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Haematology for Students and Practitioners (Including Practical Haematology)
Ramnik Sood
1:
History and Scope
2:
Blood Formation
SITES OF BLOOD FORMATION
Abnormal Sites
Examples
DEVELOPMENT OF BLOOD CELLS
In an Adult
ERYTHROPOIESIS
Erythropoiesis
Erythron Concept
Erythron-Proliferation and Maturation
Erythroid Precursors
STAGES OF NORMOBLASTIC DIFFERENTIATION
Pronormoblast
Early Normoblast (Basophilic Normoblast or Basophilic Erythroblast)
Intermediate Normoblast (Polychromatophilic Normoblast or Erythroblast)
Late Normoblast (Orthochromatic Normoblast or Erythroblast)
Reticulocyte
Action of EPO
Substances Needed for Erythropoiesis
Ineffective Erythropoiesis
LEUCOPOIESIS (Chart 2.1)
Myelopoiesis
Myeloid Series
Myeloid Series
Myeloblast
Promyelocytes (Neutrophilic)
Myelocyte (Neutrophilic)
Metamyelocyte (Neutrophilic)
Band Neutrophils
Polymorphonuclear Neutrophils
Eosinophils
Basophils and Mast Cells
Lymphocytic Series (Fig. 2.8)
Further Embryological Subdivision
Activated and Reactive Lymphocytes
Plasmocytes
Plasmoblasts and Proplasmocytes
MONOCYTIC SERIES (Fig. 2.8)
Macrophages
Lymphokines and Monokines
Interleukin-1 (IL-1)
Interleukin-2 (IL-2)
Interleukin-4 (IL-4)
Interleukin-6
Interleukin-7 and 9–13
Interferons
Lymphocytes
T Cells
B Cells
Natural Killer (NK) Cells
Lymphocyte Proliferation and Differentiation (Fig. 2.9)
Lymphocyte Circulation
Platelet Series (Fig. 2.10)
3:
Red Blood Cells
HISTORICAL ASPECTS
STRUCTURE
Shape
Dimensions
PHYSIOLOGY OF THE RED BLOOD CELLS
Red Cell Membrane (Fig. 3.1)
RED CELL MEMBRANE LIPIDS
Phospholipids
Glycolipids and Cholesterol
RBC Membrane Proteins (Fig. 3.2)
Integral Membrane Proteins
Peripheral Proteins
Deformability
Permeability
Haemoglobin Structure and Function
Haemoglobin Synthesis
Iron Delivery and Supply
Synthesis of Protoporphyrins
Globin Synthesis
HAEMOGLOBIN FUNCTION
ACTIVE RED CELL METABOLIC PATHWAYS (MAINTENANCE OF HAEMOGLOBIN FUNCTION)
RBC Senescence/Aging
Nutritional and Other Requirements of a Red Cell
NORMAL RED CELL VALUES
RBC Count:
Diurnal and Day-to-Day Variation
Red Cell Absolute Values
The Mean Cell Volume (MCV)
The Mean Cell Haemoglobin (MCH)
The Mean Cell Haemoglobin Concentration (MCHC)
ANAEMIA
Definition
Pathophysiology
Compensatory Physiological Adjustments
Cardiovascular Adjustments
Clinical Features
Symptoms and Sings
Cardiorespiratory System
Skin
Neuromuscular System
Retinopathy
Gastrointestinal System
Genitourinary System
Other Signs
History and Physical Examination
CLASSIFICATION
Classification of Anaemia Based on Pathogenesis and Aetiology
I. Blood Loss
II. Impaired Red Cell Formation
III. Increased Red Cell Destruction (Haemolytic anaemia)
Morphological Classification
Based Mainly on Two Features
Three Main Types of Anaemias are Recognised
Kinetic Classification
Kinetic Classification of Anaemia
4:
Basic Principles in Diagnosing and Treating Anaemias
INVESTIGATIONS
Is the Patient Anaemic?
What is the Type of Anaemia?
Cause of Anaemia
Other Investigations which should be Done Routinely (Besides Hb and peripheral smear examination)
EXAMINATION OF BLOOD FILM
Method
Always Note
RBCs (Table 4.2)
WBCs
Platelets
TREATMENT
Treatment Includes
Treatment of Disorder Causing Anaemia
Administration of Haematinics
GENERAL CONSIDERATIONS
5:
Iron Deficiency Hypochromic Anaemia
RELEVANT NORMAL VALUES
Haemoglobin Synthesis in the Developing Red Cell
Iron Metabolism
Absorption of Iron
Mechanism and Control of Iron Absorption (Fig. 5.1)
Iron Balance
Pathogenesis of Iron Deficiency Anaemia
Iron Deficiency: Stages of Development
Prevalence
Impaired Absorption
Blood Loss
Gastrointestinal Bleeding
Menstruation
Haemoglobinuria
Hemostasis Disorders
Chronic Renal Failure Treated with Hemodialysis
Nosocomial Blood Loss
Infancy
Decreased Total Body Iron at Birth
Foetomaternal Transfusion
Growth
Diet in Infancy and Childhood
Blood Loss in Infancy
Pregnancy and Lactation
IRON DEFICIENCY ANAEMIA
Aetiology
Blood Loss
Other Causes of Chronic Blood Loss
Increased Requirements
Impaired Absorption
Inadequate Intake
Maldistribution
Clinical Features
CLINICAL PRESENTATION
Growth
Fatigue
Neuromuscular Symptoms
Epithelial Changes
Immunity and Infection
Pica
Genitourinary System
Skeletal System
Spleen
Peripheral Blood Picture
Peripheral Smear
Bone Marrow
Serum Biochemistry
Treatment
Response to Treatment
Failure of Response to Oral Iron
Complicating Factors
Management
Oral Iron Therapy
Parenteral Iron Therapy
Side Effects
Response to Treatment
Preventive Therapy
