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Hematology for Students and Practitioners
Ramnik Sood
1:
History and Scope
2:
Blood Formation
SITES OF BLOOD FORMATION
DEVELOPMENT OF BLOOD CELLS
IN AN ADULT
Blood Formation has to Undergo Three Stages
ERYTHROPOIESIS (Chart 2.1, Figs 2.4 and 2.5).
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
Ineffective Erythropoiesis
LEUKOPOIESIS (FIG. 2.8)
MYELOPOIESIS
MYELOID SERIES
Myeloblast (Fig. 2.10A)
Promyelocytes (Neutrophilic) (Fig. 2.10B)
Myelocyte (Neutrophilic) (Fig. 2.10C)
Metamyelocyte (Neutrophilic) (Fig. 2.10D)
Band Neutrophils (Fig. 2.10E)
Polymorphonuclear Neutrophils (Fig. 2.10F)
EOSINOPHILS (FIG. 2.10G)
BASOPHILS AND MAST CELLS (FIGS 2.10H AND I)
LYMPHOCYTIC SERIES
Lymphoblast
Prolymphocyte
Large Lymphocyte
Small Lymphocyte
Further Embryological Subdivision
Activated and Reactive Lymphocytes
Plasmocytes
Plasmoblasts and Proplasmocytes (Figs 2.11F to H)
MONOCYTIC SERIES (FIGS 2.11I TO K AND 2.12)
Monoblast
Promonocyte
Monocyte
MACROPHAGES
Control of Leukopoiesis
LYMPHOKINES AND MONOKINES
Interleukin-1 (IL-1)
Interleukin-2 (IL-2)
Interleukin-4 (IL-4)
Interleukin-6
Interleukin-7 and 9–13
Interferons
Lymphocytes
PLATELET SERIES
Megakaryoblast
Promegakaryocyte
Megakaryocyte
Mature Platelet
Control of Platelet Formation
3:
Red Blood Cells
HISTORICAL ASPECTS
STRUCTURE
SHAPE
Dimensions
PHYSIOLOGY OF THE RED BLOOD CELLS
RED CELL MEMBRANE (FIG. 3.1)
Outerlayer
Middle Layer
Inner Layer
RED CELL MEMBRANE LIPIDS
Phospholipids
Glycolipids and Cholesterol
RBC Membrane Proteins (Fig. 3.2)
Integral Membrane Proteins
Peripheral Proteins
Deformability
Permeability
HEMOGLOBIN STRUCTURE AND FUNCTION
Hemoglobin Synthesis
Iron Delivery and Supply
Synthesis of Protoporphyrins
Globin Synthesis
HEMOGLOBIN FUNCTION
Active Red Cell Metabolic Pathways (Maintenance of Hemoglobin Function)
RBC Senescence/Aging
Nutritional and Other Requirements of a Red Cell
NORMAL RED CELL VALUES
RBC COUNT
Hemoglobin
Hematocrit (Packed cell volume) (PCV)
Mean Cell (corpuscular) Volume (MCV)
Mean Cell Hemoglobin (MCH)
Mean Cell Hemoglobin Concentration (MCHC)
Reticulocyte Count Blood
Mean Cell Diameter (Erythrocyte: Dry film)
Diurnal and Day-to-Day Variation
Red Cell Absolute Values/Indices
ANEMIA
DEFINITION
PATHOPHYSIOLOGY
Compensatory Physiological Adjustments
Clinical Features
Symptoms and Signs
Cardiorespiratory System
History and Physical Examination
CLASSIFICATION
Classification of Anemia Based on Pathogenesis and Etiology
Morphological Classification
Kinetic Classification
4:
Basic Principles in Diagnosing and Treating Anemias
INVESTIGATIONS
IS THE PATIENT ANEMIC?
History
WHAT IS THE TYPE OF ANEMIA?
CAUSE OF ANEMIA
EXAMINATION OF BLOOD FILM
Method
Always Note
TREATMENT
Treatment Includes
General Considerations
5:
Iron Deficiency Hypochromic Anemia
RELEVANT NORMAL VALUES
HEMOGLOBIN SYNTHESIS IN THE DEVELOPING RED CELL
IRON METABOLISM
ABSORPTION OF IRON
Mechanism and Control of Iron Absorption (Figs 5.1, 5.2 and Table 5.2)
IRON BALANCE
PATHOGENESIS OF IRON DEFICIENCY ANEMIA
Iron Deficiency: Stages of Development
Prevalence
Impaired Absorption
Blood Loss
Decreased Total Body Iron at Birth
Fetomaternal Transfusion
Growth
Diet in Infancy and Childhood
Blood Loss in Infancy
Pregnancy and Lactation
IRON DEFICIENCY ANEMIA
ETIOLOGY
Blood Loss
Other Causes of Chronic Blood Loss
Increased Requirements
Impaired Absorption
Inadequate Intake
Maldistribution
CLINICAL FEATURES
Anemia
Epithelial Tissue Changes
CLINICAL PRESENTATION
Growth
Fatigue
Neuromuscular Symptoms
Epithelial Changes
Immunity and Infection
Pica
Genitourinary System
Skeletal System
Spleen
Peripheral Blood Picture (Fig. 5.4)
Bone Marrow
Serum Biochemistry
Treatment
Differential Diagnosis of Iron Deficiency Anemia
PLUMMER-VINSON SYNDROME (PATERSON KELLY SYNDROME)
INVESTIGATIONS OF A PATIENT WITH IRON DEFICIENCY ANEMIA
HISTORY
Menstruating Females
Male and Post-menopausal Women
Infants and Children
PHYSICAL EXAMINATION
SPECIAL INVESTIGATIONS
Investigations Needed Commonly
Investigations Needed Occasionally
IRON OVERLOAD
HEMOSIDEROSIS
HEMOCHROMATOSIS
SIDEROBLASTIC ANEMIAS
RADIOACTIVE 59Fe STUDIES
BONE MARROW IRON
Criteria for Grading Iron Stains in Bone Marrow Aspirated
6:
Megaloblastic, Macrocytic Anemias
RELEVANT NORMAL VALUES
VITAMIN B12 KINETICS
FOLATE KINETICS
VITAMIN B12 DEFICIENCY
ETIOLOGY 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
Epithelial Changes
Gastrointestinal Secretion
Serum LDH and Other Enzymes
OTHER BIOCHEMICAL FINDINGS
COURSE, PROGNOSIS, AND COMPLICATIONS
OTHER CONDITIONS ASSOCIATED WITH VITAMIN B12 DEFICIENCY
Dietary Deficiency of Vitamin B12
