Introduction to Medical Immunology - part 10 doc

Combined immunodeficiencies, 591-596
Common acute lymphoblastic leukemia antigen (cALLA), 170
Common acute lymphocytic leukemia antigen (CALLA), 571
Common variable immunodeficiency (acquired hypogammaglobulinemia), 583, 585-586
Compatibility testing, 457
Competitive binding, 274, 275
Complement,
activation, 5, 99, 131-132, 481
alternative pathway, 137, 148-151, 240
classical pathway (
see
Classical complement pathway)
consequences of, 99
exaggerated, pathological situations of, 153-156
in Goodpasture's syndrome, 421
antigen-antibody reactions, 137
cascade, 137
catabolic rates of, 156
components, 137
deficiencies, 157, 248, 596-597
functions, 137-138
levels, in disease, 156-157
metabolism, 156-157
microbial anticomplementary mechanisms, 157-158
synthetic rates of, 156
see also
specific complement components
Complement activators, proteolytic enzymes, 148
Complementarity-determining regions, 84
Complement-based assays, for soluble immune complex screening, 487-488
Complement fixation tests, 270-271

Complement inhibitors, 141, 153
Complement receptor (CR),
1, 146-147, 150-153, 239, 244, 319
2, 152, 153


3, 152, 153, 239
4, 152, 153
in human cell membranes, 151-153
in immune complex development, 484
Complete Freund's adjuvant (CFA), 53, 218
Concanavalin A (ConA), 305
Congenital thymic aplasia (DiGeorge syndrome), 589, 590
Conjugate monoclonal antibodies, 546
Conjugate vaccines, 230-231
Constant region homogeneity, 105
Constant regions, 80-82
Contact hypersensitivity,
experimental, 426-427
in humans, 427
induction, 426
Contact sensitivity, 298-299
Coombs test,
direct, 455, 456, 463, 465, 467
indirect, 269, 456, 457
Copolymer-1 (COP-1; copaxone), 378
Co-receptors,
addition of, 168-169
association with, 164
TcR2, 177

in TcR complex, 175
Cortex,
of lymph node, 16
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[Cortex]
of thymus, 21
Corticosteroids, for systemic lupus erythematosus, 394
Corynebacterium parvum,
511
Co-stimulatory molecules, 66
Counterimmunoelectrophoresis, 260, 261
C1q, 139
C1q receptor, 240
C1r, 139
Crabbés syndrome, 587
CR1 antibodies, 387
C-reactive protein, 240, 241
C1 receptors, 142
Cromolyn, 449
Cross-immunization, 346
Cross-match,
for blood transfusion, 457-458
for bone marrow transplantation, 519
Cross-reactions,
antibody-antigen, 125-126
disease susceptibility and, 354
natural antibodies and, 242
with tissue antigens, 253
Cross-reactive autoantibodies, 386
Cross-reactive idiotypes, 353

Cross-regulation, of T-helper subsets, 212-213
Cryoglobulinemia, essential or idiopathic, 487
Cryoglobulins, 485-487
C1s, 139
CsA (cyclosporine A), 523
CTLA-4, 171
Cutaneous hypersensitivity, 6
Cycle-active agents, 498-499
Cyclooxygenase blockers, 600
Cyclophosphamide (Cytoxan),
clinical applications, 504, 505
inhibition of signal transduction, 500, 523
for systemic lupus erythematosus, 394
Cyclosporine A (Sandimmune; CsA),
clinical applications, 504-505, 505
inhibition of signal transduction, 500-503, 523
Cytochrome C reduction, 326
Cytokines,
assays, 307-309
biological activity, 196-197
in B-lymphocyte activation, 219
chemotactic (chemokines), 247
down-regulatory, 221
fusion, custom-made, 545
genes, 199-200
proinflammatory, 195, 197-199, 428
receptors, 200-201
sources, cellular, 196-197
structure, 199-200
synthesis, down-regulation of, 497-498

