Hematology and Coagulation: A Comprehensive Review for Board Preparation, Certification and Clinical Practice

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Hematology and Coagulation
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Dedication
Preface
1 - Complete blood count and peripheral smear examination
	Introduction
	Analysis of various parameters by hematology analyzers
		Histograms
		Red blood cell histogram
		Platelet histogram
		White blood cell histogram
		Red blood cell count and hemoglobin measurement
		Hematocrit, RDW, MCV, MCH, and MCHC
		Reticulocyte count
		White blood cell count and differential
		Platelet count, mean platelet volume, platelet differential width
	Review of peripheral smear
		Red cell variations and inclusions
		White blood cell morphology
		Platelets
	Special situations with complete blood count and peripheral smear examination
		Splenic atrophy or postsplenectomy
		Microangiopathic hemolysis
		Leukoerythroblastic blood picture
		Parasites, microorganisms and nonhematopoietic cells in the peripheral blood
		Buffy coat preparation
		Key points
	References
2 - Bone marrow examination and interpretation
	Introduction
	Fundamentals of bone marrow examination
		Dry tap
		Granulopoiesis
		Erythropoiesis
		Monopoiesis, megakaryopoiesis, thrombopoiesis, and other cells in bone marrow
	Various bone marrow examination findings and bone marrow failure
		Disorders of erythropoiesis, granulopoiesis, and thrombopoiesis
		Infections
		Granulomatous changes
		Storage disorders
		Metabolic bone diseases
		Metastatic tumors
		Hemophagocytic syndrome
		Bone marrow necrosis/infarction
		Serous atrophy
		Bone marrow fibrosis
		Reactive lymphoid aggregate
		Bone marrow infiltration in lymphoproliferative disorders
		Amyloidosis
		Key points
	References
3 -
 Red blood cell disorders
	Introduction
	Anemia: morphological and etiological classification
	Common causes of anemia
		Dimorphic red cells
		Anemia due to blood loss
		Iron deficiency anemia
		Lead poisoning
		Anemia of chronic disease
		Sideroblastic anemia
		Megaloblastic anemia
		Bone marrow failure
		Congenital dyserythropoietic anemia
	Hemolytic anemia
		Hemolytic anemia due to corpuscular defects
		Hemolytic anemia due to membrane defects
		Hereditary spherocytosis (aka Minkowski-Chauffard disease)
		Hereditary elliptocytosis
		Hereditary pyropoikilocytosis
		Hereditary stomatocytosis
		Rh null disease
		Hemolytic anemia due to enzyme defects
		Pyruvate kinase deficiency
		Glucose-6-phosphate dehydrogenase deficiency
		Paroxysmal nocturnal hemoglobinuria
		Hemolytic anemias due to extracorpuscular defects
	Red cell poikilocytosis
	Red cell inclusions
		Malaria parasites
		Key points
	References
4 - Hemoglobinopathies and thalassemias
	Introduction
	Hemoglobin structure and synthesis
	Introduction to hemoglobinopathies and thalassemias
		α-thalassemia
			Diagnosis of α-thalassemias
		β-thalassemia
			Diagnosis of β-thalassemia
				β-Thalassemia trait
				β-Thalassemia major
		δ-thalassemia
		δβ-Thalassemia
		Sickle cell disease
			Diagnostic approach to an individual who demonstrates HbS on electrophoresis
			Diagnosis of sickle cell trait
			Diagnosis of sickle cell trait with concomitant α-thalassemia trait
			Diagnosis of sickle cell disease
			Diagnosis of sickle cell disease patient who has been transfused or has undergone red blood cell exchange
			Diagnosis of sickle cell/β-thalassemia (this in turn can be S/β 0- or S/β+- or S/β++ thalassemia)
			Diagnosis of sickle cell disease patient on hydroxyurea
			Diagnosis of Hb SC disease
			Diagnosis of HbS/hereditary persistence of fetal hemoglobin
			Diagnosis of normal individual who has been transfused red blood cells from a sickle cell trait donor
		Hereditary persistence of fetal hemoglobin
	Other hemoglobin variants
	Individuals with high hemoglobin F
	Fast, unstable, and other rare hemoglobins
	Laboratory investigation of hemoglobinopathies
		Gel electrophoresis
		High-performance liquid chromatography
		Capillary electrophoresis
	Reporting normal hemoglobin electrophoresis pattern
	Apparent hemoglobinopathy after blood transfusion
	Universal newborn screen
		Key points
	References
5 - Benign white blood cell and platelet disorders
	Introduction
	Hereditary variation in white blood cell morphology
	Changes in white cell counts
		Neutrophilia