Gastric Surgery
Intestinal Disorders
Hemodialysis
HIV Infection
ETIOLOGY (FIG. 6.1)
Gastric Cause
Intestinal Cause
Transport Protein Defects
Hemodialysis
CLINICAL MANIFESTATIONS
SPECIAL TESTS FOR DIAGNOSING VITAMIN B12 DEFICIENCY
Serum Vitamin B12 Assay
Radioactive Vitamin B12 Absorption Test
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
Tropical Sprue
Gluten-induced Enteropathy
CAUSES OF FOLATE DEFICIENCY (FIG. 6.2)
1. Nutritional
2. Malabsorption
3. Increased Demand
4. Excess Urinary Loss
5. Anticonvulsant Drug Therapy and Oral Contraceptives
6. Mixed
7. Cytotoxic Drugs
CLINICAL MANIFESTATIONS
SPECIAL TESTS FOR DIAGNOSING FOLATE DEFICIENCY
1. Serum Folate Assay
2. FIGLU Test
3. Radioactive Folic Acid Test
MANAGEMENT
Doses and Routes
PERIPHERAL BLOOD FINDINGS IN VITAMIN B12 OR FOLIC ACID DEFICIENCY (FIGS 6.3 TO 6.5)
BONE MARROW (FIGS 6.4A TO E)
Dyserythropoiesis
WBC Series
Megakaryocytes
MECHANISM OF ANEMIA IN MEGALOBLASTIC ANEMIA
Biochemical Basis of Megaloblastic Anemia
dU Suppression Test
DIAGNOSIS OF PERNICIOUS ANEMIA
SIGNS AND SYMPTOMS OF PERNICIOUS ANEMIA
Common to all Anemias
Suggest Vitamin B12 or Folic Acid Deficiency
Suggest Vitamin B12 Deficiency
Suggest Pernicious Anemia
Diagnosis
OTHER INVESTIGATIONS NEEDED IN FOLATE DEFICIENCY
MEGALOBLASTIC ANEMIA UNRESPONSIVE TO THERAPY
INHERITED AND DRUG-INDUCED MEGALOBLASTIC ANEMIAS
INHERITED DISORDERS ALTERING DNA SYNTHESIS
HOMOCYSTINURIA AND METHYLMALONYL ACIDURIA
ABNORMAL VITAMIN B12 TRANSPORT
OROTIC ACIDURIA
THIAMINE RESPONSIVE MEGALOBLASTIC ANEMIA
OTHER POSSIBLE DISORDERS
DRUG INDUCED DISORDERS OF DNA SYNTHESIS
FOLATE ANTAGONISTS
NITROUS OXIDE
OTHER DRUGS AND TOXINS
CAUSES OF MACROCYTOSIS OTHER THAN MEGALOBLASTIC ANEMIA
7:
Symptomatic Anemias
ACUTE HEMORRHAGIC ANEMIA
BLOOD PICTURE
HEMATOLOGIC FINDINGS
CLINICAL ASPECTS
CLINICAL DESCRIPTION
PATHOPHYSIOLOGY
MANAGEMENT
TREATMENT
ANEMIA OF INFECTION
ETIOLOGY
CLINICAL ASPECTS
BLOOD PICTURE
PATHOGENESIS
BIOCHEMICAL CHANGES
TREATMENT
TUBERCULOSIS
CHRONIC RENAL FAILURE
ETIOLOGY
MECHANISMS INVOLVED IN ANEMIA OF RENAL DISEASE
Hypoproliferative Anemia
Hemolytic Anemia
Dilutional Anemia
Blood Loss Anemia
Hypersplenism
BLOOD PICTURE (FIG. 7.1)
OTHER LABORATORY FINDINGS
PATHOGENESIS
DIAGNOSIS
TREATMENT
Early Treatment
Homotransplantation
Hemodialysis
Erythropoietin Resistance
Peritoneal Dialysis
MALIGNANCY
MECHANISMS OF ANEMIA IN MALIGNANCY
Direct Effects
Indirect Effects
Treatment Associated Anemia
TYPE OF ANEMIA
ANEMIA ASSOCIATED WITH SECONDARY CARCINOMA OF THE BONE MARROW
BLOOD PICTURE
BONE MARROW
CLINICAL ASPECTS
DIAGNOSIS
PROGNOSIS
LIVER DISEASE
MECHANISMS INVOLVED IN ANEMIA OF LIVER DISEASE
ERYTHROKINETICS AND PATHOGENESIS
TREATMENT
COLLAGEN DISEASE
RHEUMATOID ARTHRITIS
Pathogenesis
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
Anemia
The White Cell Count
Thrombocytopenia
LE Cell Phenomenon
ENDOCRINE DISORDERS
ANEMIA ASSOCIATED WITH ENDOCRINE DISORDERS
MYXEDEMA
HYPERTHYROIDISM
HYPOGONADISM
HYPOPITUITARISM
HYPERPARATHYROIDISM
ADDISON'S DISEASE
PROTEIN MALNUTRITION
SCURVY
PHYSIOLOGICAL ANEMIA OF PREGNANCY
PATHOGENESIS
NUTRITIONAL ANEMIA OF PREGNANCY
ANEMIA OF CHRONIC DISORDERS
CONDITIONS ASSOCIATED WITH ANEMIA OF CHRONIC DISORDERS
CLINICAL AND LABORATORY OBSERVATIONS
ANEMIA: DEVELOPMENT AND SEVERITY
Morphologic Features
Iron Metabolism
Other Biochemial Findings
Kinetic Characteristics
Pathogenesis
Cytokines
Shortened Red Cell Survival
Impaired Marrow Response
Abnormal Iron Metabolism
Anemia in Patients with Cancer
Diagnosis
Treatment
NORMOCYTIC AND NORMOCHROMIC ANEMIAS (CHART 7.1)
ETIOLOGICAL CLASSIFICATION OF NORMOCYTIC ANEMIA
SUMMARY OF THE INVESTIGATIONS OF A PATIENT WITH NORMOCYTIC ANEMIA
HISTORY
Usual, Plus with Special Reference to–
EXAMINATION
Usual, Plus with Special Reference to–
BLOOD EXAMINATION
Take Special Notice of–
SPECIAL INVESTIGATIONS
8:
Hemolytic Anemias
DEFINITION
CLASSIFICATION
INTRACORPUSCULAR
Hereditary or Congenital
Enzyme Defects
EXTRACORPUSCULAR
Acquired
ETIOPATHOGENETIC CLASSIFICATION OF HEMOLYTIC DISORDERS
Inherited Hemolytic Disease
Acquired Hemolytic Anemias
CLINICAL MANIFESTATIONS
CHRONIC CONGENITAL HEMOLYTIC ANEMIA
Degree of Anemia
Jaundice
Crises
Splenomegaly
Cholelithiasis
Leg Ulcers
Skeletal Abnormalities
ACQUIRED HEMOLYTIC ANEMIA
LABORATORY MANIFESTATIONS
Laboratory Signs of Accelerated Red Cell Destruction
Signs of Excessive Red Cell Destruction
Catabolism of Hem
Signs of Accelerated Erythropoiesis
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)
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
HEREDITARY SPHEROCYTOSIS (HS) (FIG. 8.2)
Pathogenesis (Flow chart 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