PLUMMER-VINSON SYNDROME (PATERSON KELLY SYNDROME)
INVESTIGATIONS OF A PATIENT WITH IRON DEFICIENCY ANAEMIA
History
Menstruating Females
Male and Post-Menopausal Women
Infants and Children
Physical Examination
Special Investigations
A. Investigations Needed Commonly
B. Investigations Needed Occasionally
IRON OVERLOAD
Haemosiderosis
Haemochromatosis
SIDEROBLASTIC ANAEMIAS
Classification of the Sideroblastic Anaemia
Hereditary sideroblastic anaemias
Acquired sideroblastic anaemia
Reversible-associated with
Types of Sideroblasts
Hereditary Sideroblastic Anaemia (Pseudothalassaemia)
Acquired Primary Idiopathic Sideroblastic Anaemia (Fig. 5.5)
Secondary Sideroblastic Anaemia
Treatment
No Iron Therapy
RADIOACTIVE 59Fe STUDIES
Bone Marrow Iron
Criteria for Grading Iron Stains in Bone Marrow Aspirated
6:
Megaloblastic, Macrocytic Anaemias
RELEVANT NORMAL VALUES
VITAMIN B12 KINETICS
Transport
TCI (Transcobalamin I)
TC II
TC III
UBBC
Tissue Stores
Excretion
Function
FOLATE KINETICS
Function
VITAMIN B12 DEFICIENCY
Aetiology and Pathogenesis
Genetic Factors
Gastritis
Immunologic Mechanism
Clinical Manifestations
Mode of Onset and Initial Symptoms
General Appearance
Fever
Gastrointestinal System
Cardiovascular and Genitourinary Systems
Nervous System
Erythrocyte Kinetics, Iron and Bilirubin Metabolism
Other Biochemical Findings
Course, Prognosis, and Complications
Other Conditions Associated with Vitamin B12 Deficiency
Dietary Deficiency of Vitamin B12
Gastric Surgery
Intestinal Disorders
Small Bowel Bacterial Overgrowth
Diseases Affecting the Ileum
Zollinger-Ellison Syndrome
Fish Tapeworm Disease
Tropical Sprue and Coeliac Disease
Drug Induced Vitamin B12 Malabsorption
Familial Selective Vitamin B12 Malabsorption (Imerslund Syndrome, Imerslund-Gräsbeck Syndrome)
Functionally Abnormal Intrinsic Factor
Chronic Pancreatic Disease
Haemodialysis
HIV Infection
Aetiology (Fig. 6.1)
Clinical Manifestations
Special Tests for Diagnosing Vitamin B12 Deficiency
II. Radioactive Vitamin B12 Absorption Test
Schilling Test
Variant of Schilling Test
Management
Response to Therapy
Treatment (Basic Principles)
FOLATE DEFICIENCY
Causes of Folate Deficiency
Dietary Deficiency
Alcoholism and Cirrhosis
Pregnancy
Infants and Children
Rapid Cellular Proliferation
Congenital Folate Malabsorption
Drug Induced Folate Deficiency
Tropical Sprue and Gluten-Sensitive Enteropathy
Causes of Folate Deficiency (Fig. 6.2)
Clinical Manifestations
Special Tests for Diagnosing Folate Deficiency
Management
PERIPHERAL BLOOD FINDINGS IN VITAMIN B12 OR FOLIC ACID DEFICIENCY (Figs. 6.3 to 6.5)
BONE MARROW (Fig. 6.4)
Dyserythropoiesis
WBC Series
Megakaryocytes
Mechanism of Anaemia in Megaloblastic Anaemia
MEGALOBLASTIC MACROCYTIC ANAEMIA
Biochemical Basis of Megaloblastic Anaemia
Vitamin B12 Deficiency Impairs Folate Availability
dU Suppression Test
DIAGNOSIS OF PERNICIOUS ANAEMIA
Signs and symptoms of Pernicious Anaemia
Diagnosis
Other Investigations Needed in Folate Deficiency
Megaloblastic Anaemia Unresponsive to Therapy
Inherited And Drug-Induced Megaloblastic Anaemias
INHERITED DISORDERS ALTERING DNA SYNTHESIS
Homocystinuria and Methylmalonyl Aciduria
Abnormal Vitamin B12 Transport
Orotic Aciduria
Thiamine Responsive Megaloblastic Anaemia
Other Possible Disorders
DRUG INDUCED DISORDERS OF DNA SYNTHESIS
Folate Antagonists
Nitrous Oxide
Other Drugs and Toxins
CAUSES OF MACROCYTOSIS OTHER THAN MEGALOBLASTIC ANAEMIA
7:
Symptomatic Anaemias
ACUTE HAEMORRHAGIC ANAEMIA
Blood Picture
Haematologic Findings
Clinical Aspects
Clinical Description
Pathophysiology
Treatment
Management
ANAEMIA OF INFECTION
Aetiology
Clinical Aspects
Blood Picture
Pathogenesis
Biochemical Changes
Treatment
TUBERCULOSIS
CHRONIC RENAL FAILURE
Aetiology
Mechanisms Involved in Anaemia of Renal Disease
Blood Picture (Fig. 7.1)
Other Laboratory Findings
Pathogenesis
Diagnosis
Treatment
Erythropoietin Resistance
MALIGNANCY
Mechanisms of Anaemia in Malignancy
Type of Anaemia
ANAEMIA ASSOCIATED WITH SECONDARY CARCINOMA OF THE BONE MARROW
Blood Picture
Bone Marrow
Clinical Aspects
Diagnosis
Prognosis
LIVER DISEASE
Mechanisms Involved in Anaemia of Liver Disease
Erythrokinetics and Pathogenesis
Treatment
COLLAGEN DISEASE
Rheumatoid Arthritis
Systemic Lupus Erythematosus (SLE)
LE Cell Phenomenon
ENDOCRINE DISORDERS
Anaemia Associated with Endocrine Disorders
Myxoedema
Hyperthyroidism
Hypogonadism
Hypopituitarism
Hyperparathyroidism
Addison's Disease
PROTEIN MALNUTRITION
SCURVY
PHYSIOLOGICAL ANAEMIA OF PREGNANCY
Pathogenesis