		Eosinophilia and monocytosis
		Basophilia
		Neutropenia
		Lymphocytosis and infectious mononucleosis
		Lymphocytopenia
	Platelet disorders
		Thrombocytopenias
		Thrombocytosis
		Thrombocytopathia
		Key points
	References
6  -
Myeloid neoplasms
	Introduction
	Classification of myeloid neoplasms
	Myeloproliferative neoplasm
		Chronic myeloid leukemia, BCR-ABL1 positive
		Chronic neutrophilic leukemia
		Polycythemia vera, primary myelofibrosis, and essential thrombocythemia
		Chronic eosinophilic leukemia
		Myeloproliferative neoplasm, unclassifiable
		Mastocytosis
	Myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement
	Myelodysplastic/myeloproliferative neoplasms
	Myelodysplastic syndromes
		Features of dysplasia in red cells, erythroid precursors, granulocytes and megakaryocytes
		Arriving at a diagnosis of MDS and subclassifying MDS
			Abnormal localization of immature precursors
		Cytogenetic abnormalities associated with myelodysplastic syndrome
		Unusual situations in myelodysplastic syndrome
	Myeloid neoplasms with germline predisposition
	Acute leukemia
		Blasts
		Cytochemistry
		Classification of acute myeloid leukemia and diagnosis
		Acute myeloid leukemia with recurrent genetic abnormalities
		Acute myeloid leukemia, not otherwise specified
		Myeloid sarcoma
		Myeloid proliferations associated with Down syndrome
		Acute myeloid leukemia and flow cytometry
		Molecular and acute myeloid leukemia
		Cytogenetics and acute myeloid leukemia
		Key points
	References
7 - Monoclonal gammopathies and their detection
	Introduction
	Diagnostic approach to monoclonal gammopathy using electrophoresis
		Serum protein electrophoresis
		Urine electrophoresis
		Immunofixation studies
		Capillary zone electrophoresis
		Free light chain assay
		Heavy/light chain assay
		Paraprotein interferences in clinical laboratory tests
	Plasma cell disorders
		Monoclonal gammopathy of undetermined significance
		Idiopathic Bence Jones proteinuria
		Monoclonal gammopathy of renal significance
		POEMS
		Plasmacytoma
		Monoclonal immunoglobin deposition disease (MIDD)
		Plasma cell myeloma
		Morphology of plasma cells in myeloma
		Immunophenotype of neoplastic plasma cells
	Cytogenetics in myeloma diagnosis
		Key points
	References
8 - Application of flow cytometry in diagnosis of hematological disorders
	Introduction
	Flow cytometry and mature B-cell lymphoid neoplasms
		B-cell markers
		Chronic lymphocytic leukemia/small lymphocytic lymphomas
		B-cell prolymphocytic leukemia
		Mantle cell lymphoma
		Follicular lymphoma
		Hairy cell leukemia
		Burkitt lymphoma
		Diffuse large B-cell lymphoma
	Flow cytometry and mature T and natural killer–cell lymphoid neoplasm
		Detection of clonal or restricted population of T and natural killer cells
		Sézary syndrome and mycosis fungoides
		T-cell prolymphocytic leukemia
		T-cell large granular lymphocytic leukemia
		Chronic lymphoproliferative disorder of natural killer cells
	Plasma cell dyscrasias
	Flow cytometry and acute leukemia
		Flow cytometry and subtypes of acute myeloid leukemia
		Flow cytometry of B-lymphoblastic leukemia/lymphoma
		Flow cytometry of T-lymphoblastic leukemia/lymphoma
		Flow cytometry of mixed phenotype acute leukemia
		Flow cytometry of blastic plasmacytoid dendritic cell neoplasm
	Flow cytometry and myelodysplastic syndrome
	Flow cytometry and hematogones
		Key points
	References
9 - Cytogenetic and genetic abnormalities in hematologic neoplasms
	Introduction
	Cytogenetic abnormalities in chronic myeloid leukemia
	Cytogenetic abnormalities in myelodysplastic syndrome
	Cytogenetic abnormalities in patients with myeloid malignancies
	Cytogenetic abnormalities in myeloproliferative neoplasms
	Cytogenetic abnormalities in acute lymphoblastic leukemia
	Cytogenetic abnormalities in multiple myeloma
	Cytogenetic and genetic abnormalities in B- and T-cell lymphomas
		Key points
	References
10 -
Benign lymph node
	Introduction
	Reactive lymphoid states
		Viral lymphadenopathy
		Bacterial infections and lymphadenopathy
		Cat-scratch lymphadenopathy
		Toxoplasma gondii and lymphadenopathy
		Granulomatous lymphadenopathy
		Necrotizing lymphadenopathy
		Progressive transformation of germinal centers
		Regressive changes in germinal center
	Specific clinical entities with lymphadenopathy
		Kikuchi-Fujimoto disease
		Kimura disease
		Kawasaki disease