targets, 196-197
see also
specific cytokines
Cytokine syndrome, 524
Cytomegalovirus (CMV), 251, 605
Cytopenia, autoimmune, 349, 420
Cytotoxic agents, clinical applications, 504-505
Cytotoxic antibodies, 526
Cytotoxic cells, 4, 5
Cytotoxic hypersensitivity, 418, 420-422
Cytotoxic response, MHC-I and, 40
Cytotoxicity, T-cell-mediated, 13, 207
Cytoxan (
see
Cyclophosphamide)
D
D4, 420
DAF (decay-accelerating factor), 147, 155
DAG (diacylglycerol), 62
D antigen, 454
Decay-accelerating factor (DAF), 147, 155
Defense mechanisms,
model, immune deficiency syndromes as, 247-248
nonspecific, 2, 239-241
specific, 2
against tumors, 539-541
Degranulation, 326
Delayed-type hypersensitivity (DTH),
cell-mediated, systemic consequences of, 428
clinical manifestations, 417, 418

contact hypersensitivity reactions, 427
cutaneous reactions, 6
experimental studies, 425-427
homograft rejection, 428
Jones-Mote reaction, 427-428
prototype, 425
systemic consequences, 428
T helper lymphocytes in, 201-202
transfer of, 425
in vivo testing of, 297-299
Dendritic cells,
CR2 expression and, 152
functions, 65
morphology, 14, 15


Desensitization, 495
Page 630
Diabetes mellitus,
animal models of, 357
DQ molecules and, 44
insulin-resistant, 349
MHC and, 43
pathogenesis, 367-369
Diacylglycerol (DAG), 62
Dialyzable leukocyte extract (DLE), 510
Differential homing, 27
Differentiation genes, 537
DiGeorge syndrome (congenital thymic aplasia), 589, 590
2-Dinitrophenyl (2-DNP), 55-56

Dipeptidylpeptidase IV (CD26), 180, 299
Diphtheria toxoids, 287-288
Direct antibody assay, 277
Direct antiglobulin test (Coombs), 456, 463, 465, 467
Direct hemagglutination, 455
Discrimination, self vs. nonself, 6-7
Disodium cromoglycate, 449
Disulfide bonds, reduction, 76
Disulfide-interchanging enzyme, 93
DJ complex, 106
DLE (dialyzable leukocyte extract), 510
DNA antibodies, 272
DNA-histone complex antibodies, 386
DNA polymorphisms, 113
DNA vaccines, 232
DNP, hapten-carrier effect in irradiated animals, 56-58
Donor-recipient matching, for organ transplantation, 517-519
Double diffusion method (Ouchterlony's technique), 259-260
Double positives, 175
Down-regulation, of immune response, 345-346
DP alleles, 37
DQ alleles, 37
DQ locus, 44
DQw5, 42
DR alleles, 37
DR1
β
chains, in rheumatoid arthritis, 406-407
Drug abuse, immunosuppression of, 603
Drug-induced disorders,

hemolytic anemia, 467-470
systemic lupus-like syndrome, 392
thrombocytopenia, 469
Drug-protein conjugates, 52
Drugs (
see
specific drugs)
DTH (
see
Delayed-type hypersensitivity)
Dysgammaglobulinemia, 283, 284
E
E4, 420
EAE (experimental allergic encephalomyelitis), 356-357, 377
EBV [
see
Epstein-Barr virus (EBV)]
ECF-A (eosinophil chemotactic factors of anaphylaxis), 444
Eczema, with immunodeficiency and thrombocytopenia, 595-596
Effector cells,
cytotoxic, 309
in immune response, 2, 3
lymphocytes as, 5-6
mechanisms,
in antibody-dependent anti-infectious, 243-244
in experimental contact hypersensitivity, 426-427
EIA (
see
Enzymoimmunoassay)
ELAM-1 (endothelial-leukocyte adhesion molecule-1; E-selectin), 26, 197

