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Rapid Review of Hematology
Ramadas Nayak, Sharada Rai
SECTION 1: DISORDERS OF RED CELLS
CHAPTER 1:
Anemias of Impaired Red Cell Production
ANEMIA
Definition
Classification of Anemia
RED CELL INDICES
IRON DEFICIENCY ANEMIA
Etiology (Table 1.3)
Pathogenesis of Iron Deficiency Anemia
Laboratory Findings
Peripheral Blood
Serum Iron Profile (Table 1.4)
Reticulocyte Hemoglobin
Clinical Features of IDA
Physical Findings
Causes of Microcytic Hypochromic Anemia
MEGALOBLASTIC ANEMIA
Etiology of Megaloblastic Anemia (Table 1.5)
Pathogenesis of Megaloblastic Change
Laboratory Findings of Megaloblastic Anemia
Peripheral Blood
Biochemical Tests for Megaloblastic Anemia
PERNICIOUS ANEMIA
Etiopathogenesis
Morphology
Alimentary System
Central Nervous System
Laboratory Findings (Fig. 1.8)
Specific Diagnostic Tests for Pernicious Anemia
Clinical Features of Megaloblastic Anemia
APLASTIC ANEMIA
Etiology
Pathogenesis (Fig. 1.9)
Clinical Features
Laboratory Findings
Peripheral Blood
No Splenomegaly
Differential Diagnosis
CHAPTER 2:
Hemolytic Anemias Due to Red Cell Membrane and Enzyme Defects
HEMOLYTIC ANEMIA
Definition
Classification of Hemolytic Anemias (Table 2.1)
Location of Hemolysis
HEREDITARY SPHEROCYTOSIS
Etiopathogenesis
Mechanism of Hemolysis in HS (Fig. 2.2)
Laboratory Findings
Peripheral Blood
Biochemical Findings
Osmotic Fragility Test
Clinical Features
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY
Role of G6PD (Fig. 2.7)
Sequence of Events in G6PD Deficiency
Clinical Presentation
Laboratory Findings
Peripheral Blood
Urine
RBC Enzyme Analysis
CHAPTER 3:
Thalassemia Syndrome
CLASSIFICATION OF HEREDITARY DEFECTS IN HEMOGLOBIN
THALASSEMIA SYNDROME
Classification
β-THALASSEMIA
Molecular Pathology
Clinical and Genetic Classification (Table 3.1)
β-THALASSEMIA MAJOR
Pathophysiology of β-thalassemia Major (Fig. 3.1)
Consequence of Defective or Absent β-chains
Consequences of Ineffective Erythropoiesis
Iron Overload and its Consequences
Clinical Features
Laboratory Findings
Peripheral Blood
Biochemical Findings
Special Tests
Differences between Iron Deficiency Anemia and β-Thalassemia Major (Table 3.3)
β-THALASSEMIA MINOR/TRAIT
Laboratory Findings in β-Thalassemia Minor
α-THALASSEMIA
Molecular Pathology
Clinical Syndromes
CHAPTER 4:
Sickle Cell Disease
SICKLE CELL DISEASE
Definition
Classification of Sickle Cell Disease (Table 4.1)
SICKLE CELL ANEMIA
Characteristic Features
Etiopathogenesis
Molecular Basis of Sickling (Fig. 4.2)
Factors Affecting Sickling (Table 4.2)
Mechanism of Red Cell Damage
Pathogenesis of the Microvascular Occlusions
Clinical Features (Fig. 4.3)
Chronic Hemolytic Anemia
Crises
Increased Susceptibility to Infections
Chronic Organ Damage
Laboratory Findings in Sickle Cell Anemia
Peripheral Blood
Serum Findings