NUTRITIONAL ANAEMIA OF PREGNANCY
ANAEMIA OF CHRONIC DISORDERS
Conditions Associated with Anaemia of Chronic Disorders
Clinical and Laboratory Observations
ANAEMIA: DEVELOPMENT AND SEVERITY
Morphologic Features
Iron Metabolism
Other Biochemial Findings
Kinetic Characteristics
Pathogenesis
Cytokines
Shortened Red Cell Survival
Impaired Marrow Response
Abnormal Iron Metabolism
Anaemia in Patients with Cancer
Diagnosis
Treatment
NORMOCYTIC AND NORMOCHROMIC ANAEMIAS (CHART 7.1)
Aetiological Classification of Normocytic Anaemia
Summary of the Investigations of a Patient with Normocytic Anaemia
History
Examination
Blood Examination
Special Investiagations
8:
Haemolytic Anaemias
DEFINITION
CLASSIFICATION
Intracorpuscular
Hereditary or Congenital
Enzyme Defects
Extracorpuscular
Acquired
Aetiopathogenetic Calssification of Haemolytic Disorders
I Inherited Haemolytic Disease
II. Acquired Haemolytic Anaemias
Clinical Manifestations
Chronic Congenital Haemolytic Anaemia
Degree of Anaemia
Jaundice
Crises
Splenomegaly
Cholelithiasis
Leg Ulcers
Skeletal Abnormalities
ACQUIRED HAEMOLYTIC ANAEMIA
LABORATORY MANIFESTATIONS
Laboratory Sings of Accelerated Red Cell Destruction
Signs of Excessive Red Cell Destruction
Erythrocyte Survival
Catabolism of Haem
Rate of Haem Catabolism
Lactate Dehydrogenase (LDH)
Disappearance of Haptoglobin
Glycosylated Haemoglobin (Hb A1)
Signs of Intravascular Haemolysis
Haemoglobinemia
Haemoglobinuria
Urine Iron Excretion
Methemalbumin and Hemopexin
Signs of Accelerated Erythropoiesis
Laboratory Signs of Accelerated Erythropoiesis
Blood
Bone Marrow
Ferrokinetic
Biochemical
Reticulocytosis
Chemical Markers of Cell Age
Other Morphologic Findings in the Blood
Bone Marrow
Ferrokinetic Studies
Laboratory Tests–Findings Useful in Differential Diagnosis Specific Morphologic Abnormalities (Table 8.2)
Coombs Test (Antiglobulin Test)
Osmotic Fragility Test
Autohemolysis
Tests for Hemolytic Disorders Associated with Heinz Body Formation
APPROACH TO DIAGNOSIS
Conditions that can Sometimes be Mistaken for Hemolytic Anemia
Specific Cause Determination
TREATMENT
HEREDITARY SPHEROCYTOSIS AND OTHER ANEMIAS DUE TO ABNORMALITIES OF THE ERYTHROCYTE MEMBRANE
Red Cell Membrane-General Considerations
Hereditary Spherocytosis (HS)
Pathogenesis (Diagram 8.1)
Erythrocyte Abnormalities
Role of the Spleen in the Pathophysiology
Clinical Features
Laboratory Findings
Diagnosis
Treatment
HEREDITARY ELLIPTOCYTOSIS (HE) SYNDROMES (Table 8.3)
HE Variants-Prevalence
Pathogenesis of Common HE Disorders
Membrane Protein Defects
Spectrin Abnormalities
Protein 4.1 Defects
Glycophorin C Deficiency
Elliptocyte Formation Due to Membrane Abnormalities
Clinical and Laboratory Findings of HE Syndromes
Common HE (Fig. 8.3)
Homozygous (Doubly Heterozygous) HE
HEREDITARY PYROPOIKILOCYTOSIS (HPP)
Spherocytic Elliptocytosis (SE)
Southeast Asian Ovalocytosis (SAO)
Laboratory Findings
Treatment
STOMATOCYTIC DISORDERS
Hereditary Stomatocytosis
Hereditary Xerocytosis
Intermediate Stomatocytic Syndromes
Rh Null Disease
ACANTHOCYTIC DISORDERS
Abetalipoproteinemia
MacLeod Phenotype
Echinocytic Disorders
Target Cell Disorders
Familial Lecithin-Cholesterol Acyltransferase (LCAT) Deficiency
GLUCOSE-6 PHOSPHATE DEHYDROGENASE DEFICIENCY AND RELATED DISORDERS OF HEXOSE MONOPHOSPHATE SHUNT AND GLUTATHIONE METABOLISM
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY
Genetics
Prevalence and Geographic Distribution
G6PD Enzyme and its Variants
Pathophysiology
Hematologic and Clinical Features
Acute Hemolytic Anemia
Drug Induced Hemolysis
Infection Induced Hemolysis
Hemolysis Induced by Diabetic Acidosis
Congenital Nonspherocytic Hemolytic Anemia
Neonatal Hyperbilirubinemia
Favism
Diagnosis
Treatment
Screening
RELATED DISORDERS OF HMP SHUNT AND GLUTATHIONE METABOLISM
6-Phosphogluconate Dehydrogenase Deficiency
Glutathione Reductase Deficiency
Glutathione Peroxidase Deficiency
Defects in Glutathione Synthesis
γ-glutamyl-cysteine Sysnthetase (γ-GCSynth) Deficiency
Glutathione Synthetase Deficiency
Glutathione S-Transferase Deficiency
HEREDITARY HEMOLYTIC ANEMIAS ASSOCIATED WITH ABNORMALITIES OF ERYTHROCYTE GLYCOLYSIS AND NUCLEOTIDE METABOLISM
General Consideration (Table 8.4)
PYRUVATE KINASE DEFICIENCY
Biochemical Genetics
Pathophysiology
Clinical Features
Diagnosis
Treatment
ABNORMALITIES OF PURINE AND PYRIMIDINE NUCLEOTIDE METABOLISM (Diagram 8.4)
Pyrimidine 5' Nucleotidase Deficiency
Adenosine Deaminase Excess
Adenylate Kinase Deficiency
AUTOIMMUNE HAEMOLYTIC ANAEMIA (AIHA)
WARM ANTIBODY AIHA