		Dermatopathic lymphadenitis
		Lymphadenopathy in autoimmune diseases
		Rosai–Dorfman disease
		Langerhans cell histiocytosis
		Castleman disease
		IgG4-related sclerosing disease–associated lymphadenopathy
		Key points
	References
11  Precursor lymphoid neoplasms, blastic plasmacytoid dendritic cell neoplasm, and acute leukemias of ambiguous lineage
	Introduction
	B-lymphoblastic leukemia/lymphoma, NOS
		Morphology and immunophenotype
		Remission and cure rates
		B-lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities
	T-lymphoblastic leukemia/lymphoma
		Morphology and immunophenotype
		Prognosis
	NK-lymphoblastic leukemia/lymphoma
	Blastic plasmacytoid dendrite cell neoplasm
	Acute leukemias of ambiguous lineage
		Requirement for assigning lineage to a single blast population
		Key points
	References
12 - B-cell lymphomas
	Introduction
	Diffuse large B-cell lymphoma
		Morphologic variants include
		Molecular subtypes
		Immunohistochemistry for suspected diffuse large B-cell lymphoma
		Other lymphomas of large B cells
		Plasmablastic neoplasms
		CD5 positive aggressive B-cell lymphomas
		High-grade B-cell lymphomas
		B-cell lymphomas unclassifiable
	Follicular lymphoma
		Usual follicular lymphoma
			Patterns of follicular lymphoma
			Grading of follicular lymphoma
		Immunophenotyping in follicular lymphoma
		Genetics of follicular lymphoma
		Variants of follicular lymphoma
		In situ follicular neoplasia
		Duodenal-type follicular lymphoma
		Testicular follicular lymphoma
		Diffuse variant of follicular lymphoma
		Distinct and separate types of follicular lymphoma
	Chronic lymphocytic leukemia/small lymphocytic lymphoma
		CLL/SLL and certain percentages
		Morphology of chronic lymphocytic leukemia in peripheral blood
		Immunophenotyping for CLL/SLL
		Staging of chronic lymphocytic leukemia
			Rai staging
			Binet staging
		Morphology of lymph nodes in CLL/SLL
		CLL/SLL and cytogenetics
		CLL/SLL and prognosis
		Monoclonal B-cell lymphocytosis
	B-cell prolymphocytic leukemia
	Mantle cell lymphoma
		Prognosis
		Indolent mantle cell lymphoma
		In situ mantle cell neoplasia
	Marginal zone B-cell lymphoma
		Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue
		Splenic marginal zone lymphoma
		Nodal marginal zone lymphoma
	Burkitt lymphoma
	Lymphoblastic leukemia/lymphoblastic lymphoma
	Lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia
	Hairy cell leukemia
		Approach for diagnosis of lymphoma
		Key points
	References
13 - T- and natural killer–cell lymphomas
	Introduction
	Nodal T-cell lymphomas
		Angioimmunoblastic T-cell lymphoma
		Other nodal T-cell lymphomas of T follicular helper cell origin
		Peripheral T-cell lymphoma, not otherwise specified
		Anaplastic large cell lymphoma
		ALK positive ALCL
		ALK-negative ALCL
	Extranodal natural killer–/T-cell lymphomas
		Breast implant–associated anaplastic large cell lymphoma
		Extranodal natural killer–/T-cell lymphoma, nasal type
		Intestinal T-cell lymphoma
		Hepatosplenic T-cell lymphoma
		Subcutaneous panniculitis-like T-cell lymphoma
			EBV positive T-cell and NK-cell lymphoproliferative diseases of childhood
	Cutaneous T-cell lymphoma
		Mycosis fungoides
		Sézary syndrome
		Primary cutaneous CD30 positive T-cell lymphoproliferative disease
		Primary cutaneous peripheral T-cell lymphomas, rare subtypes
		EBV positive T-cell and NK-cell lymphoproliferative diseases of childhood
	Leukemia/disseminated
		T-cell prolymphocytic leukemia
		T-cell large granular lymphocyte leukemia
		Chronic lymphoproliferative disorders of natural killer cells
		Aggressive natural killer–cell leukemia
		Adult T-cell leukemia/lymphoma
		Key points
	References
14 -
Hodgkin lymphoma
	Introduction
	Overview of Hodgkin lymphoma
	Classification of Hodgkin lymphoma
		Nodular lymphocyte predominant Hodgkin lymphoma
			Sites of involvement
			Histology
			Immunophenotype
		Classic Hodgkin lymphoma
			Immunophenotype of CHL
		Nodular sclerosis classic Hodgkin lymphoma
		Mixed cellularity classic Hodgkin lymphoma
		Lymphocyte rich classic Hodgkin lymphoma
		Lymphocyte depleted classic Hodgkin lymphoma
	Immunostains for diagnosis of Hodgkin lymphoma
		Differential diagnosis
	Staging of Hodgkin lymphoma
		Key points
	References
15 - Lymphoproliferative disorders associated with immune deficiencies, histiocytic and dendritic cell neoplasms, and blastic pl ...
