SECTION 1: PHYSIOLOGY OF BLOOD
Chapter 1: Overview of Physiology of Blood..................................................................1
Normal Haematopoiesis 1; Red Blood Cells 6; White Blood Cells 15;
Immune System 27; Megakaryopoiesis 32; Normal Haemostasis 33
SECTION 2: DISORDERS OF RED BLOOD CELLS (ANAEMIAS)
Chapter 2: Approach to Diagnosis of Anaemias......................................................... 52
Approach to Diagnosis 53
Chapter 3: Anaemias Due to Impaired Red Cell Production.......................................71
 Iron Deficiency Anaemia 71
Normal Iron Metabolism 71; Causes of Iron Deficiency Anaemia 75;
Clinical Features 76; Laboratory Features 76; Differential Diagnosis 81;
Treatment of Iron Deficiency Anaemia 81;
 Megaloblastic Anaemias 83
Normal Vitamin B12 Metabolism 83; Normal Folate Metabolism 86;
General Morphological Features of Megaloblastic Anaemia 86;
Causes of Megaloblastic Anaemia 89
 Aplastic Anaemia and Related Disorders 97
Acquired Aplastic Anaemia 98; Constitutional Aplastic Anaemia 106;
Pure Red Cell Aplasia 107
 Anaemia of Chronic Disorders 108
Pathogenesis 109; Clinical Features 110; Laboratory Features 110;
Differential Diagnosis 111; Treatment 111
 Sideroblastic Anaemia 111
Sideroblasts 111; Types and Causes 112; Pathogenesis 112
 Anaemia of Chronic Renal Failure 114
Pathogenesis 114; Clinical and Laboratory Features 115; Treatment 115
 Anaemia of Liver Disease 115
 Myelophthisic Anaemia 116
 Congenital Dyserythropoietic Anaemias (CDA) 116
CDA Type I 117; CDA Type II 117; CDA Type III 118
Chapter 4: Anaemias Due to Excessive Red Cell Destruction .................................121
 Hereditary Spherocytosis 121
Aetiopathogenesis 121; Inheritance 122; Clinical Features 122;
Laboratory Features 123; Diagnosis of Hereditary Spherocytosis 127;
Differential Diagnosis 127; Treatment 128
Contents
Essentials of HAEMATOLOGY
Essentials of Haematology
 Hereditary Disorders of Haemoglobin 128
General Features and Approach to Diagnosis 128; The Thalassaemias 141;
Sickle-Cell Disorders 171;
 Disorders of Red Cell Enzymes 185
Glucose-6-Phosphate Dehydrogenase Deficiency 185
 Immune Haemolytic Anaemias 192
Classification 192
 Haemolytic Disease of the Newborn 202
Rh Haemolytic Disease of the Newborn 203; ABO Haemolytic Disease of Newborn 209
 Paroxysmal Nocturnal Haemoglobinuria 210
Pathogenesis 210; Clinical Features 212; Laboratory Features 212;
Treatment 215; Prognosis 215
 Mechanical Haemolytic Anaemias 216
Microangiopathic Haemolytic Anaemia 216; March Haemoglobinuria 217;
Cardiac Haemolytic Anaemia 217
 Haemolytic Anaemia Due to Direct Action of Physical,
Chemical, or Infectious Agents 217
Physical Agents 217; Chemical Agents 217; Infectious Agents 217
 Hypersplenism 218
Normal Structure and Function of Spleen 218; Causes of Splenomegaly 219;
Diagnostic Criteria 219
SECTION 3: DISORDERS OF WHITE BLOOD CELLS
Chapter 5: Acute Leukaemias......................................................................................224
Diagnosis and Classification 224; Acute Lymphoblastic Leukaemia 241;
Acute Myeloid Leukaemia 252
Chapter 6: Myelodysplastic Syndromes.....................................................................267
Pathogenesis 267; Classification of MDS 268; Clinical Features 269;
Laboratory Features 269; Differential Diagnosis 273; Prognosis 274;
Treatment 274
Chapter 7: Myeloproliferative Neoplasms...................................................................277
Pathogenesis 278; Chronic Myeloid Leukaemia 278; Polycythaemia Vera 289;
Primary Myelofibrosis (PMF) 294; Essential Thrombocythaemia 296
Chapter 8: Chronic Lymphoid Leukaemias ...............................................................299.