Clinical Picture
Haematological Picture
Immunology
Antibodies on the Red Cell Surface
Antibodies in the Serum
Red Cell Destruction-Site and Mechanism
Extravascular Destruction (Figure 8.5)
Intravascular Destruction
Diagnosis
Course and Prognosis
Therapeutics
COLD ANTIBODY AIHA
Cold Haemagglutinin Disease (CHAD)
Clinical Features
Blood Picture
Immunology
Site and Mechanism of Red Cell Destruction
Prognosis and Treatment
Paroxysmal Cold Haemoglobinuria (PCH)
Aetiology and Pathogenesis
Clinical
Diagnosis
Paroxysmal Nocturnal Haemoglobinuria (PNH)
Aetiology and Pathogenesis
Clinical
Blood Picture
Bone Marrow
Diagnosis
Treatment
HAEMOLYTIC ANAEMIA DUE TO DRUGS AND CHEMICALS
Haemolytic Anaemia due to Direct Toxic Action
Drug Therapy
Occupational Poisoning
Household Poisoning
Contamination of Haemodialysis Systems
HAEMATOLOGICAL PICTURE
Drug-induced Haemolytic Anaemia due to Unstable Haemoglobins
Immunological Drug-induced Haemolytic Anaemia
DRUG-INDUCED IMMUNE HAEMOLYTIC ANAEMIA
Drug Adsorption Mechanism
Immune Complex Mechanism
DRUG-INDUCED AUTO-IMMUNE HAEMOLYTIC ANAEMIA (Tables 8.8 and 8.9)
MECHANICAL HAEMOLYTIC ANAEMIA
Aetiology
Cardiac Haemolytic Anaemia
Micro-angiopathic Haemolytic Anaemia
The Haemolytic Uraemic Syndrome
Clinical Picture
Haematological Picture
Therapeutics and Prognosis
Thrombotic Thrombocytopenic Purpura and Disseminated Intravascular Coagulation
Disseminated Carcinoma
Malignant Hypertension and Eclampsia
March (Exertional) Haemoglobinuria
Haemolytic Anaemia Associated with Bacterial Infections and Parasitic Infestations
Clostridium Welchii Infection
Malaria
Lead Poisoning
Burns
Summary of Clinical Investigations
Physical Examination
9:
Disorders of Haemoglobin Structure and Synthesis
SICKLE HAEMOGLOBINOPATHIES
Laboratory Diagnosis
SICKLE CELL TRAIT (AS)
SICKLE CELL ANAEMIA
Clinical
Sickle Cell Crises
1. Vaso-occlusive Crises
Hand and Foot Syndrome
Bone and Joint Crises
Abdominal Crises
CNS Crises
Pulmonary Crises
2. Haematologic Crises
Aplastic Crises
Splenic Sequestration Crises
Haemolytic Crises (Hyperhaemolytic Crises)
Megaloblastic Crises
3. Infectious Crises
Blood Picture
Prognosis
Treatment
COMBINATION OF HbS WITH OTHER HAEMOGLOBINOPATHIES
Sickle Cell β Thalassaemia
Sickle Cell HbC Disease
Sickle Cell HbD Disease
OTHER HAEMOGLOBINOPATHIES
Haemoglobin C Haemoglobinopathies
Haemoglobin E Haemoglobinopathies
Haemoglobin-D Haemoglobinopathies
Unstable Haemoglobin Haemoglobinopathies
LABORATORY DIAGNOSIS
Demonstration of Heinz Bodies
Heat Instability Test
Isopropanol Precipitation Test
Haemoglobin Electrophoresis
Clinical and Laboratory Findings
Haemoglobinopathies Associated with Polycythaemia
LABORATORY DIAGNOSIS
THALASSAEMIAS
Classification
Laboratory Diagnosis
Routine Haematological Tests
Demonstration of HbH Inclusions
Haemoglobin Electrophoresis
Globin Chain Synthesis Rate Studies
Classification of Thalassaemias
Clinical
In β Thalassaemia
In α Thalassaemia
β THALASSAEMIAS
β THALASSAEMIA MINOR (TRAIT)
β THALASSAEMIA MAJOR
Clinical
Skeletal Changes
Growth and Sexual Development
Cardiopulmonary Complications
Hepatobiliary Disease
Blood Picture (Fig. 9.5)
Bone Marrow
Hb Pattern
Laboratory Diagnosis
Pathophysiology of Anaemia
Anaemia occurs because of
Prognosis
Therapeutics
Prevention
Measurement of Globin Chain Synthesis
Analysis of Fetal DNA
Delta Beta Thalassaemia
The Haemoglobin-Lepore Syndromes
Hereditary Persistence of Fetal Haemoglobin (HPFH)
α THALASSAEMIA
Classification (Table 9.6)
Alpha Thalassaemia Trait
Haemoglobin H Disease
Haemoglobin-Bart's Hydrops Fetalis
Alpha Thalassaemia and Other Haemoglobinopathies
Methaemoglobinaemia
Toxic Methaemoglobinaemia
Clinical Features
Diagnosis
Therapeutics
Hereditary Methaemoglobinaemia
Sulphaemoglobinaemia
10:
Aplastic Anaemia, Pancytopenia
PANCYTOPENIA
PANCYTOPENIA
Aetiology
Diagnosis of Pancytopenia
Investigation of Cases with Pancytopenia
Blood Examination
Bone Marrow Aapiration and Trephine
APLASTIC ANAEMIA
Classification
Aetiology of Aplastic Anaemia
Drug Idiosyncrasy
Chemical Exposure
Aplastic Anaemia and Infectious Hepatitis
Fanconi's Anaemia
Clinical
Signs and Symptoms
Blood Picture
Bone Marrow may be
Diagnosis
Course and Prognosis
Prevention
Management
Identification and Elimination of Exposure to Causative Agent
Supportive Therapy
Prevention and Treatment of Infection
Prevention and Treatment of Haemorrhage
Red Cell Infusion for Anaemia
Measures to Accelerative Recovery from Pancytopenia
Bone Marrow Transplantation
Antithymocyte Globulin Administration
