Page 2479 - Williams Hematology ( PDFDrive )

2450 Index Index 2451

Immune thrombocytopenia (ITP), Nijmegen breakage syndrome, 530t, immune dysregulation,
primary (Cont.): 1227, 1575t polyendocrinopathy, enteropathy,
genetic factors, 2001 rare, 1227 X-linked–like syndromes,
history, 1999–2000 clinical features, 1213t, 1214–1215t 1223–1224
incidence, 2001 combined immunodeficiencies. See immune dysregulation,
laboratory features, 2002–2003 Combined immunodeficiencies polyendocrinopathy, enteropathy,
pathophysiology, 2000–2001, 2084–2085 (CID) X-linked syndrome, 1181, 1215t,
during pregnancy, 121–122, 2010–2011 cytotoxicity disorders, 1227–1229 1223
therapy Chédiak-Higashi syndrome. See Schimke syndrome, 1226
for acute bleeding, 2003, 2085, 2385 Chédiak-Higashi syndrome with selective susceptibility to pathogens,
adjunctive, 2007 familial hemophagocytic 1230–1231
anti-(Rh)D, 2005 lymphohistiocytosis, 1228, 1849, impaired signaling through toll-like
azathioprine, 2006 2061 receptors, 1230
cyclophosphamide, 2007 Griscelli syndrome, 994, 1020, 1229, Mendelian susceptibility to
cyclosporine, 2007 1849 mycobacterial disease, 1231
danazol, 2007 Hermansky-Pudlak syndrome. See severe combined immunodeficiency.
dapsone, 2007 Hermansky-Pudlak syndrome See Severe combined
glucocorticoids, 2003–2004 (HPS) immunodeficiency (SCID)
initial management, 2003 X-linked lymphoproliferative disease, WASp-interacting protein deficiency,
intravenous immunoglobulin, 1228–1229, 1266, 1575t 1225
2004–2005 in defective thymic development WHIM syndrome, 994, 1226
rituximab, 2005 CHARGE syndrome, 1222 Wiskott-Aldrich syndrome. See Wiskott-
splenectomy, 2004 congenital alopecia and absence of Aldrich syndrome (WAS)
thrombopoietic receptor agonists, thymus, 1223 Immunogenicity, 2336
1825, 2005–2006 DiGeorge syndrome, 86–87, 1222 Immunoglobulin (Ig)
vinca alkaloids, 2007 genetically determined deficiencies of allotypes, 1170
types, 1999t complement system, 1232 heavy-chain, 1170
secondary hyperimmunoglobulin E syndromes, light-chain, 1170–1171
in antiphospholipid syndrome, 2008 1020t, 1026–1027, 1182, 1215t, in B-cell development, 1151
in bacterial infections, 1856 1225 B-cell disorders and defects in, 1195, 1501
in chronic lymphocytic leukemia, laboratory features, 1214–1215t genetics, 1163–1169
1541–1542 lymphocytopenia in, 1204 gene complexes, 1163–1164, 1164f,
etiology, 2007–2008, 2007f lymphomas and, 1574, 1575t 1165f, 1166f
in hepatitis C infection, 2009 neutropenia in, 993 gene rearrangement, 1166–1168, 1167f
in HIV infection, 1252–1254, 1253t, predominant antibody deficiencies, heavy-chain class switching, 1168–1169
1999 1212–1216 mechanisms for generating antibody
in H. pylori infection, 2009–2010 clinical features, 1213t, 1214t diversity, 1169
incidence, 2007f common variable immunodeficiency surrogate λ light chains, 1168
in systemic lupus erythematosus, and selective IgA deficiency, 993, idiotypes, 1160
2008–2009 1214t, 1216 IgA. See Immunoglobulin (Ig) A
in viral infections, 1513, 2009 hyperimmunoglobulin M syndromes. IgD. See Immunoglobulin (Ig) D
Immunization See Hyperimmunoglobulin M IgE. See Immunoglobulin (Ig) E
antibodies generated in response to, syndromes IgG. See Immunoglobulin (Ig) G
2347–2348 laboratory features, 1214t IgM. See Immunoglobulin (Ig) M
complications, 7 lipopolysaccharide responsive beige-like for immune-related fibrosis, 1330
Immunoadsorption apheresis, 429t, 430t anchor deficiency, 1216 oligoclonal, 1725–1726
Immunobead assay, 2000 X-linked and autosomal recessive structure and function, 1159–1163
Immunodeficiency diseases, 1211–1232 agammaglobulinemia, 993, biologic properties, 1160t
cartilage hair hypoplasia, 993, 1226 1212–1213, 1214t heavy chains, 1161t, 1162f, 1163
chromosomal instability syndromes primary, presenting as autoimmune light chains, 1159
associated with, 1226–1227 diseases, 1223–1224 physical properties, 1159, 1160f, 1160t
ataxia-telangiectasia. autoimmune lymphoproliferative surface immunoglobulin, 1163
See Ataxia-telangiectasia syndrome, 210, 1224, 1575t synthesis and secretion, 1171–1172
ataxia-telangiectasia-like disorder, autoimmune polyendocrinopathy, therapy. See Intravenous immunoglobulin
1227 candidiasis, and ectodermal (IVIg)
Bloom syndrome, 1227, 1507, 1575t dystrophy syndrome, 87, 1181, 1224 variable-region structure, 1170

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