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) (FIG. 8.4)
Spherocytic Elliptocytosis (SE)
Southeast Asian Ovalocytosis (SAO)
Laboratory Findings
Treatment
STOMATOCYTIC DISORDERS
Hereditary Stomatocytosis (Fig. 8.5)
Hereditary Xerocytosis (Fig. 8.6)
Intermediate Stomatocytic Syndromes
Rh Null Disease
ACANTHOCYTIC DISORDERS (FIG. 8.7)
Abetalipoproteinemia
MacLeod Phenotype
Echinocytic Disorders (Fig. 8.8A)
Target Cell Disorders (Fig. 8.8B)
Familial Lecithin-Cholesterol Acyl-transferase (LCAT) Deficiency (Fig. 8.8C)
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
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 (FLOW CHART 8.4)
Pyrimidine 5' Nucleotidase Deficiency
Adenosine Deaminase Excess
Adenylate Kinase Deficiency
AUTOIMMUNE HEMOLYTIC ANEMIA (AIHA)
CLASSIFICATION
Warm-reactive Antibodies
Cold-reactive Antibodies
Drug-Induced Immune Hemolytic Anemia
WARM ANTIBODY AIHA
Warm Antibody Auto-immune Acquired Hemolytic Anemia
Clinical Picture
Hematological Picture (Fig. 8.10)
Immunology
Red Cell Destruction-Site and Mechanism
Intravascular Destruction
Diagnosis
Course and Prognosis
Therapeutics
COLD ANTIBODY AIHA
Cold Antibody Autoimmune Acquired Hemolytic Anemia
Cold Hemagglutinin Disease (CHAD)
Paroxysmal Cold Hemoglobinuria (PCH)
Paroxysmal Nocturnal Hemoglobinuria (PNH) (Marchiafava-Micheli Syndrome)
HEMOLYTIC ANEMIA DUE TO DRUGS AND CHEMICALS
HEMOLYTIC ANEMIAS CAUSED BY DRUGS
Direct Toxic Action of Drugs and Chemicals
Metabolic Abnormality in the Red Cell
Immune Based Mechanisms
AGENTS THAT MAY CAUSE HEMOLYTIC ANEMIA (DRUGS AND CHEMICALS)
Drugs that Regularly Cause Hemolytic Anemia in Normal Subjects by Direct Toxic Action
Drugs that Cause Hemolytic Anemia in Subjects with Hereditary Metabolic Abnormalities of the Red Cell (mainly G6PD Deficiency)
Drugs that Cause Hemolytic Anemia by Immune Based Mechanisms
HEMOLYTIC ANEMIA DUE TO DIRECT TOXIC ACTION
Drug Therapy
Occupational Poisoning
Household Poisoning
Contamination of Hemodialysis Systems
Hematological Picture
DRUG-INDUCED HEMOLYTIC ANEMIA DUE TO HEREDITARY RED CELL ENZYME DEFICIENCIES
DRUG-INDUCED HEMOLYTIC ANEMIA DUE TO UNSTABLE HEMOGLOBINS
IMMUNOLOGICAL DRUG-INDUCED HEMOLYTIC ANEMIA
DRUG-INDUCED IMMUNE HEMOLYTIC ANEMIA
DRUG ADSORPTION MECHANISM (see next page for Pictorial presentation)
IMMUNE COMPLEX MECHANISM (see next page for Pictorial presentation)
DRUG-INDUCED AUTO-IMMUNE HEMOLYTIC ANEMIA (TABLE 8.8)
MECHANICAL HEMOLYTIC ANEMIA
ETIOLOGY
CARDIAC HEMOLYTIC ANEMIA
MICRO-ANGIOPATHIC HEMOLYTIC ANEMIA
THE HEMOLYTIC UREMIC SYNDROME
Clinical Picture
Hematological Picture
Therapeutics and Prognosis
THROMBOTIC THROMBOCYTOPENIC PURPURA AND DISSEMINATED INTRAVASCULAR COAGULATION
DISSEMINATED CARCINOMA
MALIGNANT HYPERTENSION AND ECLAMPSIA
MARCH (EXERTIONAL) HEMOGLOBINURIA
HEMOLYTIC ANEMIA ASSOCIATED WITH BACTERIAL INFECTIONS AND PARASITIC INFESTATIONS
CLOSTRIDIUM WELCHII INFECTION
MALARIA
LEAD POISONING
BURNS
SUMMARY OF CLINICAL INVESTIGATIONS
PHYSICAL EXAMINATION
MANDATORY INVESTIGATIONS FOR ALL CASES
FURTHER INVESTIGATIONS NECESSARY IN SOME CASES
9:
Disorders of Hemoglobin Structure and Synthesis
NORMAL HEMOGLOBIN
HEMOGLOBIN TYPES
HEMOGLOBIN STRUCTURE
GENETIC REGULATION
ABNORMAL HEMOGLOBINS AND THE HEMOGLOBINOPATHIES
GENETIC REGULATION
TYPES OF STRUCTURAL ABNORMALITIES (TABLE 9.2)
LABORATORY DIAGNOSIS
Routine Hematological and Biochemical Tests
Investigations Depending on Physicochemical Properties of Abnormal Hemoglobins
Hemoglobin Electrophoresis
Alkali Denaturation
The Acid Elution Test
Hemoglobin Electrophoresis
SICKLE HEMOGLOBINOPATHIES
SICKLE CELL TRAIT (AS)
SICKLE CELL ANEMIA
Clinical
Sickle Cell Crises
Blood Picture
Prognosis
Treatment
COMBINATION OF HbS WITH OTHER HEMOGLOBINOPATHIES
SICKLE CELL β THALASSEMIA
SICKLE CELL HbC DISEASE
SICKLE CELL HbD DISEASE
OTHER HEMOGLOBINOPATHIES
HEMOGLOBIN C HEMOGLOBINOPATHIES
HEMOGLOBIN E HEMOGLOBINOPATHIES
HEMOGLOBIN D HEMOGLOBINOPATHIES
UNSTABLE HEMOGLOBIN HEMOGLOBINOPATHIES
LABORATORY DIAGNOSIS
Demonstration of Heinz Bodies
Heat Instability Test
Isopropanol Precipitation Test
Hemoglobin Electrophoresis
Clinical and Laboratory Findings
HEMOGLOBINOPATHIES ASSOCIATED WITH POLYCYTHEMIA
LABORATORY DIAGNOSIS
Hemoglobin Electrophoresis
Oxygen Dissociation Studies
Clinical and Laboratory Findings
THALASSEMIAS (Reduced Synthesis Rate)
CLASSIFICATION
LABORATORY DIAGNOSIS
Routine Hematological Tests
Demonstration of HbH Inclusions
Hemoglobin Electrophoresis
Globin Chain Synthesis Rate Studies
CLASSIFICATION OF THALASSEMIAS
In β Thalassemia
In α Thalassemia
β THALASSEMIAS
β THALASSEMIA MINOR (TRAIT)
