Page 2435 - Williams Hematology ( PDFDrive )
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2406 Index Index 2407
β-globin gene cluster Biliverdin, 499 Bleeding
β-thalassemia mutations unlinked to, 733 Bim, 206, 1672 anticoagulant therapy and, 2277
δβthalassemia-like disorders resulting Binet clinical staging system, 1533t antifibrinolytic therapy for, 2318
from mutations in, 734–735 Bing-Neel syndrome, 1791 in antiphospholipid syndrome, 2240–2241,
dominantly inherited β-thalassemia and, Biogenesis of lysosome-related organelles 2240t
732 complexes (BLOCs), 1843, 2053, APC concentrate and, 2213
regulation, 730 2053f in cardiac surgery, 2085–2086
sickle cell disease and, 762, 763 Biologic false-positive (BFP) syphilis test, categories, 2382–2383, 2383t
structure, 729, 729f 2233 chronic, 553
β-globin synthesis, 730 Biopterin, 585 in chronic myeloproliferative neoplasms,
β-integrins. See under Integrin Biotin method, for red cell life span 2080–2081
β-thalassemias measurement, 496 consultative approach to, 46
β+, 726 Birbeck granules, 310, 1101 dental. See Dental bleeding/extractions
0
β , 726 BIRC3, 231t, 233t, 236t, 1664t disorders. See Hemostasis, disorders
β-globin chain variants and, 733, 749–750 BIR (baculovirus inhibitor of apoptosis in disseminated intravascular coagulation,
clinical features, 743–745 repeat) domains, 207, 298 2203–2204, 2205t
differential diagnosis, 748, 749f Bischloroethylnitrosourea (BCNU). See enhanced fibrinolysis and, 2312
dominantly inherited, 732–733 Carmustine (BCNU) in essential thrombocythemia, 1308–1309
epidemiology/population genetics, Bispecific antibodies, 344 in fibrinolytic therapy, 2317, 2317t
727–728 1,3-Bisphosphoglycerate (1,3-BPG), 692, 695 gingival, 5, 8, 1987
forms, 726 2,3-Bisphosphoglycerate (2,3-BPG) in Glanzmann thrombasthenia, 2046,
gene therapy, 441, 441f in glucose metabolism, 692–693, 695 2046t
hemoglobin A levels in, 750–751 hemoglobin M and, 792 in hemophilia, 2118, 2120, 2121–2122,
2
hemoglobin variants causing, 778t hemoglobin oxygen affinity and, 504, 760, 2122t, 2123
inclusion body, 732 760f, 2377 history, 1985–1986
intermedia, 744–745, 744t, 746f Bisphosphoglycerate mutase iatrogenic, 629
laboratory features, 745–747, 746f (bisphosphoglyceromutase) in immune thrombocytopenia, 2001–2002,
major, 725, 743–744, 744f, 745–746 abnormalities, 701t 2002t, 2003
minor, 636, 745, 746–747, 746f actions, 692t iron deficiency and. See Iron deficiency
molecular basis, 730–733, 731f, 731t, 732f in glucose metabolism, 695 in liver disease and liver transplantation,
pathophysiology. See Thalassemias, Bisphosphoglycerate mutase deficiency, 695, 2192–2193, 2193t
pathophysiology 705, 876 major, 2277, 2382, 2382t
in pregnancy, 126 Bisphosphonates in myeloma, 1740–1741
prevention, 753–754 adverse effects, 1756, 1757 newborn, 111
prognosis, 753 for bone disease in primary myelofibrosis, platelet count and risk of, 2382–2383
silent, 733, 750 1330 platelet transfusion threshold for, 2385. See
treatment, 441, 441f, 751–753, 2372 mechanism of action, 1738 also Platelet transfusion
variant forms, 733, 751 for myeloma, 1738, 1756 in polycythemia vera, 1293
BET (bromodomain and extraterminal)- for osteoporosis in mastocytosis, 977 in pregnancy, 120–122
family, 169 Bivalirudin, 399 retinal, 8
BET (bromodomain and extraterminal) clinical uses, 399 sources of, 628–630, 628t
inhibitors, 170, 338 for heparin-induced thrombocytopenia, in thrombocythemic syndromes, 1286
Bevacizumab 2030, 2031t Bleeding time
adverse effects, 2263 pharmacology, 399 in newborn, 107
for myeloma, 346t for unstable angina, 2296 in von Willebrand disease, 2174
Bexarotene, 1685–1686, 1702 BL. See Burkitt lymphoma (BL) Bleomycin, 331–332. See also ABVD
BFP (biologic false-positive) syphilis test, Blackfan-Diamond anemia, 539–540 regimen; BEACOPP regimen;
2233 Black tree fungus, 2079 Stanford V regimen
BFU-E. See Burst-forming unit-erythroid Blackwater fever, 817. See also Malaria adverse effects, 331–332
(BFU-E) Bladder, primary lymphomas in, 1582 for α-heavy-chain disease, 1808
BH3 domain, 206 Bladder dysfunction, history of, 5 for mycosis fungoides, 1687
Bicytopenia, 1277 Blast crisis, of chronic myelogenous pharmacology, 331
Bid, 204f, 206 leukemia. See Chronic resistance to, 319t
Bilineal (biphenotypic) leukemia, 1386 myelogenous (myeloid) leukemia Blimp-1 (B-lymphocyte-induced maturation
Bilirubin, destruction of hemoglobin and (CML), accelerated phase and blast protein-1), 1171–1172, 1710, 1710f
excretion of, 499–500 crisis Blinatumomab, 1536, 1539
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