Page 454 - Essential Haematology

440 / Index

haemoglobin SS (Hb SS) 99 haemophagocytic haemosiderinuria 45
haemolysis lymphohistiocytosis 122, haemostasis
extravascular 76 123 acquired disorders 368
intravascular 76–7 haemophilia A 346, 347, 348–50, hereditary disorders 364–8
haemolytic anaemia 74–87 351, 352 pregnancy 416
acquired 82–7 antenatal diagnosis 349 haemostatic function tests 327–8
acute 77, 80, 81 carrier detection 349, 351 disseminated intravascular
alcohol intoxication 387 clinical features 346, 347, 348, coagulation 357, 358
alloimmune 84 350 haemostatic plug formation 324–5
chemical agents 86 coagulation factor activity 349 haemostatic response 324–5, 326
classifi cation 75 disease severity 349 components 315–16, 317,
clinical features 75–6 factor VIII gene 346, 347 318–19, 320, 321
defective red cell metabolism factor VIII inhibitors 352 platelet reactions 324–5
79–82 freeze-dried factor VIII primary haemostatic plug
drug-induced 86 concentrates 411 formation 324–5
drug-induced immune 84–5 gene therapy 352 vasoconstriction 324
extravascular haemolysis 76 hepatitis C 348 haemostatic support, haematological
hereditary 75, 77–82 HIV infection 346, 348 malignancies 168
immune 82–5 inheritance 346, 350 hairy cell leukaemia (HCL) 241–2
infections 85–6 laboratory fi ndings 348–9 hand–foot syndrome 100, 102
intravascular haemolysis 76–7 molecular genetics 346, 347 Hand–Schüller–Christian disease
laboratory fi ndings 76 prophylaxis 350, 352 122
macrocytic anaemia induction treatment 349–50, 352 haptoglobins 74, 76
72 haemophilia B see factor IX Hashimoto’s diseae 388
March haemoglobinuria 85 defi ciency heart
membrane defects 77–9 haemophilic pseudotumours 346, iron overload in thalassaemia
microangiopathic 85, 86, 382, 348 54
384 Haemophilus infl uenzae type B, risk see also cardiac entries
physical agents 86 in hyposplenism 148 heavy chain diseases 267, 282
red cell fragmentation syndromes haemopoiesis 2–14 Heinz bodies 30, 86
85 extramedullary 2 Helicobacter pylori 41
reticulocytosis 75 spleen 144 autoimmune gastritis 64
secondary 86 ineff ective 215 gastric MALT lymphoma 265
Wilson’s disease 86, 387 regulation 6, 112, 113 pathogenesis 154
see also autoimmune haemolytic site 2 investigations 45
anaemia (AIHA) haemopoietic growth factors 6–8 hemojuvelin (HJV) 37, 38
haemolytic disease of the newborn aplastic anaemia 294 gene mutations 53
418–19, 420–1, 422 myelodysplasia 219 HEMPAS (hereditary erythroblast
blood count 416 haemopoietic malignancies, multinuclearity with a
clinical features 421, 422 infections associated 254, positive acidifi ed serum
fetal assessment 419, 420 255 lysis test) 295
Rh 84, 419, 420–1, 422 haemopoietic progenitor cells 2–3, Henoch–Schönlein syndrome 332,
treatment 422 4 333
haemolytic shock 406–7 haemopoietic stem cells 2–3 heparin
haemolytic transfusion reactions haemorrhagic disease of the administration 373–4
406–7 newborn 354–5 bleeding during therapy 373
haemolytic uraemic syndrome 337, haemorrhagic syndrome 183 disseminated intravascular
338 haemosiderin 34, 35, 76 coagulation 358