Elastase, 321
Electrophoresis, in B-cell dyscrasia diagnosis, 554


Electrostatic bonds, 121
EM allotypes (IgE heavy-chain allotypes), 113
Endarteritis, 401
Endothelial-leukocyte adhesion molecule-1 (ELAM-1; E-selectin), 26, 197
Enhancement, immunological, 541
Enhancing antibodies, 609
Env, 605
Envelope glycoproteins, 606
Environment,
autoimmune disease pathogenesis and, 354-356
control, in immediate hypersensitivity prevention, 446
systemic lupus erythematosus pathogenesis and, 392
Enzyme-linked immunoassay test (ELISA), for anti-HIV antibodies, 612-613
Enzymoimmunoassay (EIA),
advantages, 279-280
competitive, 277
cytokine, 307
limitations, 279-280
rapid diagnosis, 278-279
Eosinophil chemotactic factors of anaphylaxis (ECF-A), 444
Page 631
Eosinophilic cationic protein, 446
Eosinophils,
in cytotoxic reactions, 244
functions, 15-16
in immediate hypersensitivity, 445-446

Epinephrine, 448
Epithelial cells, synthesis of secretory component, 93
Epitope (
see
Antigenic determinants)
Epstein-Barr nuclear antigen-1, 574
Epstein-Barr virus (EBV),
B-cell lymphoma associated with, 574
immunosuppressive effects, 251
post-transplant lymphoproliferative disease, 527-528
Equilibration phase, 225, 226
Equilibrium constant, 122
Equilibrium dialysis, 122-123
Equivalence, 127-128
Equivalence zone, 127
Erythroblastosis fetalis (hemolytic disease of newborn), 461-464
Erythropoietin, 614
E-selectin (endothelial-leukocyte adhesion molecule), 26, 197
Exotoxins, bacterial, 252
Experimental allergic encephalomyelitis (EAE), 356-357, 377
Experimental contact hypersensitivity, 426-427
Experimental models, of serum sickness, 475
F
Fab fragment, 76, 77, 78
F(ab')
6
fragment, 78
Fab region, 83
Fabricius, bursa of, 11
Factor B, 149, 150

Factor D, 150
Factor H, 150, 153
Factor I,
C3 activation, 143
C3b cleavage, 146, 150-151
C3b inhibition, 153
cofactors, 150
deficiency, 596-597
Fc fragment, 76, 77, 78
Fc receptors,
antibody-dependent cell-mediated cytotoxicity and, 244
binding, 99-101
Fc
ε
, 100-101
Fc
δδ
, 319
binding, 99-101
in immune complex development, 484
Fc
ε
-RI, 441-442
Fc
ε
-RII, 442
IgG-specific, 95-96
mediation of cell activation, 131
Fd fragment, 77
Felty's syndrome, 402, 411

α
-Fetoprotein (AFP), 538
Fetus, 163
Fisher-Race theory, 454-455
FKBP, 503, 504
FK506 (tacrolimus), 503-504, 523
Flavin adenine dinucleotide (FAD), 322
Flow cytometry, of T-lymphocyte subpopulations, 299-302
Fluorescence assays, 326
Folic acid deficiency, 599
Follicles, lymphoid (nodules), 16, 17, 18
c-Fos, 191-192


Fractional turnover rate, of immunoglobulins, 95
Frameshift mutations, 110
G
Gag, 605
GALT (
see
Gut-associated lymphoid tissue)
Gamma globulin,
classification, 75
intravenous,
for humoral immunodeficiencies, 585, 586, 588
for idiopathic thrombocytopenic purpura, 379-380
Gastrointestinal tract, autoimmune diseases of, 371-373
G-CSF (granulocyte colony stimulating factor), 509, 614
Gell and Coombs classification of hypersensitivity, 417-418
Gene conversion, 111

Gene therapy,
for cancer, 544
for chronic granulomatous disease, 331
Genetic factors,
in autoimmune disease, 353-354
in diabetes mellitus, 369-371
graft rejection and, 31
in IgE synthesis control, 439
immunogenic responsiveness and, 52
in malignant transformation, 536
in rheumatoid arthritis, 406-407, 409
Page 632
[Genetic factors]
in study of lymphocyte diversity/ontogeny, 166-167
in systemic lupus erythematosus, 390-391
Genomes, microbial, 251
Germ-cell teratocarcinoma, 538
Germinal center reaction, 222-223
Germinal centers, 18
Giardia lamblia
, 250
Glomerulonephritis,
acute proliferative, 355
in systemic lupus erythematosus, 389
Glucocorticoid-responsive elements, 496
Glucocorticoids,
anti-inflammatory effects, 498
clinical applications, 504, 505
for graft rejection prevention, 522-523
for idiopathic thrombocytopenia purpura, 379

