Immunology : clinical case studies and disease pathophysiology

419 Pages·2009·81.33 MB·English

by  Coico, Richard, Gottesman, Susan R. S., Strober, Warren

IMMUNOLOGY
Clinical Case Studies
and Disease Pathophysiology

IMMUNOLOGY
Clinical Case Studies
and Disease Pathophysiology
Warren Strober
Laboratory of Host Defenses
National Institute of Allergy and Infectious Diseases/NIH
Susan R.S. Gottesman
Department of Pathology
SUNY Downstate Medical Center
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LibraryofCongressCataloging-in-PublicationData:
Immunology:clinicalcasestudiesanddiseasepathophysiology/[editedby]WarrenStrober,SusanGottesman.
p.;cm.
Companionto:Immunology/RichardCoico,GeoffreySunshine.6thed.c2008.
Includesbibliographicalreferencesandindex.
ISBN978-0-471-32659-5(pbk.)
1. Immunologicdiseases.2. Immunologicdiseases–Casestudies.3. Clinicalimmunology. I.Strober,Warren. II.Gottesman,Susan.
III.Coico,Richard.Immunology.
[DNLM:1. ImmuneSystemDiseases–diagnosis–CaseReports.2. ImmuneSystemDiseases–physiopathology–CaseReports.
3. ImmuneSystemDiseases–therapy–CaseReports. WD300I33152009]
RC582.I4732009
616.97–dc22
2009000198
PrintedintheUnitedStatesofAmerica
10987654321
InmemoryofHyamGottesman.
Dedicatedtoourchildren,David,Lena,andMatthew.
WithspecialthankstoRichardCoicoandthehundredsofmedicalstudents,graduatestudents,andresidentsatSUNY
DownstateMedicalCenterwhohavetaughtmehowtoteach.
SusanR.S.Gottesman
Dedicatedtomywife,HeatherBirnie.
WarrenStrober

CONTENTS
Preface,ix 7
X-LINKEDLYMPHOPROLIFERATIVE
Contributors,xi
SYNDROME,91
IndexofLessonsinNormalImmunity,xiii
8
HEREDITARYANGIOEDEMA,101
INTRODUCTIONTOCASESTUDIES:AGUIDETO
9
USINGTHISBOOK,1 ACQUIREDIMMUNODEFICIENCY
SYNDROME,111
Unit I IMMUNODEFICIENCYDISEASES,3
Unit II IMMEDIATEHYPERSENSITIVITYAND
INTRODUCTION:ORGANIZATIONOFTHE
MASTCELLDISORDERS,125
IMMUNESYSTEMANDEVALUATIONOFTHE
IMMUNODEFICIENTPATIENT,5 INTRODUCTION:WHENANTIGENSBECOME
ALLERGENS,127
1
X-LINKEDAGAMMAGLOBULINEMIA
10
(BRUTON’SAGAMMAGLOBULINEMIA),15 MASTOCYTOSIS,129
2 11
SEVERECOMBINEDIMMUNODEFICIENCY ANAPHYLAXIS,137
DISEASE,27
12
ASTHMA,147
3
DiGEORGESYNDROME(CONGENITAL
THYMICAPLASIA),41 13
THEVASCULITIDES,161
4
HYPER-IgMSYNDROME,53
UnitIII AUTOIMMUNEDISORDERS,173
5
COMMONVARIABLEIMMUNODEFICIENCY INTRODUCTION:OVERVIEWOFAUTOIMMUNE
DISEASE,67 DISORDERSANDMECHANISMSOF
TOLERANCE,175
6
AUTOIMMUNELYMPHOPROLIFERATIVE
14
SYNDROME(ALPS),81 MYASTHENIAGRAVIS,183
vii
viii CONTENTS
15
MULTIPLESCLEROSIS,195
UnitIV MALIGNANCIESOFTHELYMPHOID
SYSTEM,309
16
DIABETESMELLITUSTYPE1,207
INTRODUCTION:CLASSIFICATIONOF
LYMPHOIDMALIGNANCIES,311
17
CELIACDISEASE,219
24
CHRONICLYMPHOCYTIC
18 LEUKEMIA/SMALLLYMPHOCYTICLYMPHOMA,317
INFLAMMATORYBOWELDISEASE,231
25
19 FOLLICULARLYMPHOMA,327
PSORIASIS,245
26
20 PLASMACELLNEOPLASMS,337
IgANEPHROPATHYANDKIDNEY
TRANSPLANTATION,255
UnitV 349
21
RHEUMATOIDARTHRITIS,269
LABORATORYTESTSUSEDINTHEWORKUPOF
22 PATIENTSWITHIMMUNOLOGICDISEASES,351
SYSTEMICLUPUSERYTHEMATOSUS,283
23 Index,369
SCLERODERMA,299