Diagnostic/Confirmatory Tests
SICKLE CELL TRAIT
Pathogenesis
Clinical Features
Laboratory Findings
Peripheral Blood
Diagnostic Tests
CHAPTER 5:
Other Anemias
IMMUNOHEMOLYTIC ANEMIAS
Classification of Immunohemolytic Anemias (Table 5.1)
Alloimmune Hemolytic Anemia
HEMOLYTIC DISEASE OF THE NEWBORN
Rh Hemolytic Disease of the Newborn (Fig. 5.1)
Pathogenesis
Clinicopathological Features
Laboratory Findings
ABO Hemolytic Disease of the Newborn
ANTIGLOBULIN (COOMBS) TEST
Principle
Types of Antiglobulin Test (Fig. 5.2)
Direct Antiglobulin Test (Fig. 5.2)
Uses of Direct Antiglobulin Test
Indirect Antiglobulin Test (Fig. 5.2)
Uses of Indirect Antiglobulin Test
AUTOIMMUNE HEMOLYTIC ANEMIA
Warm Antibody Type
Cold Agglutinin Type
Cold Hemolysins Type (Donath-Landsteiner Antibodies)
FRAGMENTATION SYNDROME
Classification
PAROXYSMAL NOCTURNAL HEMOGLOBINURIA
Etiology and Pathogenesis
Clinical Features
Laboratory Findings
ANEMIAS OF BLOOD LOSS
Acute Blood Loss (Hemorrhage)
Chronic Blood Loss
SIDEROBLASTIC ANEMIAS
SECTION 2: DISORDERS OF WHITE CELLS
CHAPTER 6:
Quantitative and Qualitative Disorders of Leukocytes
NORMAL DIFFERENTIAL LEUKOCYTE COUNT (DLC)
QUANTITATIVE DISORDERS OF LEUKOCYTES
Leukocytosis
Leukopenia
Disorders of Neutrophils
Neutrophilia (Fig. 6.1)
Leukemoid Reaction
Neutropenia (Agranulocytosis)
Eosinophilia (Fig. 6.2)
Basophilia
Monocytosis (Table 6.8)
Lymphocytosis
Lymphocytopenia
QUALITATIVE DISORDERS OF LEUKOCYTES
INFECTIOUS MONONUCLEOSIS: (GLANDULAR FEVER)
Pathogenesis
Clinical Features
Laboratory Findings
CHAPTER 7:
Acute Leukemia
ACUTE LEUKEMIA
Definition
Etiology and Pathogenesis
Classification
FAB Classification of Acute Leukemias
WHO Classification (2008) of Acute Leukemia (Table 7.4)
Differences between Myeloblast and Lymphoblast (Table 7.5)
ACUTE LYMPHOBLASTIC: LEUKEMIA/LYMPHOMA
Molecular Pathogenesis
Classification of Acute Lymphoblastic: Leukemia (Tables 7.3, 7.4 and 7.6)
Clinical Features
Laboratory Findings
Peripheral Blood
Cytochemistry of Lymphoblasts
Immunophenotyping
Biochemical Findings
CSF Examination
ACUTE MYELOGENOUS LEUKEMIA
Molecular Pathogenesis
Clinical Features
Laboratory Findings
Peripheral Blood
Cytochemistry of Myeloblasts (Figs 7.10 and 7.11)
Immunophenotyping
Cytogenetics
MYELOID SARCOMA
CHAPTER 8:
Myelodysplastic Syndromes
MYELODYSPLASTIC SYNDROMES
Classification
Clinical Features
Laboratory Findings
Bone Marrow Trephine Biopsy
CHAPTER 9:
Myeloproliferative Neoplasms
MYELOPROLIFERATIVE NEOPLASMS (MPN)
WHO Classification of MPN
Pathogenesis
POLYCYTHEMIA OR ERYTHROCYTOSIS
POLYCYTHEMIA VERA
Molecular Pathogenesis (Figs 9.1 and 9.2)
Clinical Features
Phases
Laboratory Findings
Peripheral Blood (Fig. 9.3)
Bone Marrow Biopsy
Other Findings
ESSENTIAL THROMBOCYTHEMIA
Etiology
Clinical Features
Laboratory Findings
PRIMARY MYELOFIBROSIS