Anabolic Agent Administration
Corticosteroid Administration
Pure Red Cell Aplasia
Congenital or Diamond-Blackfan Anaemia
Acquired Pure Red Cell Aplasia
Differential Diagnosis of Pancytopenia
Myelodysplastic Disorders
Bone Marrow Infiltration or Replacement
Multiple Myeloma
Systemic Lupus Erythematosus
Paroxysmal Nocturnal Haemoglobinuria
11:
White Blood Cells
PHYSIOLOGY OF WHITE BLOOD CELLS
Sites of Production
Production and Life-span of White Blood Cells
Metabolic and Enzymatic Characteristics of White Blood Cells
Function of White Blood Cells
Leucocyte Surface Antigens
Normal White Cell Values
Pathological Variations in White Cell Counts
Neutrophilia
Conditions Causing Cell Necrosis or Destruction
Eosinophilia
Lymphocytosis
Monocytosis
Basophilia
Basophilopenia
Eosinopenia
Lymphopenia
Bone Marrow Plasmacytosis
Causes of Leucoerythroblastic Change
Neutrophil Alkaline Phosphatase
NEUTROPENIA AND AGRANULOCYTOSIS
Neutopenia and the Attendent Risk of Infection
Cause of Neutropenia
Causes of Neutropenia
Clinical
DRUG INDUCED NEUTROPENIA
Mechanisms of Neutropenia Induction
Clinical Features
Blood Picture
Bone Marrow
Diagnosis
Treatment
CHRONIC IDIOPATHIC NEUTROPENIA (AGRANULOCYTOSIS)
Clinical
Blood Picture
Diagnosis
Differential Diagnosis
Treatment
INFECTIOUS MONONUCLEOSIS
Aetiology
Clinically
Blood Picture
Paul-Bunnell Test for Heterophile Antibody
Differential Diagnosis
A. Disorders of Similar Clinical Onset
B. Disorders with a Similar Blood Picture
DISORDERS OF PHAGOCYTIC LEUCOCYTES CHARACTERISED BY MORPHOLOGIC CHANGES
These Include
Pelger-Huet Anomaly
Pseudo or Acquired Pelger-Huet Anomaly
Alder Reilly Anomaly
May-Hegglin Anomaly
Chediak-Steinbrinck-Higashi Anomaly
Mucopolysaccharidosis VI and VII
Other Causes of Lymphocyte Vacuolation
12:
Leukaemias
FAB CLASSIFICATION OF MYELODYSPLASTIC SYNDROMES
THE ACUTE LEUKAEMIAS
Incidence
Clinical Features
Laboratory Findings
Routine Haematologic Investigations and Fig. 12.2)
Differentiation of ALL and AML
Special Investigations
Differential Diagnosis
DISORDERS WITH SIMILAR CLINICAL FEATURES
Disorders with a Similar Blood Picture
Course and Prognosis
Treatment
General Considerations
Specific Therapeutic Agents
Therapy in the Individual Patient
CHILDHOOD ACUTE LEUKAEMIA
Acute Lymphoblastic Leukaemia
Maintenance Therapy
Acute Myeloid Leukaemia
ADULT ACUTE LEUKAEMIA
Acute Lymphoblastic Leukaemia
Remission Induction
Maintenance Therapy
Acute Myeloid Leukaemia
Induction of Remission
Maintenance Therapy
Supportive Care
Specific Aspects of Supportive Care
Myelodysplastic Disorders
Clinical Features
Blood Picture
Bone Marrow
THE CHRONIC LEUKAEMIAS
Chronic Granulocytic Leukaemia (CML/CGL)
Clinical Features
Blood Picture
Bone Marrow
Course of Disorder
Chloroma
Treatment of Chronic Phase Disease
Chemotherapy
Side-effects
Other Forms of Treatment
Treatment of Blast Crisis
CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)
Classification of the Chronic Lymphoid Leukaemias
B Cell
T Cell
Clinical Features
Blood Picture (Fig. 12.5)
Bone Marrow
Diagnosis
Course and Prognosis
Clinical Staging of CLL (Table 12.11)
Clinical Staging System Adopted by International Workshop on CLL
Treatment
Radiotherapy
Chemotherapy
Corticosteroids
Choice of Therapy
Symptomatic and Supportive Therapy
Other Chronic Lymphoproliferative Disorders
Hairy Cell Leukaemia
Prolymphocytic Leukaemia
LEUKAEMOID REACTIONS
Leukaemoid Blood Picture
The Diagnostic Problem
Myeloid Leukaemoid Reactions
CAUSES OF MYELOID LEUKAEMOID REACTIONS
Leuco-erythroblastic Blood Picture
Causes of Lymphatic Leukaemoid Reactions
Myelokathexis
13:
Paraproteinemias
MULTIPLE MYELOMA
Diagram Showing Clinical Features and Complications in Multiple Myeloma
Blood Picture
Bone Marrow (Fig. 13.2)
Blood Chemistry
Urine
Bone X-ray
Diagnosis
Criteria for the Diagnosis of Multiple Myeloma
Other Laboratory Findings
Emergency Situations
Myeloma Staging System
Subclassification
Example
Management
Symptomatic Measures
Chemotherapy
Radiotherapy
Response and Prognosis
MACROGLOBULINEMIA
Classification of Macroglobulinemia
Waldenstrom's Macroglobulinemia
Clinical Features
Blood Picture
Bone Marrow
Lymph Nodes
Blood Chemistry
Treatment
HEAVY CHAIN DISEASES (HCD)
BENIGN MONOCLONAL GAMMOPATHY
AMYLOIDOSIS
MALIGNANT LYMPHOMA AND CHRONIC LYMPHOCYTIC LEUKAEMIA
MISCELLANEOUS DISORDERS
PRINCIPLES OF THE DIFFERENTIAL DIAGNOSIS OF MONOCLONAL GAMMOPATHIES
Causes of Hyperviscosity Syndrome
14:
Lymphoid Tissue Tumours (Malignant Lymphomas)
CLASSIFICATION OF LYMPHOMAS HODGKIN'S DISEASE