for immediate hypersensitivity, 449
for multiple sclerosis, 378
for myasthenia gravis, 376
for rheumatoid arthritis, 410-411
β
-Glucuronidase, 321
Glutathione, 322
G1M3, 112
G1M17, 112
GM allotype (IgG heavy-chain allotypes), 111-113, 115
GM-CSF (
see
Granulocyte-monocyte colony stimulating factor)
GM genes, 116
Goiter, diffuse toxic or exophthalmic (Graves disease), 353, 363-365
Goodpasture's syndrome, 364, 420-421
gp28 (CD40 ligand), 181, 205
gp39, 171
gp41, 606
gp120, 231, 606, 611
gp160, 231, 606
Graft rejection,
antigens (
see
Human leukocyte antigens)
cell-mediated immunity and, 6
genetic control of, 31
mediation, 203
memory, 31, 32
in organ transplantation, 519-522

acute, 520-521
case example, 522
delayed or chronic, 521-522
hyperacute or early, 520
specificity, 31, 32
Graft-versus-host reaction (GVH), 528-529, 592
Granulocyte colony stimulating factor (G-CSF), 509, 614
Granulocyte-monocyte colony stimulating factor (GM-CSF),
biological activity, 196
clinical applications, 509-510
hematopoietic effects, 199
macrophage activation and, 247, 409
sources, cellular, 196
targets, 196
Granulocytes,
functions, 14-16
in immune complex diseases, 483
in immune complex-induced hypersensitivity, 423-424
protective effects, 2
Granulomatous reactions, T-cell hypersensitivity and, 202-203
Granzymes,
A, 209


apoptosis and, 13
B, 209
Graves disease, 353, 363-365
Guillain-Barré syndrome, 355
Gut-associated lymphoid tissue (GALT),
antigenic stimulation, 227, 228

distribution, 16
Peyer's patches, 22, 23, 24
tonsils, 22, 23
GVH reaction (graft-versus-host reaction), 528-529, 592
H
Hageman factor, 154
Hairy cell leukemia, 509, 545, 570
Haplotypes, 38, 39, 112-113
Hapten-carrier effect, 55-58
Hapten-carrier conjugates, 52
Haptens, 49, 52
Hashimoto's thyroiditis (autoimmune thyroiditis), 356, 364, 365-366
Hassall's corpuscles, 22, 23
HAT (hypoxanthine-rich medium), 165, 166
Hay fever, 419
HBsAg (hepatitis B surface antigen), 231
Heart transplantation, 519
Heavy-chain diseases, 555, 566-567
Heavy chains, 76, 77
allelic exclusion, 114
chromosome 14, 105, 106, 107
class switching, 108
Page 633
[Heavy chains]
constant region, 82
domains/regions, 83
variable region or V region, 82, 105
Heavy-chain V-region allotypes (HV1), 113
Hemagglutination, 129, 130
direct, 268-269

indirect, 269
inhibition, 270
passive, 269-270
Hematopoiesis, interleukins and, 199
Hematopoietic system, 163
Hemochromatosis, 45
Hemodialysis, pulmonary vascular leukostasis and, 155-156
Hemoglobinemia, 420
Hemoglobinuria, 420
Hemolysis,
from blood transfusion reaction, 458-460
extravascular, 420
intravascular, 420
Hemolytic anemia,
autoimmune,
case example of, 470
diagnosis, 269
mechanisms, 420
target tissue, 364
warm antibody type, 465-466, 467
case example, 465, 470
cold agglutinin disease/syndromes, 466-467, 560
cold-induced, 560
drug-induced, 467-470
Hemolytic disease of newborn (erythroblastosis fetalis), 461-464
Hemopoiesis, 372
Hemopoietin-receptor family, 200
Heparin, 155, 444
Hepatitis,
chronic active, 253, 373-375