	Introduction
	Lymphoproliferative disorders associated with immune deficiency
		Lymphoproliferative disorders associated with primary immune deficiency
		Lymphoproliferative disorders associated with human immunodeficiency virus infection
		Posttransplant lymphoproliferative disorders
		Other iatrogenic immunodeficiency–associated lymphoproliferative disorders
	Histocytic and dendritic cell neoplasms
		Histiocytic sarcoma
		Dendritic cell neoplasms
		Tumors derived from Langerhans cells
		Langerhans cell histiocytosis
		Langerhans cell sarcoma
		Indeterminate dendritic cell tumor
		Interdigitating dendritic cell sarcomas
		Follicular dendritic cell sarcoma
		Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma
		Erdheim–Chester disease
	Blastic plasmacytoid dendritic cell neoplasm
		Key points
	References
16 -
Essentials of coagulation
	Introduction
	Normal hemostasis
		Platelets and platelet events
	Thrombocytopenia and thrombocytopathia
		Hereditary thrombocytopenias
		Pseudothrombocytopenia
			Thrombocytopathia
	Tests for platelet function
		Bleeding time
		Capillary fragility test
		PFA-100
			Interpretation of PFA-100
		VerifyNow
		Plateletworks
		Platelet aggregation
		Thromboelastography
		Use of TEG in management of bleeding patient
		Platelet mapping
		Idiopathic thrombocytopenic purpura
		Heparin-induced thrombocytopenia
			Pathophysiology
			Assessment of a patient with heparin-induced thrombocytopenia
			Calculating the 4T score
	Secondary hemostasis
		Extrinsic pathway
		Intrinsic pathway
		Common pathway
	Tests for secondary hemostasis
		Prothrombin time
		Partial thromboplastin time
		Thrombin time
		PT/PTT mixing study
		Factor assays
		Tests for bleeding patients
		Von Willebrand disease
		Diagnosis of various types of von Willebrand disease
	Antiplatelets and anticoagulants
		Key points
	References
17  -
Thrombophilia and their detection
	Introduction
	Thrombophilia: inherited versus acquired
	Factor V Leiden
	Activated protein C resistance test
	Prothrombin gene mutation
	Protien C deficiency
	Protein S deficiency
		Assays for protein C and protein S
	Antithrombin III deficiency
	Hyperhomocysteinemia
	Increased factor VIII activity
	Acquired causes of thrombophilia
		Antiphospholipid syndrome
		Laboratory testing for lupus anticoagulant
		Key points
	References
18 -
 Sources of errors in hematology and coagulation
	Introduction
	Errors in common hematology testing
		Errors in hemoglobin measurement and red blood cell count
		Errors in mean corpuscular volume and related measurements
		Errors in WBC counts and WBC differential counts
		Errors in platelet count
	Errors in specific hematology testing
		Cold agglutinins
		Cryoglobulins
		Pseudothrombocytopenia
		Spurious leukocytosis
		False-positive osmotic fragility test
	Errors in coagulation testing
		Errors in PT and aPTT measurements
		Errors in thrombin time measurement
			Incorrectly filled tubes
			Dilution or contamination with anticoagulants
			Traumatic phlebotomy
			Fibrinolysis products and rheumatoid factor
		Platelet aggregation testing with lipemic, hemolyzed, or thrombocytopenic samples
		Challenges in anticoagulants and lupus anticoagulant tests
		Key points
	References
Index
	A
	B
	C
	D
	E
	F
	G
	H
	I
	K
	L
	M
	N
	O
	P
	Q
	R
	S
	T
	U
	V
	W
	X
	Z
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