β THALASSEMIA MAJOR
Clinical
Skeletal Changes
Growth and Sexual Development
Cardiopulmonary Complications
Hepatobiliary Disease
Blood Picture (Fig. 9.5) (Resembles iron deficiency anemia)
Bone Marrow
Hb Pattern
Laboratory Diagnosis
Pathophysiology of Anemia
Prognosis
Therapeutics
Prevention
DELTA BETA THALASSEMIA
THE HEMOGLOBIN-LEPORE SYNDROMES
HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN (HPFH)
α THALASSEMIA
CLASSIFICATION (TABLE 9.6)
ALPHA THALASSEMIA TRAIT
HEMOGLOBIN H DISEASE
HEMOGLOBIN-BART'S HYDROPS FETALIS
ALPHA THALASSEMIA AND OTHER HEMOGLOBINOPATHIES
METHEMOGLOBINEMIA
TOXIC METHEMOGLOBINEMIA
Drug Causes
Occupational Causes
Household Causes
Clinical Features
Diagnosis
Therapeutics
HEREDITARY METHEMOGLOBINEMIA
SULPHEMOGLOBINEMIA
10:
Aplastic Anemia, Pancytopenia
PANCYTOPENIA
ETIOLOGY
DIAGNOSIS OF PANCYTOPENIA
INVESTIGATION OF CASES WITH PANCYTOPENIA
Blood Examination
Bone Marrow Aspiration and Trephine
APLASTIC ANEMIA
CLASSIFICATION
ETIOLOGY OF APLASTIC ANEMIA
DRUG IDIOSYNCRASY
CHEMICAL EXPOSURE
APLASTIC ANEMIA AND INFECTIOUS HEPATITIS
FANCONI'S ANEMIA
CLINICAL
Primary
Secondary
SIGNS AND SYMPTOMS
BLOOD PICTURE
BONE MARROW MAY BE
Aspiration
DIAGNOSIS
COURSE AND PROGNOSIS
PREVENTION
MANAGEMENT
Identification and Elimination of Exposure to Causative Agent
Supportive Therapy
Measures to Accelerative Recovery from Pancytopenia
PURE RED CELL APLASIA
CONGENITAL OR DIAMOND-BLACKFAN ANEMIA
ACQUIRED PURE RED CELL APLASIA
DIFFERENTIAL DIAGNOSIS OF PANCYTOPENIA
MYELODYSPLASTIC DISORDERS
BONE MARROW INFILTRATION OR REPLACEMENT
MULTIPLE MYELOMA
SYSTEMIC LUPUS ERYTHEMATOSUS
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
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
LEUKOCYTE SURFACE ANTIGENS
NORMAL WHITE CELL VALUES
PATHOLOGICAL VARIATIONS IN WHITE CELL COUNTS
Neutrophilia
Eosinophilia
Lymphocytosis
Monocytosis
Basophilia
Basophilopenia
Eosinopenia
Lymphopenia
BONE MARROW PLASMACYTOSIS
Acute Infections
Chronic Infections
Allergic States
Collagen-vascular Disorders
Neoplasms
Other
CAUSES OF LEUKOERYTHROBLASTIC CHANGE
NEUTROPHIL ALKALINE PHOSPHATASE
Neutrophil Alkaline Phosphatase may be Raised in
Lowered in
NEUTROPENIA AND AGRANULOCYTOSIS
NEUTROPENIA AND THE ATTENDANT RISK OF INFECTION
CAUSE OF NEUTROPENIA
CLINICAL
Manifestations in Acute Neutropenia may be
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
FELTY'S SYNDROME
INFECTIOUS MONONUCLEOSIS
Etiology
Clinically
Blood Picture
Paul-Bunnell Test for Heterophile Antibody
Differential Diagnosis
DISORDERS OF PHAGOCYTIC LEUKOCYTES CHARACTERIZED BY MORPHOLOGIC CHANGES
Pelger-Huet Anomaly (Fig. 11.3)
Pseudo or Acquired Pelger-Huet Anomaly
Alder Reilly Anomaly (Fig. 11.4)
May-Hegglin Anomaly (Fig. 11.5)
Chediak-Steinbrinck-Higashi Anomaly (Fig. 11.6)
Mucopolysaccharidosis VI and VII (Fig. 11.7)
Other Causes of Lymphocyte Vacuolation
12:
Leukemias
ETIOLOGY
MOLECULAR BIOLOGY OF LEUKEMOGENESIS
ABNORMALITIES OF THE CHROMOSOMES
RADIATION
CHEMICALS
VIRUSES
GENETIC FACTORS
CLASSIFICATION
REVISED FAB CLASSIFICATION (WITH CRITERIA) OF AML
FAB CLASSIFICATION OF MYELODYSPLASTIC SYNDROMES
THE ACUTE LEUKEMIAS
COMMONLY FOUND ARE
INCIDENCE
CLINICAL FEATURES
LABORATORY FINDINGS
Routine Hematologic Investigations
Differentiation of ALL and AML
Special Investigations
Differential Diagnosis
DISORDERS WITH SIMILAR CLINICAL FEATURES
Other Reasons for Pharyngeal Ulceration
Other Reasons of Arthralgia and Bone Pain
Other Reasons of Fever and Malaise
DISORDERS WITH A SIMILAR BLOOD PICTURE
COURSE AND PROGNOSIS
TREATMENT
General Considerations
Specific Therapeutic Agents
Therapy in the Individual Patient
CHILDHOOD ACUTE LEUKEMIA
Acute Lymphoblastic Leukemia
Maintenance Therapy
Acute Myeloid Leukemia
ADULT ACUTE LEUKEMIA
Acute Lymphoblastic Leukemia
Acute Myeloid Leukemia
Supportive Care
Specific Aspects of Supportive Care
MYELODYSPLASTIC DISORDERS
Clinical Features
Blood Picture
Bone Marrow
THE CHRONIC LEUKEMIAS
CHRONIC GRANULOCYTIC LEUKEMIA (CML/CGL)
Clinical Features
Blood Picture
Bone Marrow
Course of Disorder
Chloroma
Treatment of Chronic Phase Disease
Chemotherapy
Other Forms of Treatment
Treatment of Blast Crisis
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
Classification of the Chronic Lymphoid Leukemias
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
OTHER CHRONIC LYMPHOPROLIFERATIVE DISORDERS
Hairy Cell Leukemia (see Fig. 12.2U)
PROLYMPHOCYTIC LEUKEMIA (see Fig. 12.2P(a))
LEUKEMOID REACTIONS
LEUKEMOID BLOOD PICTURE
Neutrophilic
Lymphocytic
Eosinophilic
THE DIAGNOSTIC PROBLEM
Myeloid Leukemoid Reactions
CAUSES OF MYELOID LEUKEMOID REACTIONS
Infections
Secondary to Non-hematological Malignancy