viral, 373
Hepatitis B surface antigen (HBsAg), 231
Hepatitis B vaccine, 231
Hepatitis B virus, 250
Hepatocellular carcinoma, 538
Hepatocytes, IgA transport, 93
Hereditary angioneurotic edema, 153-154
Heroin immunosuppression, 603
Herpes virus infection,
immunotherapy, 233
spread, 251
Heterotypic interactions, 171
HEV (high endothelium venules), 25, 27
HGPRT (hypoxanthine guanine phosphoribosyl transferase), 165
HiB-OC, 230
High endothelium venules (HEV), 25, 27
Hinge region, 83
Histamine, 420, 443-444
Histocompatibility antigen (
see
Human leukocyte antigen)
HIV (
see
Human immunodeficiency virus)
Hives, 419
HIV vaccines, 231, 510, 615-616
HLA (
see
Human leukocyte antigen)
H

2
O
2
, 322
Hodgkin's disease, 574
Homocytotropic antibody, 87, 418
Homograft rejection, 428
Hormones,
immunosuppressive, 496-498
in rheumatoid arthritis, 409-410
synthesis, 22
in systemic lupus erythematosus, 392
thymic, 511
HTLV-1-associated T-cell leukemia,
case example, 568-569, 574-575
pathogenesis, 572-573
Human aggregate-free globulin (HAGG), 339
Human immunodeficiency virus (HIV),
antigenic variation, 251
enzymoimmunoassay test, 278
immunoprophylaxis, 614-616
immunosuppressive effects, 252
infection,
active immunization for, 510
asymptomatic stage of, 609-610
decline of immune functions in, 610-612
early stages of, 608-609
escape from immune response, 612
immunotherapy, 233
of phagocytic cells, 249

replication, 606
seroconversion, 608
serological diagnosis, 612-613
structural components, 605-607
therapy, 613-614
transmission modes, 607-608
vaccines, 231, 510, 615-616
Human leukocyte antigens (HLAs),
alleles, disease susceptibility and, 354
B27, 38, 45, 353, 355


biological role, 5, 33
Page 634
[Human leukocyte antigens (HLAs)]
Bw35, 45
Bw53, 42
cell-mediated immunity and, 6
cellular distribution, 37
class, 33-34
I, 34, 36, 171
II, 14, 36, 171
DR, 179, 355
DR2, 45
DR4, 355, 406-407
genes, 116
class II, 44
in humans, 38
linkage disequilibrium, 45
graft rejection and, 31

haplotypes, 38, 39, 45
I, 176, 178, 241
identification, 35-37
II, 19, 59, 176, 178, 213
expression, in insulin-dependent diabetes, 370
major, 32-33
matching, in organ transplantation, 518-519
minor, 33
in rheumatoid arthritis, 406-407
serologically defined, 36
specificities, 36-37
transplantation and, 33
w designation, 37
Humoral immune response,
defined, 4
description, 243
down-regulation, 221-222, 223
effectiveness, 244-246
helper T lymphocytes and, 203-207
in HIV infection, 612
induction, 217-219
investigations, additional, 289-292
mucosal, 226-229
Humoral immunodeficiencies,
antigen-selective, 588
common variable or acquired hypogammaglobulinemia, 583, 585-586
defined, 581-582
hyper IgM syndrome, 587-588
of immunoglobulin A, 583, 586-587
infantile agammaglobulinemia, 582-585

transient hypogammaglobulinemia of infancy, 583, 585
HVI (heavy-chain V-region allotypes), 113
Hybridomas, 164-166, 299
Hydantoin, induction of systemic lupus-like syndrome, 392
Hydralazine, induction of systemic lupus-like syndrome, 392
Hydrogen bonding, 121
Hydrophobic interactions, 121
21 Hydroxylase deficiency, 45
Hyper-IgE syndrome (Job's syndrome), 328-329
Hyper-IgM syndrome,
clinical presentation, 285, 583, 588
genetics/physiopathology, 207, 587-588
therapy, 588
Hyperlipidemia, 560
Hypermutations (
see
Somatic mutations)
Hypersensitivity diseases, 9
Hypersensitivity reactions,
allergy and, 417