Molecular Pathogenesis
Clinical Features
Laboratory Findings
Bone Marrow Biopsy
CHAPTER 10:
Chronic Myelogenous Leukemia
CHRONIC MYELOGENOUS LEUKEMIA
Definition
Etiology and Pathogenesis
Molecular Pathogenesis
Philadelphia (Ph) Chromosome (Fig. 10.1)
BCR-ABL Fusion Gene (Fig. 10.2)
Clinical Features
NATURAL HISTORY OF CHRONIC MYELOID LEUKEMIA
Chronic/Stable/Indolent Phase (CP)
Laboratory Findings
Accelerated Phase (AP) (Figs 10.5 and 10.6)
Blast Phase/Crisis (BP)
CHAPTER 11:
Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma
CHRONIC LYMPHOCYTIC LEUKEMIA
Definition
Etiology and Pathogenesis
Cytogenetic Abnormalities
Clinical Features
Laboratory Findings
Peripheral Blood
Immunophenotype
Lymph Node
HAIRY CELL LEUKEMIA
Definition
Laboratory Findings
Peripheral Blood
Spleen
Immunophenotype and Molecular Characteristics
Clinical Features
CHAPTER 12:
Plasma Cell Neoplasms
INTRODUCTION
Definition
Characteristics of Plasma Cell Neoplasms
Classification of Plasma Cell Neoplasms (Table 12.1)
PLASMA CELL MYELOMA (MULTIPLE MYELOMA)
Definition
Etiology
Risk Factors
Laboratory Findings
Peripheral Blood
Serum Findings
Electrophoretic Studies on Serum and Urine (Figs 12.5 and 12.6)
Morphology of Organs Involved
Clinical Manifestations (Fig. 12.8)
PLASMACYTOMA
IMMUNOGLOBULIN DEPOSITION DISEASE
Primary Amyloidosis
MONOCLONAL GAMMOPATHY OF UNCERTAIN SIGNIFICANCE (MGUS)
CHAPTER 13:
Lymphoid Neoplasms
CLASSIFICATION OF LYMPHOID NEOPLASMS (TABLE 13.1)
FOLLICULAR LYMPHOMA (FL)
Morphology
Gross
Microscopy
Clinical Features
DIFFUSE LARGE B CELL LYMPHOMA (DLBCL)
Microscopy
Immunophenotype
Cytogenetics and Molecular Profile
Clinical Features
BURKITT LYMPHOMA (BL)
Clinical Variants
Microscopy
Immunophenotype
Cytogenetic and Molecular Genetic Features (Fig. 13.5)
Translocations of c-MYCgene
MATURE T CELL AND NK CELL NEOPLASMS
Peripheral T Cell Lymphoma (PTCL), NOS
Microscopy
Immunophenotype
Mycosis Fungoides
Microscopy
Sézary Syndrome
CHAPTER 14:
Hodgkin Lymphomas
DEFINITION
CLASSIFICATION (TABLE 14.1)
Cell of Origin and Immunophenotype
MORPHOLOGY OF NEOPLASTIC CELLS
CLASSICAL HODGKIN LYMPHOMA
Nodular Sclerosis Classical Hodgkin Lymphoma (NSCHL)
Microscopy of NSCHL (Fig. 14.3)
Immunophenotype
Mixed Cellularity Classical Hodgkin Lymphoma (MCCHL)
Microscopy of MCCHL (Fig. 14.4)
Lymphocyte-rich Classical Hodgkin Lymphoma (LRCHL)
Microscopy of LRCHL (Fig. 14.5)
Lymphocyte-depleted Classical Hodgkin Lymphoma (LDCHL)
Microscopy of LDCHL (Fig. 14.6)
NODULAR LYMPHOCYTE PREDOMINANT HODGKIN LYMPHOMA (NLPHL)
Microscopy of NLPHL (Fig. 14.7)
ETIOLOGY AND PATHOGENESIS OF HODGKIN LYMPHOMA
Pathogenesis (Fig. 14.8)
LABORATORY FINDINGS
Spread
STAGING OF HODGKIN LYMPHOMA (TABLE 14.3)
DIFFERENCES BETWEEN HODGKIN LYMPHOMA AND NON-HODGKIN LYMPHOMA
CHAPTER 15:
Langerhans Cell Histiocytosis/Histiocytosis X
INTRODUCTION
MORPHOLOGY