Acute Hemolysis
Leuko-erythroblastic Blood Picture
Causes of Lymphatic Leukemoid Reactions
Myelokathexis
13:
Paraproteinemias
MULTIPLE MYELOMA
DIAGRAM SHOWING CLINICAL FEATURES AND COMPLICATIONS IN MULTIPLE MYELOMA
Clinical Presentation
Bone Pain
Tumor Formation
Nervous System Involvement
Anemia
Renal Insufficiency
Bleeding Manifestations
Infections
Amyloidosis
Visceral Involvement
Cryoglobulinemia
Clinical Features in Relation to Immunoglobulin Class
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
Symptomatic Measures
Chemotherapy
Radiotherapy
Response and Prognosis
MACROGLOBULINEMIA
Classification of Macroglobulinemia
Waldenstrom's Macroglobulinemia
HEAVY CHAIN DISEASES (HCD)
BENIGN MONOCLONAL GAMMOPATHY (BENIGN PARAPROTEINEMIA)
AMYLOIDOSIS
MALIGNANT LYMPHOMA AND CHRONIC LYMPHOCYTIC LEUKEMIA
MISCELLANEOUS DISORDERS
PRINCIPLES OF THE DIFFERENTIAL DIAGNOSIS OF MONOCLONAL GAMMOPATHIES
CAUSES OF HYPERVISCOSITY SYNDROME
14:
Lymphoid Tissue Tumors (Malignant Lymphomas)
CLASSIFICATION OF LYMPHOMAS HODGKIN'S DISEASE
CLASSIFICATION OF NON-HODGKIN'S LYMPHOMAS
KIEL (LENNERT)
Low Grade Malignancy
High Grade Malignancy
LUKES-COLLINS
Undefined
RAPPAPORT (MODIFIED)
Nodular and/or Diffuse
Diffuse
NATIONAL CANCER INSTITUTE WORKING FORMULATION
Low Grade Malignancy
Intermediate Grade Malignancy
High Grade Malignancy
Miscellaneous
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
PRINCIPLES OF TREATMENT IN LYMPHOMA
General Considerations in Treatment
Basic Principles for Use of Chemotherapeutic Agents
Agents Used in Treatment of Lymphomas
Vinca Alkaloids
COMBINATION CHEMOTHERAPY FOR HODGKIN'S DISEASE
CHEMOTHERAPY FOR NON-HODGKIN'S LYMPHOMA
OUTCOME IN MALIGNANT LYMPHOMA
15:
Myeloproliferative Disorders
POLYCYTHEMIA
VARIOUS CAUSES OF POLYCYTHEMIA
Primary
Secondary
POLYCYTHEMIA VERA
PATHOPHYSIOLOGY
CLINICAL PRESENTATION
Central Nervous System
Cardiovascular System
Peripheral Vascular Disorders
Gastrointestinal Symptoms
Visual Disturbances
Thrombosis and Hemorrhage
Gout
Generalized Pruritus
GENERAL PHYSICAL EXAMINATION AND X-RAY
Skin and Mucous Membranes
Conjunctival Vessels
Splenomegaly
Chest X-ray
Peripheral Blood Picture
Biochemical Parameters
Bone Marrow Examination (Fig. 15.2)
ESTABLISHING DIAGNOSIS
Polycythemia Study Group Criteria for Diagnosis of Polycythemia Vera
Differential Diagnosis (Table 15.2)
COURSE AND PROGNOSIS
TREATMENT
Plan of Therapy in the Individual Patient
Choice of Marrow Depressive Agent
Venesection
Symptomatic Treatment
FAMILIAL POLYCYTHEMIA (FAMILIAL ERYTHROCYTOSIS)
RELATIVE POLYCYTHEMIA
PSEUDOPOLYCYTHEMIA
THROMBOCYTHEMIA
CAUSES OF RAISED PLATELET COUNT
Reactive
Endogenous
ESSENTIAL THROMBOCYTHEMIA (FIG. 15.3)
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 Hemostasis
NORMAL HEMOSTATIC MECHANISM (FIG. 16.1)
HEMOSTATIC PLUG–EVENTUAL FATE
EXTRAVASCULAR FACTORS
HEMORRHAGIC DISORDERS DUE TO CAPILLARY DEFECTS: NON THROMBOCYTOPENIC PURPURA (see Table 16.1)
ACQUIRED HEMORRHAGIC VASCULAR DISORDERS
Simple Easy Bruising (Purpura Simplex)
Senile Purpura (Involutional Purpura)
THE SYMPTOMATIC VASCULAR (NON-THROMBOCYTOPENIC) PURPURAS
Infections
Drugs
Uremia
Cushing's Disease and Corticosteroid Administration
Scurvy
Dysproteinemia
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 HEMORRHAGIC VASCULAR DISORDERS
Hereditary Hemorrhagic Telangiectasia
Ehlers-Danlos Disease
HEMORRHAGIC DISORDERS DUE TO PLATELET ABNORMALITIES
PLATELET PRODUCTION
Platelet Antigens and Antibodies
THROMBOCYTOPENIA
GENERAL CONSIDERATIONS
Platelet Count Vs. Bleeding
Positive Tourniquet Test
Excessive Bleeding Time
Etiology
IDIOPATHIC THROMBOCYTOPENIC PURPURA
Clinical Picture
Type and Site of Bleeding
Course of the Bleeding
Blood Picture
Bone Marrow
Diagnosis
EXCLUSION OF OTHER CAUSES (TABLE 16.3)
Pathogenesis
TREATMENT
Corticosteroids
Splenectomy
Supportive Treatment
Immunosuppressive Treatment
Immunoglobulin Infusion
Androgens
Pregnancy
IDIOPATHIC CYCLICAL THROMBOCYTOPENIA PURPURA
SECONDARY THROMBOCYTOPENIA
Etiology
Treatment
THROMBOCYTOPENIA DUE TO DRUGS AND CHEMICALS
Mechanism
Clinical Picture
Prognosis
Treatment Consists of
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
Medical
Surgical
FUNCTIONAL PLATELET DISORDERS
CLASSIFICATION OF PLATELET DYSFUNCTION
CONGENITAL QUALITATIVE PLATELET DEFECTS
Thrombasthenia (Glanzmann's Disease)
BERNARD-SOULIER SYNDROME
DEFECTS OF PLATELET ENZYMES
GRANULE DEFECTS
Storage Pool Disease (δ-SPD)
Gray Platelet Syndrome (α-SPD)
Combined Defects (αδ-SPD)
ACQUIRED PLATELET DYSFUNCTION
THROMBOCYTOSIS AND THROMBOCYTHEMIA
IDIOPATHIC (HEMORRHAGIC) THROMBOCYTHEMIA
Clinical Features
Blood Picture
Treatment