CLASSIFICATION OF NON-HODGKIN'S LYMPHOMAS
Kiel (Lennert)
Lukes-Collins
Rappaport (Modified)
National Cancer Institute Working Formulation
Clinical Manifestations in Hodgkin's Disease
Common
Less Common
Occasional
NON-HODGKIN'S LYMPHOMA (NHL)
Diagnosis
Diagnosis when Superficial Nodes are not Enlarged
Staging
Stage
Staging Laparotomy
Factors Adversely Influencing Prognosis in Non-Hodgkin's Lymphomas
Lymphadenopathy Simulating Malignant Lymphoma
Follicular (nodular) Pattern
Sinus Pattern
Diffuse Pattern
Mixed Patterns
Pre-treatment Clinical Evaluation of a Patient with Malignant Lymphoma
History
Examination
Special Investigations
Further Investigations Required in Some Cases
TREATMENT
General Considerations in Treatment
Advice to the Patient and Relatives
Therapy–its Objects
Radiation Therapy
Response to Radiation Therapy
Chemotherapy
Basic Principles for Use of Chemotherapeutic Agents
Agents Used in Treatment of Lymphomas
Nitrogen Mustard
Cyclophosphamide
Chlorambucil
Melphalan
Nitrosoureas
VINCA ALKALOIDS
Combination Chemotherapy for Hodgkin's Disease
Chemotherapy for Non-Hodgkin's Lymphoma
Outcome in Malignant Lymphoma
15:
Myeloproliferative Disorders
POLYCYTHAEMIA
Various Causes of Polycythaemia
Secondary
POLYCYTHAEMIA VERA
Pathophysiology
Clinical Presentation
General Physical Examination and X-ray
Peripheral Blood Picture
Bone Marrow Examination
Establishing Diagnosis
Polycythaemia Study Group Criteria for Diagnosis of Polycythaemia Vera
Category A (Major Criteria)
Category B (Minor Criteria)
Differential Diagnosis (Table 15.2)
Course and Prognosis
Treatment
Plan of Therapy in the Individual Patient
Choice of Marrow Depressive Agent
RADIOACTIVE PHOSPHORUS
Chemotherapy
Venesection
Symptomatic Treatment
FAMILIAL POLYCYTHAEMIA (FAMILIAL ERYTHROCYTOSIS)
RELATIVE POLYCYTHAEMIA
Pseudopolycythaemia
THROMBOCYTHAEMIA
Causes of Raised Platelet Count
Reactive
Endogenous
Essential Thrombocythaemla
Laboratory Findings, Treatment and Course
MYELOFIBROSIS (MYELOSCLEROSIS)
Primary Myelofibrosis
Clinical Picture
Blood Picture
Bone Marrow
Diagnosis
Differential Diagnosis
Course and Prognosis
Treatment
Acute Myelofibrosis
Causes of Marrow Fibrosis
16:
Platelets, Blood Coagulation and Haemostasis
HAEMORRHAGIC DISORDERS DUE TO CAPILLARY DEFECTS: NON-THROMBOCYTOPENIC PURPURA
ACQUIRED HAEMORRHAGIC VASCULAR DISORDERS
Simple Easy Bruising (Purpura Simplex)
Senile Purpura (Involutional Purpura)
THE SYMPTOMATIC VASCULAR (NON-THROMBOCYTOPENIC) PURPURAS
Infections
Drugs
Uraemia
Cushing's Disease and Corticosteroid Administration
Scurvy
Dysproteinaemia
THE HENOCH-SCHONLEIN SYNDROME (ANAPHYLACTOID PURPURA)
Clinical Picture
Blood Picture
Course and Prognosis
Treatment
MISCELLANEOUS DISORDERS
Orthostatic Purpura
Mechanical Purpura
Fat Embolism
Auto-erythrocyte Sensitization
Systemic Disorders
Congenital Haemorrhagic Vascular Disorders
Hereditary Haemorrhagic Telangiectasia
Clinical Picture
Peripheral Smear
Diagnosis
Course and Prognosis
Localised Treatment
General Treatment
Ehlers-Danlos Disease
HAEMORRHAGIC DISORDERS DUE TO PLATELET ABNORMALITIES
Platelet Production
Normal Values
Thrombocytopenia
General Considerations
Idiopathic Thrombocytopenic Purpura
Clinical Picture
Type and Site of Bleeding
Course of the Bleeding
Blood Picture
Bone Marrow
Diagnosis
EXCLUSION OF OTHER CAUSES (TABLE 16.4)
Pathogenesis
Treatment
Corticosteroids
Splenectomy
Supportive Treatment
Immunosuppressive Treatment
Immunoglobulin Infusion
Androgens
Pregnancy
Idiopathic Cyclical Thrombocytopenia Purpura
SECONDARY THROMBOCYTOPENIA
Aetiology
More Common Causes
Less Common Causes
Unusual Causes
Treatment
Thrombocytopenia Due to Drugs and Chemicals
Clinical Picture
Thrombotic Thrombocytopenic Purpura
Neonatal and Inherited Thrombocytopenias
Immune Thrombocytopenia
Drug Ingestion by the Mother
Infection
Megakaryocytic Hypoplasia
Inherited Thrombocytopenias
PLATELET TRANSFUSION
Clinical Effect
Iso-(allo)immunization and Reactions
Indications
FUNCTIONAL PLATELET DISORDERS
Special Tests
CLASSIFICATION OF PLATELET DYSFUNCTION
Congenital Qualitative Platelet Defects
Thrombasthenia (Glanzmann's Disease)
Bernard-Soulier Syndrome
Defects of Platelet Enzymes
Granule Defects
Acquired Platelet Dysfunction
Thrombocytosis and Thrombocythaemia
Idiopathic (Haemorrhagic) Thrombocythaemia
Clinical Features
17:
Coagulation Disorders
PLASMA PROTEIN COAGULATION FACTORS
Calcium
Platelet and Lipid (Platelet Factor 3) and Membrane Binding Sites for Coagulation Factors
Foreign Surfaces
Tissue Factor (Tissue Thromboplastin)