LABORATORY FINDINGS
SECTION 3: DISORDERS OF HEMOSTASIS
CHAPTER 16:
Disorders of Primary Hemostasis
NORMAL HEMOSTASIS
Primary Hemostatic Plug
Secondary Hemostatic Plug
CLASSIFICATION OF HEMOSTATIC DISORDERS (TABLE 16.1)
BLEEDING DISORDERS CAUSED BY VESSEL WALL ABNORMALITIES
BLEEDING DISORDERS DUE TO ABNORMALITIES OF PLATELET
Classification of Platelet Disorders (Table 16.3)
THROMBOCYTOPENIA
Clinical Features of Thrombocytopenia
Severity of Bleeding
Causes of Thrombocytopenia (Table 16.4)
IMMUNE THROMBOCYTOPENIC PURPURA
Types of Immune Thrombocytopenic Purpura (ITP)
Acute Immune Thrombocytopenic Purpura
Chronic Immune Thrombocytopenic Purpura
Laboratory Findings
Peripheral Blood
THROMBOCYTOSIS
QUALITATIVE PLATELET DISORDERS
CHAPTER 17:
Bleeding Disorders: Due to Abnormalities of Coagulation/Clotting Factor
INTRODUCTION
CLASSIFICATION OF COAGULATION DISORDERS (TABLE 17.2)
HEREDITARY COAGULATION DISORDERS
Factor VIII-vWF Complex
HEMOPHILIA
HEMOPHILIA A (FACTOR VIII DEFICIENCY)
Mode of Inheritance (Fig. 17.1)
Molecular Genetics
Clinical Features
Laboratory Findings
Complications
Due to Hemophilia
Due to Therapy
HEMOPHILIA B (CHRISTMAS DISEASE, FACTOR IX DEFICIENCY)
Laboratory Findings
VON WILLEBRAND DISEASE (vWD)
Categories
Clinical Features
Laboratory Findings
ACQUIRED COAGULATION DISORDERS
Coagulation Factor Abnormalities
DISSEMINATED INTRAVASCULAR COAGULATION
Etiology
Pathogenesis (Fig. 17.2)
1. Thrombi/Clot Formation
2. Hemorrhagic Diathesis
Clinical Features
Laboratory Findings in DIC
Screening Assays
Confirmatory Tests
CHAPTER 18:
Thrombotic Disorders: Hypercoagulable State
HYPERCOAGULABLE STATE (THROMBOPHILIA)
Causes of Hypercoagulability State (Table 18.1)
INHERITED HYPERCOAGULABLE STATES
Clinical Presentation
Deficiency of Antithrombotic Factors
Antithrombin (AT) III Deficiency
Protein C and S Deficiency
Increased Prothrombotic Factors
Activated Protein C (APC) Resistance (Factor V Leiden)
ACQUIRED HYPERCOAGULABLE STATES
Causes
Antiphospholipid Antibody Syndrome (APLA/APS)
Types
Clinical Features
Laboratory Tests
SECTION 4: CLINICAL SCENARIO
CHAPTER 19:
Clinical Scenario
INTRODUCTION
SYMPTOMS AND SIGNS THAT SUGGEST A BLOOD DISEASE (TABLE 19.1)
PATTERNS STRONGLY SUGGESTIVE OF A BLOOD DISEASE
Pattern 1: Iron Deficiency Anemia
Pattern 2: Megaloblastic Anemia
Pattern 3: Hereditary Spherocytosis
Pattern 4: Thalassemia Major
Pattern 5: Sickle Cell Anemia
Pattern 6: Immune Thrombocytopenic Purpura (ITP)
Pattern 7: Hemophilia
Pattern 8: Acute Lymphoblastic Leukemia (ALL)
Pattern 9: Acute Myeloblastic Leukemia (AML)
Pattern 10: Chronic Myelogenous Leukemia (CML)
Pattern 11: Chronic Lymphocytic Leukemia (CLL)
Pattern 12: Multiple Myeloma
Pattern 13: Hodgkin Lymphoma (HL)
Appendix
HEMOPARASITES
Bibliography
INDEX
TOC
Index
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