Course and Prognosis
17:
Coagulation Disorders
BLOOD COAGULATION PHYSIOLOGY
THE COAGULATION SYSTEM
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 (see Fig. 17.4)
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
General Supportive Measures and Care
CLINICAL DISORDERS DUE TO COAGULATION ABNORMALITIES
CONGENITAL COAGULATION DISORDERS
HEMOPHILIA A AND HEMOPHILIA B
Inheritance
Incidence
Clinical Features
Severity of the Bleeding Tendency
Variability of the Bleeding Tendency
Type and Site of Bleeding
Complications of Hemorrhage
Diagnosis
Course and Prognosis
Management
Surgery
VON WILLEBRAND'S DISEASE
OTHER CONGENITAL DISORDERS
Congenital Fibrinogen (Factor I) Deficiency
Inherited Dysfibrinogenemia
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
ACQUIRED COAGULATION DISORDERS
VITAMIN K DEFICIENCY
Etiology of Vitamin K Deficiency
Treatment
LIVER DISEASE
Pathogenic Factors in Bleeding
Bleeding in Hepatitis
Treatment
ANTICOAGULANT DRUGS
MONITORING OF ANTICOAGULANT THERAPY
BLEEDING DURING ANTICOAGULANT THERAPY
Increased Susceptibility
Accidental Overdose
Deliberate Overdosage
Surgery and Anticoagulation Therapy
TREATMENT
Bleeding during Heparin Therapy
Bleeding during Oral Anticoagulant Therapy
DISSEMINATED INTRAVASCULAR COAGULATION
PATHOGENESIS
ETIOLOGY
CLINICAL FEATURES
TYPE OF BLEEDING
PREGNANCY
Abruptio Placentae
Amniotic Fluid Embolism
Intrauterine Death
SURGERY
Heatstroke with Hyperpyrexia
LABORATORY DIAGNOSIS
TREATMENT
Elimination of Precipitating Factor
Replacement of Coagulation Factors and Platelets
Inhibition of the Clotting Process
HEMORRHAGE AND BLOOD TRANSFUSION
BLEEDING AFTER TRANSFUSION OF LARGE AMOUNTS OF STORED BLOOD
The Amount
AGE OF BLOOD
The Circumstances Requiring Blood Transfusion
Treatment
HEMORRHAGIC DISORDERS DUE TO CIRCULATING INHIBITORS OF COAGULATION
Inhibitors in Hemophilia
Treatment
OUTLINE OF INVESTIGATIONS OF A PATIENT WITH A BLEEDING TENDENCY
IS THE BLEEDING DUE TO LOCAL PATHOLOGICAL LESION, A HEMORRHAGIC DISORDER, OR A COMBINATION OF THE TWO?
DUE TO A HEMORRHAGIC DISORDER, WHICH OF THE THREE COMPONENTS OF THE HEMOSTATIC MECHANISM IS AFFECTED?
WHAT IS CAUSE OF THE HEMORRHAGIC DISORDER?
18:
Thrombosis: Clinical Features and Management
DEFINITION OF THROMBOSIS
TYPES OF THROMBUS
EFFECTS OF THROMBOSIS
ETIOLOGY OF THROMBOSIS
VASCULAR DISEASE AND ARTERIAL THROMBOSIS
Hyperlipidemia
Diabetes Mellitus
Smoking
Exercise and Body Build
Hypertension
Hypercoagulability
Inherited, Racial, and Dietary Factors
CONDITIONS ASSOCIATED WITH VENOUS THROMBOSIS (see Table 18.1)
Congenital
Acquired
FACTORS PREDISPOSING TO BOTH ARTERIAL AND VENOUS THROMBOSIS
CLINICAL SYNDROMES OF THROMBOSIS
ARTERIAL THROMBOSIS
MYOCARDIAL ISCHEMIA AND INFARCTION
OCCLUSIVE CEREBROVASCULAR DISEASE AND TRANSIENT ISCHEMIC ATTACKS
PERIPHERAL ARTERIAL OCCLUSIVE DISEASE
VENOUS THROMBOSIS
MICROCIRCULATION THROMBOSIS
INVESTIGATIONS OF THROMBOTIC DISORDERS (see Table 18.3)
MANAGEMENT OF THROMBOTIC DISORDERS
INHIBITORS OF PLATELET FUNCTION
ARTERIAL THROMBOSIS
ANTICOAGULANTS
Heparin
Warfarin or Vitamin K Antagonists
Thrombolytic Therapy with Streptokinase and Urokinase (see Fig. 18.2)
Recent Advances in Therapeutic Plasminogen Activators
Thrombosis During Pregnancy
19:
Hypersplenism, Splenomegaly
NORMAL SPLENIC FUNCTIONS
HYPERSPLENISM
ETIOLOGY
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
Hematological Effects
SPLENIC ATROPHY
CLINICAL EFFECTS
Susceptibility to Infection after Splenectomy
20:
Bone Marrow Transplantation
INDICATIONS
BONE MARROW (OR PERIPHERAL BLOOD STEM CELL) TRANSPLANTATION
Allogeneic (or Syngeneic)
Autologous
COMPLICATION OF BONE MARROW TRANSPLANTATION
EARLY (<100 DAYS)
Acute GVHD
LATE (>100 DAYS)
Chronic GVHD
GRAFT VERSUS HOST DISEASE (GVHD)
21:
Immunodeficiency Disorders
ACQUIRED IMMUNE DEFICIENCY SYNDROME (AIDS)
CLINICAL STAGES OF INFECTIONS WITH THE HIV VIRUS
Group I
Group 2
Group 3
Group 4
HEMATOLOGICAL MANIFESTATIONS OF HIV DISEASE
OVERVIEW OF HIV/AIDS
HUMAN IMMUNODEFICIENCY VIRUS
HIV Virus
Origin of HIV
The HIV Virion
HIV Types and Variants
Life Cycle of the HIV
Clinically Apparent Disease
HIV Specific Prognostic Markers
Non-specific Markers of HIV Progression (Fig. 21.12)
HIV Diagnostic Testing Techniques
Enzyme Immunoassays (EIA)
Virus Detection Methods
p24 Antigen and Nucleic Acid Technologies (PCR)
Rapid Tests for the Detection of Anti-HIV Antibodies
Flow Through Technology
Lateral Flow Technology
Diagnosis
Approach to Confirmatory Supplementary Tests
Western Blot (WB)
NAT-PCR Based Methods
Alternative Approach to Confirmatory Testing
WHO/UNAIDs Strategies for HIV Antibody Screening
Discrimination Between HIV-1 and HIV-2 Infection