Theory of Blood Coagulation
Reaction Sequence
Inhibitors of Coagulation
Plasma Fibrinolytic System
PLASMINOGEN-PLASMIN SYSTEM
Activators
Fibrinolysis
Pathogenesis of Coagulation Abnormalities
Deficiency of One or More Blood Coagulation Factors
Inhibition of Coagulation by Acquired Inhibitors
Fibrinolysis
Miscellaneous
DIAGNOSIS OF COAGULATION DISORDERS
Clinical Assessment
Laboratory Investigations (Table 17.3)
SCREENING TESTS
One-stage Prothrombin Time (Quick's Method)
Activated Partial Thromboplastin Time
Thrombin Clotting Time
Screening Tests for Detection Increased Fibrinolysis
SPECIAL TESTS
General Principles in the Treatment of Coagulation Disorders
Treatment of the Underlying Causative Disorder
Correction of the Coagulation Abnormality with Drugs and Transfusion Replacement Therapy
Replacement Therapy
Local Treatment at the Bleeding Site
Wounds and Mucous Membrane Bleeding
HAEMATOMAS AND HAEMARTHROSIS
General Supportive Measures and Care
CLINICAL DISORDERS DUE TO COAGULATION ABNORMALITIES
CONGENITAL COAGULATION DISORDERS
Haemophilia A and Haemophilia B
Clinical Features
Severity of the Bleeding Tendency
Variability of the Bleeding Tendency
Type and Site of Bleeding
Complications of Haemorrhage
Diagnosis
Course and Prognosis
Management
Basic Aspects
Treatment of Bleeding
General Supportive Measures
Local Haemostatic Measures
Replacement Therapy
SURGERY
Special Therapeutic Measures
Von Willebrand's Disease
Other Congenital Disorders
Congenital Fibrinogen (Factor I) Deficiency
Congenital Deficiency of Factor II (Prothrombin), Factor V, Factor VII, or Factor X
Factor XI Deficiency
Factor XII (Hageman Factor) Deficiency
Factor XIII (Fibrin-Stabilizing Factor) Deficiency
ACQUIRED COAGULATION DISORDERS
VITAMIN K DEFICIENCY
Aetiology of Vitamin K Deficiency
Disorders that Impair Fat Absorption
Sterilization of the Bowel by Antibiotic Drugs
Haemorrhagic Disease of the Newborn
TREATMENT
LIVER DISEASE
Pathogenic Factors in Bleeding
Bleeding in Hepatitis
Treatment
General Principles
Established Bleeding
ANTICOAGULANT DRUGS
Monitoring of Anticoagulant Therapy
Bleeding During Anticoagulant Therapy
Treatment
DISSEMINATED INTRAVASCULAR COAGULATION
Pathogenesis
Aetiology
Clinical Features
Type of Bleeding
Pregnancy
Surgery
Laboratory Diagnosis
Treatment
HAEMORRHAGE AND BLOOD TRANSFUSION
Treatment
Haemorrhagic Disorders due to Circulating Inhibitors of Coagulation
Outline of Investigations of a Patient with a Bleeding Tendency
Is the Bleeding Due to Local Pathological Lesion, a Haemorrhagic Disorder, or a Combination of the two?
If due to a Haemorrhagic Disorder, Which of the three Components of the Haemostatic Mechanism is Affected?
What is cause of the haemorrhagic disorder?
18:
Thrombosis: Clinical Features and Management
CONDITIONS ASSOCIATED WITH NERVOUS THROMBOSIS
Congenital
Acquired
FACTORS PREDISPOSING TO BOTH ARTERIAL AND VENOUS THROMBOSIS
Clinical Syndromes of Thrombosis
Arterial Thrombosis
Myocardial Ischaemia and Infarction
Occlusive Cerebrovascular Disease and Transient Ischaemic Attacks
Peripheral Arterial Occlusive Disease
Venous Thrombosis
Microcirculation Thrombosis
Investigations of Thrombotic Disorders
Management of Thrombotic Disorders
Inhibitors of Platelet Function
Arterial Thrombosis
ANTICOAGULANTS
Heparin
Treatment of Established Thrombosis with Heparin
Duration of Treatment
Low Molecular Weight Heparin and Heparinoid Preparations
Warfarin or Vitamin K Antagonists
Control of Warfarin Dose
Thrombolytic Therapy with Streptokinase and Urokinase
Recent Advances in Therapeutic Plasminogen Activators
Thrombosis During Pregnancy
19:
Hypersplenism, Splenomegaly
NORMAL SPLENIC FUNCTIONS
HYPERSPLENISM
Aetiology
Causes of Hypersplenism
Diagnosis
The Diagnostic Criteria of Hypersplenism
Treatment
SPLENOMEGALY
Causes of Splenomegaly
Mild Splenomegaly
Moderate Enlargement
Marked Enlargement
INDICATIONS FOR SPLENECTOMY
Effects of Splenectomy
Haematological Effects
SPLENIC ATROPHY
Clinical Effects
20:
Blood Transfusion
HAEMOLYTIC DISEASE OF THE NEWBORN (HDN)
Cause of HDN
Clinical Features
Laboratory Findings at Birth
21:
Bone Marrow Transplantation
22:
Immunodeficiency Disorders
23:
Bone Marrow in Non-Haemopoietic Disease
GRANULOMATOUS DISEASE
Tuberculosis
Sarcoidosis
Other Granulomas
KALA-AZAR (VISCERAL LEISHMANIASIS)
OTHER INFECTIONS
GAUCHER'S DISEASE
NIEMANN-PICK DISEASE
SEA-BLUE HISTIOCYTE SYNDROME
Causes of Sea-Blue Histiocytes
Large Numbers
Occasional or Moderate Numbers
CYSTINOSIS
OSTEOPETROSIS (ALBERS-SCHOENBERG OR MARBLE BONE DISEASE)
AMYLOIDOSIS