Chronic Lymphocytic Leukaemia 299; Prolymphocytic Leukaemia 306;
Hairy Cell Leukaemia 308
Chapter 9: Plasma Cell Dyscrasias ............................................................................312
Investigations in Plasma Cell Dyscrasias 312; Multiple Myeloma 318;
Waldenström’s Macroglobulinaemia 329;
Monoclonal Gammopathy of Undetermined Significance 332
Chapter 10: Malignant Lymphomas.............................................................................335
Hodgkin’s Lymphoma 336; Non-Hodgkin’s Lymphoma 340
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Contents
Chapter 11: Quantitative and Qualitative Disorders of Leucocytes ........................350
 Disorders of Granulocytes 351
Neutrophilia 351; Leucoerythroblastic Reaction 353; Leukaemoid Reaction 353;
Neutropaenia 354; Eosinophilia 356; Basophilia 357;
Disorders of Phagocytic Leucocytes Characterised by Morphologic Changes 357
 Disorders of MonocyteMacrophage
System360
Monocytosis 360; Storage Disorders 361
 Lymphocytosis 364
Infectious Mononucleosis 365
 Immunodeficiency Diseases 369
Classification of Immunodeficiency Diseases 370
Chapter 12: Haematopoietic Stem Cell Transplantation ..........................................375
Types of Haematopoietic Stem Cell Transplantation (HSCT) 375;
Sources of Haematopoietic Stem Cells 380; Recent Advances in HSCT 380
SECTION 4: DISORDERS OF HAEMOSTASIS
Chapter 13: Approach to the Diagnosis of Bleeding Disorders ...............................382
Clinical Evaluation 382; Laboratory Evaluation 385; Laboratory Tests 385;
Specific Tests 391
Chapter 14: Bleeding Disorders Caused by Abnormalities
of Blood Vessels (The Vascular Purpuras)..........................................399
Anaphylactoid Purpura (Henoch-Schönlein Purpura, Allergic Purpura) 400;
Infections 400; Scurvy 400; Senile Purpura 400; Purpura Simplex 401;
Mechanical Purpura 401;
Hereditary Haemorrhagic Telangiectasia (Osler-Weber-Rendu Disease) 401
Chapter 15: Bleeding Disorders Caused by Abnormalities of Platelets .................402
Thrombocytopaenia 402; Thrombocytosis 413; Disorders of Platelet Function 414
Chapter 16: Disorders of Coagulation ........................................................................420
 Inherited Disorders of Coagulation 420
Haemophilia A 420; von Willebrand Disease 432; Haemophilia B 438;
Inherited Disorders of Fibrinogen 439
 Acquired Disorders of Coagulation 440
Vitamin K Deficiency 440; Liver Disease (Cirrhosis of Liver) 441;
Disseminated Intravascular Coagulation 442;
Acquired Inhibitors of Coagulation (Circulating Anticoagulants) 447;
Heparin Therapy 450; Oral Anticoagulants 451;
Other Acquired Coagulation Disorders 453
SECTION 5: BLOOD TRANSFUSION
Chapter 17: Blood Group Systems..............................................................................456
ABO System 457; The Rh System 460
Chapter 18: Serologic and Microbiologic Techniques ..............................................463
Serologic Techniques 463; Microbiologic Techniques 473
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Essentials of Haematology
Chapter 19: Collection of Donor Blood, Processing and Storage ...........................477
Types of Blood Donors 477; Criteria for Selection of Blood Donors 478;
Collection of Donor Blood 482; Processing of Donor Blood 484;
Storage of Donor Blood Unit 484
Chapter 20: Whole Blood, Blood Components and Blood Derivatives....................486
Whole Blood 487; Blood Components 489; Blood Derivatives 495
Chapter 21: Transfusion of Blood to the Recipient ...................................................497
Selection of Donor Blood for Whole Blood or Packed Red Cell Transfusion 498;
Selection of Donor Plasma 499; Antibody Screening and Identification 499;
Compatibility Test 499; Issue of Donor Blood Unit 499;
Transfusion of Blood Unit 500
Chapter 22: Adverse Effects of Transfusion .............................................................502
Immediate Complications 503; Delayed Complications 507;
Complications Associated with Massive Blood Transfusion 512
Chapter 23: Autologous Transfusion..........................................................................513
Predeposit Autologous Blood Transfusion 513;
Acute Normovolaemic Haemodilution 514; Blood Salvage 515
Chapter 24: Alternatives to Blood Transfusion .........................................................516
Haematopoietic Growth Factors (HGFs) 517; Red Cell Substitutes 517
APPENDICES
Appendix A: Reference Ranges ................................................................................................519.
Appendix B: Selected CD Antigens .........................................................................................522
Appendix C: Critical Values in Haematology.........................................................................525
Suggested Reading..........................................................................................................................527.
Index ..............................................................................................................................................531

Essentials of HAEMATOLOGY

Solubility test: Small amount of blood is added to a solution that contains high-phosphate
buffer, a reducing agent (sodium dithionite) and saponin. Red cells are haemolysed
and HbS, if present, is reduced by dithionite. Reduced HbS forms insoluble polymers,
which refract light, and solution becomes turbid. A reader scale is held at the back of
the tube; in negative test lines will be clearly seen since HbA is soluble in phosphate
buffer, while lines will not be seen in positive test due to formation of polymers of
HbS (Fig. 4.10). Positive result is also obtained with HbS Travis, and HbC Harlem. The
solution remains clear in the presence of HbA, HbF, HbC, HbD, HbG, and HbO-Arab.