Pediatric HIV Infection
Transmission of HIV from Mother to Infants
Diagnostic Challenges: Child Born to a HIV Infected Mother
RAPID IMMUNOCONCENTRATION TEST FOR HIV 1 AND HIV 2 ANTIBODIES FLOW THROUGH METHOD Retroquick-HIV® Courtesy: Tulip Group of Companies
THIRD GENERATION DOUBLE ANTIGEN SANDWICH ENZYME LINKED IMMUNOSORBENT ASSAY (ELISA) FOR THE DETECTION OF TOTAL ANTIBODIES TO HIV-1 AND HIV-2 INCLUDING HIV-1 GROUP “O” IN HUMAN SERUM OR PLASMA RETROLISA 3.0® Courtesy: Tulip Group of Companies
22:
Bone Marrow in Non-Hemopoietic Disease
GRANULOMATOUS DISEASE
TUBERCULOSIS (FIG. 22.3)
SARCOIDOSIS
OTHER GRANULOMAS
KALA-AZAR (VISCERAL LEISHMANIASIS) (FIG. 22.4)
OTHER INFECTIONS
GAUCHER'S DISEASE (FIG. 22.5)
NIEMANN-PICK DISEASE (FIG. 22.6)
SEA-BLUE HISTIOCYTE SYNDROME (FIG. 22.7)
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
23:
Hemoparasites
MALARIAL PARASITES OF MAN
BLOOD FLAGELLATES OF MAN
LEISHMANIASIS
VISCERAL LEISHMANIASIS (KALA AZAR) CAUSED BY LEISHMANIA DONAVANI
CUTANEOUS LEISHMANIASIS (ORIENTAL SORE, CHICLERO'S DISEASE, UTA, ETC.) CAUSED BY LEISHMANIA TROPICA
TRYPANOSOMIASES: AFRICAN TYPE: SLEEPING SICKNESS
AFRICAN TRYPANOSOMIASES (SLEEPING SICKNESS) (CONTD…)
TRYPANOSOMIASIS SOUTH AMERICAN TYPE: CHAGAS’ DISEASE
SOUTH AMERICAN TRYPANOSOMIASIS (CHAGAS’ DISEASE) (CONTD…)
TISSUE ROUNDWORMS OF MAN, CHIEFLY FILARIAE
PLASMID NEMATODES (CONTD…)
The Filarial worms
The Body Fluid and Tissue Flagellates (Causing Leishmaniasis and Trypanosomiasis)
Laboratory Diagnosis of Malaria
Malaria Species Identification in the Mosquito—Pigment in Oocysts
Recapitulation
TOXOPLASMOSIS (FIG. 23.1)
BABESIOSIS (FIG. 23.2)
BARTONELLOSIS (FIG. 23.3)
RELAPSING FEVER (FIG. 23.30)
24:
Practical Hematology
WAYS OF OBTAINING BLOOD
CAPILLARY OR PERIPHERAL BLOOD
VENOUS BLOOD (VENIPUNCTURE)
Complications
Clinical Alert
ANTICOAGULANTS
EDTA
Advantages of EDTA
Disadvantages of EDTA
Making EDTA Bulbs
OXALATES
Making Double Oxalate Bulbs
TRISODIUM CITRATE
HEPARIN
SPECIAL ANTICOAGULANTS
WHEREVER POSSIBLE, THE NECESSARY TESTS, INVESTIGATIONS AND PREPARATION OF BLOOD FILMS SHOULD BE DONE IMMEDIATELY
ANTICOAGULATED BLOOD STORAGE AND BLOOD CELL MORPHOLOGY
Peripheral Smears
EDTA Blood
DIAGNOSTIC ALERTS
BLOOD COLLECTION SYSTEM
HEMOGLOBIN (Hb)
HEMOGLOBIN ESTIMATION: SAHLI'S METHOD: (SAHLI'S HEMOGLOBINOMETER) (FIG. 24.3)
CYANMETHEMOGLOBIN METHOD
Drabkin's Reagent
SHEARD-SANFORD OXYHEMOGLOBIN METHOD
OTHER METHODS
Alkali Hematin Method
Gasometric Method
Specific Gravity Method
Chemical Methods
SODIUM LAURYL SULPHATE METHOD (AVAILABLE FROM CORAL CLINICAL SYSTEMS, GOA)
Normal Hemoglobin Values
HEMATOCRIT/PACKED CELL VOLUME (PCV)
DEFINITION
METHODS
Wintrobe's Tube
Microhematocrit
INTERPRETATION
BLOOD CELL COUNTS
WBC
Counting Chamber
Methods
Calculation
RBCs
Diluting Fluid
Method
Interpretation
PLATELETS
Method
Rees-Ecker Method for Platelet Count
Rough Estimation of Platelet Count from Stained Thin Smear
ERYTHROCYTE INDICES
1. THE MEAN CELL VOLUME (MCV)
2. THE MEAN CELL HEMOGLOBIN (MCH)
3. THE MEAN CELL HEMOGLOBIN CONCENTRATION (MCHC)
COMPLETE BLOOD COUNT (CBC), BLOOD
NORMAL VALUES
COMPLETE BLOOD COUNT (CBC)
ERYTHROCYTE SEDIMENTATION RATE (ESR)
METHODS
WESTERGREN'S METHOD
Normal Values
WINTROBE'S METHOD
Normal Values
MICROSEDIMENTATION (LANDAU) METHOD
Procedure
Normal Values
ZETA SEDIMENTATION RATE (ZSR)
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
INTERFERING FACTORS
BLOOD FILM EXAMINATION
PREPARATION OF A THIN BLOOD FILM
MAKING OF SPREADERS (FIG. 24.9)
MAKING THICK SMEARS
FIXING OF BLOOD FILMS
STAINING OF BLOOD FILMS
Buffer Solution used in the Laboratory
Stain Preparation and Staining
Staining of Thick Films
Mounting and Preservation of Films
RAPID DIAGNOSTICS
AUTOMATION IN HEMATOLOGY COULTER PRINCIPLE
THE BASICS OF HEMATOLOGY ANALYZERS IN A NUTSHELL
COULTER¨ MAXM AND MAXM AL HEMATOLOGY FLOW CYTOMETRY SYSTEMS (FIG. 24.11)
EXAMINATION OF A BLOOD FILM
Method
Always Note
BONE MARROW EXAMINATION
Bone Marrow Examination and Reporting
RETICULOCYTE COUNT
Normal Values
Staining
Stain
Method
RED CELL FRAGILITY TEST
Quantitative Test
Clinical Implications
QUALITATIVE ASSESSMENT OF G6PD DEFICIENCY
Methemoglobin Reduction Test
QUANTITATIVE ESTIMATION OF G6PD Courtesy: Tulip Group of Companies
EXAMINATION OF FETAL HEMOGLOBIN
Qualitative Method
Quantitative Method
TESTS FOR SICKLING
Methods
LABORATORY DIAGNOSIS OF LEUKEMIAS
Cytochemical Methods for Staining Leukocytes
Neutrophil Alkaline Phosphatase (Kaplow's Method)
Peroxidase (Myeloperoxidase, Kaplow's Method)
Periodic Acid-Schiff (PAS) Reaction
Sudan Black B Stain (Sheehan and Storey)
Nonspecific Esterase (Yam et al)
QUALITY CONTROL IN HEMATOLOGY