Classification of Amyloidosis
RENAL OSTEODYSTROPHY AND OSTEOMALACIA
PAGET'S DISEASE OF BONE (OSTEITIS DEFORMANS)
ANOREXIA NERVOSA
PRIMARY OXALURIA
24:
Haemoparasites
HAEMOPARASITES
Malaria
A. Plasmodium vivax (Benign Tertian Malaria) (Fig. 24.1)
B. Plasmodium falciparum (Malignant Tertian Malaria) (Fig. 24.2)
FILARIASIS
Filariasis (Wuchereria bancrofti) (Fig. 24.3)
TOXOPLASMOSIS
TRYPANOSOMIASIS
BABESIOSIS
LOIASIS
BARTONELLOSIS
RELAPSING FEVER
25:
Practical Haematology
WAYS OF OBTAINING BLOOD
Capillary or Peripheral Blood
Venous Blood (Venipuncture)
Complications
ANTICOAGULANTS
HAEMOGLOBIN (Hb)
Haemoglobin Estimation
Sahil's Method
Cyanmethaemoglobin Method
Sheard-Sanford Oxyhaemoglobin Method
Other Methods
Alkali Haematin Method
Gasometric Method
Specific Gravity Method
Chemical Method
HAEMATOCRIT/PACKED CELL VOLUME (PCV)
Definition
Method
Wintrobe's Tube
Microhaematocrit
BLOOD CELL COUNTS
WBC
The Diluting Fluid
Counting Chamber
Methods
Falsely High Counts Occur due to
Falsely Low Counts Occur due to
Correcting the white cell count for nucleated red cells
Calculation
RBCs
Diluting Fluid
Method
PLATELETS
Method
ERYTHROCYTE SEDIMENTATION RATE
Methods
A. Westergren's Method
Normal Values
B. Wintrobe's Method
Normal Values
Sources of Error for Any ESR Method
Interpretation of ESR
Rapid ESR is found in
Slow ESR is usually seen in
Factors That Play a Role in ESR
1. Plasma Factors
2. Red Cell Factors
3. Anticoagulants
Stages in ESR
BLOOD FILM EXAMINATION
Preparation of a Thin Blood Film
Making of Spreaders
Making Thick Smears
Fixing of Blood Films
Staining of Blood Films
Buffer solution used in the laboratory.
Stain Preparation and Staining
Wright's Stain
Method
Leishman's Stain
Method
Giemsa's Stain
Method
Staining of Thick Films
Field's Stain
Method
Simeon's Modification of Boye's and Sterenel's Method
Method for Staining Thin Films
Procedure for Staining Thick Smears
Mounting and Preservation of Films
EXAMINATION OF A BLOOD FILM
Method
ALWAYS NOTE
Differential Leucocyte Count
BONE MARROW EXAMINATION
BONE MARROW EXAMINATION AND REPORTING
Marrow Film Preparation (Fig. 25.2)
Imprints
Crush Prepration
Staining of Marrow Preparations
Examination
Note the Undermentioned Points
Indications for Bone Marrow Aspiration
Bone Biopsy may be Needed in
Contraindications
Reticulocyte Count
Staining
Stain
Method
Normal Values
Red Cell Fragility Test
Quantitative Test
Reagents
Method
Factors Affecting Osmotic Fragility Tests
Interpretation
Qualitative Assessment of G6 PD Deficiency Methaemoglobin Reduction Test
Reagents
Method
Interpretation
Estimation of Foetal Haemoglobin
Qualitative Method
Interpretation
Quantitative Method
Step A
Step B
Tests for Sickling
Methods
Reagents
Methods
Reagents
Method
Interpretation
Method
Interpretation
Paul-Bunnell Test for Heterophile Antibody
LE Cell/Phenomenon (Lupus Erythematosus Cells)
Method
CAPILLARY FRAGILITY TEST OF HESS (Rumple-Leede Sign, Tourniquet Test)
BLEEDING TIME
Duke's Method
Requirements
Method
Precautions
Lvys's Method (Preferred because of greater ease of standardization).
Method
Interpretation
COAGULATION TIME
Capillary Tube Method of Wright
Lee and White Method
Requirements
Method
Normal Values
Precautions and Errors
CLOT RETRACTION
BLOOD BANKING IMMUNOHAEMATOLOGY
Preparation of Red Cell Suspensions
Method (for 2 percent Suspension)
Media for Collection and Preservation of Reagent Red Cells
Precautions
ABO Grouping
Six-Tube Method
Interpretation
Slide ABO Grouping Test
Rh Typing
Tube Test
Slide Test
Precautions and Errors
Rh Typing With Antisera Containing lgM Antibodies
COOMBS' TEST (Antiglobulin Test)
Direct Coombs’ Test
Interpretation
Indirect Coombs’ (Antiglobulin) Test
Method
Precautions and Errors
Titration of lgM Rh Antibodies
Method
BLOOD TRANSFUSION
Blood Donors
Donor Screening
Drawing of Blood
COMPATIBILITY TESTING
The Cross Match
Saline Cross Match
A. The Open Slide Method
B. Saline Tube Method
Immediate-Spin and Thermal Incubation Modification
C. Albumin Tube Method
D. Coombs’ Cross Matching
Method of Cross-Matching Universal Donor Blood
Factors leading to false results
If Still An Unexpected Incompatibility is Obtained
CHOICE OF MATERIAL FOR TRANSFUSION
BLOOD AND ITS PRODUCTS
1. Whole Blood
Fresh blood
2. Packed Cells
3. Plasma
BLOOD TRANSFUSION COMPLICATIONS
Complications Appearing Early
Complications Appearing Late
INVESTIGATIONS IN A CASE OF TRANSFUSION REACTION
Proceed as Mentioned Below
Interpretation of results
INDEX
TOC
Index
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