anti-IgA antibody, prevention of, 587
classification, 417-418
cytotoxic or type II, 420-422
hapten-carrier effect and, 52
IgE-mediated (
see
Immediate hypersensitivity)
immediate or type I, 418-420

immune complex-induced or type III, 422-425
immunosuppressive drugs in, 504-505
type IV (
see
Delayed-type hypersensitivity)
Hypersensitivity states, 53
Hypervariable regions, 34, 84
Hyperviscosity syndrome, 557, 558, 559
Hypoadrenalism, chronic primary (Addison's disease), 43, 364, 366-367
Hypocomplementemia, immune complex clearance and, 157
Hypogammaglobulinemia,
acquired, 583, 585-586
in chronic lymphocytic leukemia, 569
immunoglobulin levels in, 283-284
Hyposensitization, 446-447
Hypoxanthine guanine phosphoribosyl transferase (HGPRT), 165
Hypoxanthine-rich medium (HAT), 165, 166
I
I-A alleles, 37
Ia antigens (I region-associated antigens), 14
I antigen, 466, 560
ICA (anti-islet cell antibodies), 367
Page 635
ICAM (
see
Intercellular adhesion molecule)
ICE (interleukin converging enzyme), 195
IDDM (
see
Insulin-dependent diabetes mellitus)

IDDM1 locus, 369
IDDM2 locus, 369
Idiopathic thrombocytopenic purpura (ITP), 358, 364, 378-380
Idiotypes, 111
I-E alleles, 37
IEP (immunoelectrophoresis), 260-261
Ig (
see
Immunoglobulin)
Ig
α
(CD79a), 65, 168
Ig
β
(CD79b), 65, 168
IgA (
see
Immunoglobulin A)
IgD (immunoglobulin D), 79, 86, 96
IgE (
see
Immunoglobulin E)
IgG (
see
Immunoglobulin G)
IgM (
see
Immunoglobulin M)
IgM-IgG switch, 220, 223
IkB (inhibitor kB), 191

IL-2 receptors,
expression, 193-194, 304
as markers, 179, 180
serum levels, 308-309
ILs (
see
Interleukins)
Immediate hypersensitivity,
atopy and, 420
case example, 434-435, 449-450
characteristics, 417, 418, 433
clinical manifestations, 419, 433-435
defined, 5
drug therapy, 447-449
eosinophils in, 445-446
experimental models, 418-419
historical background, 418-419
pathogenesis, 419-420
IgE antibodies and, 436-438
sequence of events in, 435-436
prevention, 446-447
Immune complexes,
absorption, 478
in autoimmune diseases, 350
circulating, 477
deposition of, 479-481
fate of, 422
clearance, hypocomplementemia and, 157
deposition in tissues, 424-425
diseases of,

cells/soluble factors in, 483
classification of, 489-490
clinical expression of, 490-491
development, host factors and, 484
physiopathology, 475-483
screening tests, 483
treatment, 491
formation, 475
in interstitial fluids, 477
in situ, 477-478
in Goodpasture's syndrome, 421
in HIV infection, 611-612
induction of hypersensitivity reactions, 422-425
physicochemical characteristics, 477
screening tests, 489
in situ formation of, 422
soluble,


detection of, 483, 484-489
in drug-induced hemolytic anemia, 467-469
formation of, 424
precipitation of, 487
in systemic lupus erythematosus, 387-389
tissue deposition of, 478-481
transfer of, 478
triggering of inflammatory circuits, 481-483
uptake, infection as consequence of, 253
Immune deficiency syndromes, as models for immune defense study, 247-248
Immune elimination, 225, 226

Immune recognition, 58
Immune response,
abnormal,
consequences of, 252-253
in systemic lupus erythematosus, 391-392
anti-idiotypic, 346
avoidance, 248-252
characteristics, 2
down-regulation of, 345-346
immunoglobulin allotypes and, 115
induction,
antigen processing/presentation and, 59-60
antigen receptors and, 58-59
B lymphocytes and, 65
cytotoxic T-lymphocytes and, 62-65
factors associated with, 52-53
helper T lymphocyte activation and, 60-62
immune recognition and, 58
ineffective, 250