INTERNAL QUALITY CONTROL
Testing Control Sample
Control Chart (Levy-Jennings or L-J Chart)
Cusum Analysis
Duplicate Tests
Inbuilt Quality Control
EXTERNAL QUALITY ASSESSMENT
STANDARDIZATION
PROFICIENCY SURVEILLANCE
PLATELETS, COAGULATION AND BLEEDING DISORDERS: LABORATORY INVESTIGATIONS
CAPILLARY FRAGILITY TEST OF HESS
(Rumpel-Leede Sign, Tourniquet Test)
Clinical Implications
LABORATORY DIAGNOSIS OF VASCULAR BLEEDING DISORDERS
Interfering Factors
BLEEDING TIME
Duke's Method
Ivys's Method
Interpretation
Clinical Implications
Interfering Factors
Patient Preparation
COAGULATION TIME
Capillary Tube Method of Wright
Lee and White's Method
CLOT RETRACTION
Principle
Requirements
Interpretation
Errors
SENSITIVE THROMBOPLASTIN REAGENT FOR PROTHROMBIN TIME (PT) DETERMINATION (ISI=1.0) Uniplastin® (Courtesy: Tulip Group of Companies)
APTT/PTTK CEPHALOPLASTIN REAGENT FOR PARTIAL THROMBOPLASTIN TIME (APTT) DETERMINATION USING ELLAGIC ACID AS ACTIVATOR Liquicecin-E® (Courtesy: Tulip Group of Companies)
REQUIREMENTS
Method
Result
DILUTE BLOOD CLOT LYSIS TIME
FDPS A Qualitative and Semiquantitative Latex Slide Test for Detecting Cross-linked Fibrin Degradation Products in Human Plasma X-L FDP Courtesy: Tulip Group of Companies
AUTOMATION IN COAGULATION ANALYSIS
HEMOSTAR
Technical Features
HEMOSTAR XF
Technical Features
CoaLAB 6000
Compact
Versatile
User Friendly
Technical Specifications
TROUBLESHOOTING
GENERAL INSTRUCTIONS FOR COAGULATION TEST
Sample Collection Techniques
Sample Processing and Storage
Calibration of Instruments/Equipments
Storage and Procedure
Critical Requirement of MNPT in the Derivation of INR
ISI Value of PT Used and Method of Clot Detection
INR SYSTEM
PROTHROMBIN TIME
Uniplastin®/Liquiplastin®/Lyoplastin®
DPTT/PTTK
FIBRINOGEN DEGRADATION PRODUCTS (D-DIMER) ESTIMATION
25:
Immunohematology Blood Banking
BLOOD GROUP ANTIBODIES
NATURALLY OCCURRING ANTIBODIES
GENETICS OF ABO SYSTEM
BIOCHEMISTRY
SUBGROUPS OF A AND AB
Subgroups of B
Antibodies of ABO System
Anti-H
ABO TESTING PROCEDURES
ABO GROUPING
SLIDE ABO GROUPING TEST
SIX-TUBE METHOD (FIG. 25.3)
Interpretation
Rh BLOOD GROUP SYSTEM
Rh(D) GROUPING PROCEDURES
Slide Testing
Interpretation
Testing for Du
ANTIHUMAN GLOBULIN TEST
HISTORICAL PERSPECTIVE
PRELIMINARY NOTES: ANTIGEN AND ANTIBODY RELATED TO BLOOD GROUP SEROLOGY
ANTIBODY NOMENCLATURE WITH RESPECT TO BLOOD GROUP SEROLOGY
IgM CLASS ANTIBODIES
IgG CLASS ANTIBODIES
ROLE OF ENHANCEMENT MEDIUM
ENHANCEMENT TECHNIQUES
Albumin Additives
Enzymes
Positively Charged Molecules
Polyethylene Glycol (PEG)
LISS (Low Ionic Salt Solution) and LISS additives
COMPLEMENT
Immunoglobulin Requirements for Activation of Classical Pathway
Complement Binding Antibodies (Table 25.5)
BASIC CONCEPTS OF ANTI-HUMAN GLOBULIN TESTING
DIRECT ANTI-HUMAN GLOBULIN TEST (DAT)
MAJOR APPLICATIONS OF DAT IN BLOOD GROUP SEROLOGY
Hemolytic Disease of the Newborn (HDN)
Transfusion Reactions
Other Immune Hemolytic Diseases
Classification of Autoimmune Hemolytic Anemia
Drug-induced Hemolytic Anemia
Importance of Serological Studies in DAT Positive Results (Table 25.6)
INDIRECT ANTI-HUMAN GLOBULIN TEST (IAT)
Applications of IAT
Probable Sources of Error in Anti-human Globulin Testing
BLOOD TRANSFUSION
BLOOD DONORS
Donor Screening
Drawing of Blood
ADVERSE donor reactions
COMPATIBILITY TESTING
The Cross-match
Method of Cross-matching Universal Donor Blood
CHOICE OF MATERIAL FOR TRANSFUSION
BLOOD AND ITS PRODUCTS
Fresh Blood
Packed Cells
Plasma
BLOOD TRANSFUSION COMPLICATIONS
Complications Appearing Early
INVESTIGATIONS IN A CASE OF TRANSFUSION REACTION
Proceed as Mentioned Below
Interpretation of Results
LABORATORY DIAGNOSIS OF HEMOLYTIC DISEASE OF THE NEWBORN
TROUBLESHOOTING
GENERAL INSTRUCTIONS FOR BLOOD GROUPING
Sample Preparation
Sample Processing
Sample Storage
Equipments
Reagents
Common Causes of False-negative and False-positive Results in ABO Testing
ABO GROUPING
Problem: False Positive Results
Problem: False Negative Results
Problem: Hemolysis of Red Blood Cells
Problem: Weak Agglutination
Problem: Mixed Field Agglutination in ABO Grouping or Discrepancy Observed Between the Red Cell Group and the Reverse Group
Problem: Delayed Agglutination
Rh TYPING
Problem: False Positive Results
Problem: False Negative Results
Problem: Hemolysis of Red Blood Cells
Problem: Delayed or Weak Agglutination
GENERAL INSTRUCTIONS FOR ANTIHUMAN GLOBULIN (COOMBS REAGENT)
Sources of Error in Antiglobulin Testing—Coombs Cells
ANTI-HUMAN GLOBULIN (AHG OR COOMBS REAGENT)
Problem: False Positive Results
Problem: False Negative Results
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