLEUKIN 2 PRODUCTION

HIV VIRION

HIV INFECTION

CLINICAL LATENCY

CLINICAL SYMPTOMS

IMMUNOPATHOGENESIS OF HIV INFECTION

CD4+T CELLS AND MACROPHAGES ARE THE MAYOR TARGET OF HIV INFECTION OF THESE TWO CELLS LEAD A MARKED LOSS OF CD4+TCELL DISSEMINATION OF HIV TO VARIOUS TISSUE ESPECIALLY THE CNS

DECREASED: RESPONSE TO SOLUBLE ANTIGEN LYMPHOKINE SECRETION

HIV

DIMINISHED CYTOTOXIC ABILITY DECREASED CHEMOTAXIS REDUCED IL1 SECRETION POOR ANTIGEN PRESENTATION

MACROPHAGE

DECREASED SPECIFIC CYTOTOXITY

DEPRESED IG PRODUCTION IN RESPONSE TO NEW ANTIGEN

DECREASE KILLING OF TUMOR CELL

TRANSMISSION OF HIV
SEXUAL CONTACT FROM MOTHER TO CHILD THROUGH THE PLACENTA TRANSFUSION OF BLOOD OR BLOOD PRODUCT INJECTION DRUG ABUSER

DEVELOPMENT OF HIV INFECTION

TRANSIENT ILNESS (15%) HIV
FEVER,SWOLLEN LYMPH NODE, RASH AND INFLAMMATION OF MENINGES

85 %
ASYMPTOMATIC

PGL ( PROGRESSIVE GENERALIZED LYMPHADENOPATHIE)

ARC (AIDS-RELATED COMPLEX)

FEVER, NIGHT SWEATS,WEIGHT LOSS , MINOR INFECTION

FULL-BLOWN AIDS MAJOR OPPURTUNISTIC INFECTION MALIGNANCY KAPOSIS SARCOMA

HIV INFECTION
FORMATION OF GIANT CELL IN THE BRAIN

TYPICAL COURSE OF HIV INFECTION

the end

THE EXAMINATION

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