Page 2513 - Williams Hematology ( PDFDrive )

2484 Index Index 2485

Protein C pathway, components (Cont.): clinical implications of testing for, 2228t history of, 6, 8
protease activated receptor-1. See epidemiology, 2222t in mycosis fungoides, 1680
Protease (proteinase)-activated etiology and pathogenesis, 1927, 1953, in polycythemia vera, 1293
receptor (PAR)-1 2222–2223 symptomatic therapy for, 1300
protein C. See Protein C venous thromboembolism risk and, 1927, PSC-833, 1404
protein S. See Protein S 1958, 2225t, 2226t PSD95, 1872
thrombomodulin. See Thrombomodulin Protein tyrosine phosphatases (PTPs), 253 P-selectin (GMP140, PADGEM, CD62P),
(TM) Protein Z, 1938, 1952t 1870
in disseminated intravascular coagulation, Protein Z-dependent protease inhibitor activities, 68
2201f, 2202 (ZPI), 1916t, 1938, 1952t, 1960 characteristics, 1858–1859t
in inflammatory response, 1971–1972, Proteoglycans, 62 in coagulation system, 290
2202 Proteomics, platelet, 1853–1854 counterreceptor, 282t
Protein deficiency, anemia of, 654 Proteus infections, 383, 2207. See also in disseminated intravascular coagulation,
Protein inhibitor of activated STATs (PIAS), Bacterial infections 2207
253 Proteus vulgaris, 2340 distribution, 67t
Protein kinase B (Akt), 208f, 209, 251, 1882 Prothrombin (factor II) in eosinophils, 950
Protein kinase C (PKC), 1016, 1178, 1837, activation and activity, 1854, 1918–1919 expression, 281
1841 characteristics, 1917t glycoprotein Ib and, 1868
Protein kinase C-θ deficiency, 2058 conversion to thrombin, 1918–1919, 1919f, hematopoietic stem cell trafficking and,
Proteins 2136–2137 355
adaptor, 251–252 gene structure and variations, 1920, 1920f, in inflammatory response, 1976–1977,
in α-heavy-chain, 1807–1808 2137 1978–1979
α-heavy-chain disease, 1807, 1807f lupus anticoagulant activity and, 2186 ligands, 67t
cross-presentation, 308 structure, 1917f, 1918, 2136 L-selectin (CD62L), 66
in erythrocytes, 469t Prothrombin complex concentrate (PCC), in neutrophils, 1006, 1007f
fibrinolytic, 2304–2305t 396, 2135, 2136, 2136t platelets expressing, 1830–1831f, 1834,
fusion, 1015–1016 Prothrombin (factor II) deficiency, 1852f, 1854
in γ-heavy-chain disease, 1803, 1805f 2135–2137 PSGL-1 and, 1854, 1855–1856, 1870
iron-containing, 631 clinical features, 2137 in sickle cell disease, 765
in iron homeostasis, 621t definition, 2135–2136 von Willebrand factor and, 2166
leukemia-associated, 414 gene mutations, 2135t, 2137 Pseudoaddiction, 767
membrane. See Membrane proteins incidence, 2133, 2134t Pseudodiploid, 175t
in μ-heavy-chain disease, 1809–1810, therapy, 2136t, 2137 Pseudoleukemia, 1393
1810f Prothrombin G20210A Pseudolymphoma syndrome, 1097
in neutrophil granules, 1011–1014 arterial thromboembolic disease risk and, Pseudomonas exotoxin, 345
nonimmunologic adsorption, 825f, 826t, 2226–2227 Pseudomonas infections. See also Bacterial
830t clinical implications of testing for, infections
nonimmunologic protein adsorption, 831 2227–2228, 2228t disseminated intravascular coagulation
phosphorylation, 250–251 epidemiology, 2222t, 2224 and, 2207
prion, 1871 etiology and pathogenesis, 2224 in immunocompromised host, 383
secretory, 1161 venous thromboembolism risk and, 2225, in leukocyte adhesion deficiency, 1023
synthesis 2225t, 2226t, 2268 neutropenia and, 985
genes and, 147 Prothrombin time (PT) neutrophil abnormalities and, 986
in mature neutrophils, 932, 933t in anticoagulant monitoring, 395 in newborn, 105, 106
Protein S, 1949, 1952–1953 in disseminated intravascular coagulation, in specific granule deficiency, 1021
activated protein C and, 1955, 2202 2205t Pseudoneutropenia, 985
anticoagulant activity, 1958 in hemostatic disorders, 1988, 1989f Pseudoneutrophilia (demargination),
cofactor function, 1926 Prothymocytes, 87 999–1000
gene structure and variations, 1926–1927, Protoheme-ferrolyase, 895 Pseudoscleroderma, 907
1928f, 1953 Protoporphyrin IX, 891f, 892f, 893f Pseudothrombocytopenia (spurious
in platelets, 1845 Protoporphyrinogen oxidase (PPO), 895 thrombocytopenia), 43, 1994–1996
structure, 1926, 1927f, 1951f, 1952t, Prourokinase. See Urokinase plasminogen Pseudotumors (blood cysts), 2119, 2120f
1953–1954 activator (u-PA) Pseudovasculogenesis, 2011
Protein S deficiency Proximal vein thrombosis, 2268, 2273 Pseudo- (platelet-type) von Willebrand
acquired causes, 2224t PRPS2, 200 disease, 2050–2051, 2174
clinical features, 2100, 2223 Pruritus/itching Pseudoxanthoma elasticum, 2108

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