Page 2470 - Williams Hematology ( PDFDrive )

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2440 Index Index 2441

Hematopoietic lineages, morphologic plasticity, 54–55, 269 disorders of, 778–782. See also specific
differentiation of, 33–34 preleukemic, 1376 types and Sickle cell disease
Hematopoietic microenvironment, 263–267 proliferation, apoptosis, and maturation, in erythropoiesis, 479–480
aging and, 267 74–75 fetal, synthesis of, 101, 101t, 102f, 728, 730
anatomy, 263 release, 71–74 genetic control, 728–730, 729f
cell adhesion in, 65–68, 67t surface phenotype, 260 high-oxygen affinity, 782, 872, 876
cell proliferation, apoptosis, and trafficking and homing, 68–71, 355 intravascular destruction of red cells and,
maturation in, 74–75 transcription factor profile, 262–263 499, 500f
cellular homing in, 68–71 viral transgenesis, 453–454 low-oxygen affinity, 782, 794–795, 794t
cellular release in, 71–74, 72f, 73f Hematopoietic stem cell transplantation measurement, 13–14
cytokines, 264–266, 264t (HSCT). See Hematopoietic cell neonatal, 102–103
general considerations, 53–54 transplantation (HCT) nitric oxide and, 796–797, 796f, 797f
histogenesis of, 55 Hematopoietic tissue, 55 polymerization of, 763–764, 765f
matrix proteins, 266–267 Hematuria, 5, 766, 1987, 2119, 2374 quaternary structures, 761t, 763–764
in myeloma, 1712 Heme red cell viscosity and, 668
in neutrophilic granulopoiesis, 939 biosynthesis, 891f, 892–896, 893f reference ranges, 18t
stromal cells, 264 defects in synthesis, 916 structure and function, 759–761, 760f,
Hematopoietic niche, 56 intravascular destruction of red cells and, 761f, 761t
Hematopoietic progenitors, 258f, 269–272 5, 500f thresholds for red cell transfusion,
assays, 269 structure, 761, 761f, 790–791, 791f, 892, 2369–2370, 2370t. See also Red cell
B cells, 1151, 1168 892f transfusion
in chronic myelogenous leukemia, 1440, Heme iron, 619 unstable, 781–782
1447 Heme oxygenase (HO), 499 Hemoglobin A, 481, 728, 751, 762
dendritic cell, 310, 1076f, 1081 Heme pocket, substitutions near, 781 Hemoglobin A , 728, 750–751
2
in erythropoiesis, 480–481 Heme synthetase (ferrochelatase), 893f, 895, Hemoglobin AS (sickle cell trait), 762, 766
lymphoid, 269–270 917 Hemoglobin Bart’s, 726
macrophage, 1076, 1076f, 1081 Hemin Hemoglobin Bart’s hydrops fetalis syndrome
myeloid, 270–272 for ALA dehydratase porphyria, 900 α-thalassemia and, 739
in primary myelofibrosis, 1326 intravenous, for acute intermittent clinical features, 745
Hematopoietic stem cells (HSCs), 259–263 porphyria, 904 laboratory features, 747
adhesion, 65–66 structure, 892, 892f pathophysiology, 740
aging and, 132–133, 263 Hemochromatosis, 639–645 Hemoglobin C, 762
assays, 259–260 classification, 640, 640t Hemoglobin C disease
cell-cycle characteristics, 261 clinical features, 643 clinical features, 779
circulation, 55–56, 56f course and prognosis, 644–645 definition and history, 778
derivation from induced pluripotent stem definition and history, 639–640 differential diagnosis, 780
cells, 449 differential diagnosis, 643 etiology and pathogenesis, 778–779
differentiation and maturation, 1282, 1282f epidemiology, 640 laboratory features, 779, 779f
early development, 54, 54f etiology and pathogenesis, 640–643 therapy, 780
expansion, self-renewal, and animal models, 642–643 Hemoglobin Constant Spring, 748, 748f
differentiation, 268–269 genetics, 642 Hemoglobin C thalassemia, 749–750
fetal, 100f iron overload, 641 Hemoglobin D, 762
functional definition, 259 iron toxicity, 640–641 Hemoglobin D disease, 779f, 781
gene expression profile, 261–262 pathology, 641–642 Hemoglobin D Ibadan , 762, 781
in gene therapy, 437 hereditary, 429t, 432, 640, 640t, 643 Hemoglobin D Los Angeles , 762, 781
for hematopoietic cell transplantation. See juvenile, 640, 643 Hemoglobin D Punjab , 762, 781
Hematopoietic cell transplantation, laboratory features, 643 Hemoglobin E, 762
sources of stem cells for neonatal, 640 Hemoglobin E disease
integrins, 260–261 secondary, 640, 643 clinical features, 780
kinetics, 259 treatment, 644 course and prognosis, 781
metabolic characteristics, 196–197, 197f, Hemochrome, 892, 892f definition and history, 780
261 Hemochromogen, 892 etiology and pathogenesis, 780
microenvironment, 53–54. See also Hemoglobin (Hb), 617 laboratory features, 779f, 780
Hematopoietic microenvironment abnormal, 762, 776–778, 778t therapy, 780
ontogeny of, 101 concentration in pregnancy, 119 Hemoglobin E thalassemia, 749, 779f
organization, 64–65 deoxygenation of, 796–797, 796f, 797f Hemoglobin E-β -thalassemia, 780
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