51 research outputs found
Cytotoxic T lymphocyte antigen-4 (CTLA-4) A49G polymorphism and autoimmune blood diseases
Objective: The cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is expressed on T lymphocytes, and inhibits the T-cell responses. In animal models, it has been shown that complete CTLA-4 deficiency was lethal due to massive infiltration of tissues by polyclonally proliferating lymphocytes. CTLA-4 A49G polymorphism, which has been suggested to reduce the inhibitory function of the CTLA-4 molecule, was found to be associated with various autoimmune diseases in recent studies. Material and Methods: In this study, we evaluated the frequency of CTLA-4 A49G polymorphism in 46 patients with autoimmune hemolytic anemia (AIHA), 62 patients with immune thrombocytopenic purpura (ITP), and 150 healthy individuals. Results: Allele frequencies and genotype distributions were similar in both ITP and AIHA patients compared to healthy individuals. In subgroup analysis, however, we found that in chronic lymphocytic leukemia (CLL) patients with AIHA (n=4), all patients had CTLA-4 A49G polymorphism (3 had AG, 1 had GG). There was no significant statistical association between G allele and systemic lupus erythematosus (SLE) or AIHA.Conclusion: These data suggest that CTLA-4 A49G polymorphism does not contribute to the pathogenesis of lymphoproliferative diseases itself, nor does it increase the risk of autoimmune complications in patients with lymphoproliferative disease
Acute myocardial infarction in a patient with hemophilia A and factor V Leiden mutation
Factor VIII:C, epsilon amino-caproic acid or tranexamic acid are prophylactic agents used in
preventing hemorrhage pre-operatively in patients with hemophilia A. Although hemophilia A
seems to be a factor that avoids the development of acute myocardial infarction (AMI) as it
tends to be associated with increased bleeding, it should be kept in mind that prothrombotic
agents used pre-operatively for prophylaxis may increase the risk for AMI in the presence of the
factor V Leiden mutation. In this report, we discuss the development of AMI following the use
of recombinant factor VIII and tranexamic acid for prophylaxis in a patient with known
hemophilia before a tooth extraction in conjunction with the relevant literature
Importance of Eosinopenia in COVID-19 Infection
Objective: Dynamic changes in the number of eosinophils are observed during the diagnosis and follow-up in coronavirus disease-2019(COVID-19). Our aim was to show the role of the absolute eosinophil count in the diagnosis of COVID-19 and the relationship with diseaseseverity and prognosis.Methods: In this study, 191 patients (130 inpatients, 61 outpatients) diagnosed with COVID-19 pneumonia with the polymerase chain reactiontest and lung computed tomography; and 22 patients with positive influenza test were included as the control group. All demographic,biochemical data, clinical and radiological characteristics were recorded.Results: The mean eosinophils on first day of the inpatient COVID-19 group were found to be statistically lower than the influenza group andthe ambulatory groups (p=0.001, p=0.0001).Conclusion: A low eosinophil count in complete blood count, can aid in the early diagnosis of infection. Persistent eosinopenia progresseswith disease severity and may help determine the prognosis of the disease
Diffuse alveolar hemorrhage associated with thrombotic thrombocytopenic purpura after allogeneic bone marrow transplantation
ABSTRACT Alveolar hemorrhage is an early complication after bone marrow transplantation (BMT) and often associated with inflammatory pulmonary processes. We present a case of diffuse alveolar hemorrhage associated with BMT associated thrombotic thrombocytopenic purpura (BMT-TTP). An 18-years-old man with acute myeloid leukaemia (FAB; M5) underwent ABO incompatible BMT from his HLA-identical sister. On the 37 th day of BMT, BMT-TTP was diagnosed with the occurrence of red cell fragmentation and rise in serum lactic dehydrogenase (LDH) level with severe sudden decrease in hemoglobin and platelet levels. Cyclosporine A (CsA) was ceased and plasma infusion with plasma exchange was started. On the 42 nd day of BMT, the diagnosis of diffuse alveolar hemorrhage was made by the clinical, bronchoscopic and bronchoalveolar lavage fluid findings. Alveolar hemorrhage among patients with BMT-TTP has been scarce reported. These two complications may be regarded as related, as small vessel injury is a central feature in both and they may share aetiological and pathogenetic factors
Acquired disorders of platelet function
Acquired disorders of platelet function are among the most common hematologic abnormalities, a reflection of the sensitivities of platelets to external and internal perturbations. The clinical challenge in evaluating acquired disorders of platelet function is to determine whether observed derangements in platelet function pose a threat to the patient.CONTENTS
PART I
MOLECULAR AND CELLULAR BASIS OF HEMATOLOGY 1
Chapter 1
Anatomy and Physiology of the Gene 2
Andrew J. Wagner, Nancy Berliner, and Edward J. Benz, Jr.
Chapter 2
Genomic Approaches to Hematology 16
Todd R. Golub
Chapter 3
Regulation of Gene Expression, Transcription, Splicing, and RNA Metabolism 27
Christopher R. Cagle, Amy Meacham, and Robert A. Hromas
Chapter 4
Protein Synthesis, Processing, and Trafficking 35
Randal J. Kaufman and Laura Popolo
Chapter 5
Protein Architecture: Relationship of Form
and Function 48
Jia-huai Wang, Angela Toms, Ming-Ming Zhou,
and Michael J. Eck
Chapter 6
Signaling Transduction and Regulation
of Cell Metabolism 55
Pere Puigserver
Chapter 7
Pharmacogenomics and Hematologic Diseases 65
Leo Kager and William E. Evans
PART II
CELLULAR BASIS OF HEMATOLOGY 77
Chapter 8
Hematopoietic Stem Cell Biology 78
John P. Chute
Chapter 9
Hematopoietic Microenvironment 88
Lev Silberstein and David Scadden
Chapter 10
Cell Adhesion 97
Rodger P. McEver and Francis W. Luscinskas
Chapter 11
Hematopoietic Cell Trafficking and Chemokines 105
Steffen Massberg, Alexander G. Khandoga, and
Ulrich H. van Andrian
Chapter 12
Dynamic Interactions Between Hematopoietic Stem and
Progenitor Cells and the Bone Marrow: Current Biology
of Stem Cell Homing and Mobilization 117
Shiri Gur-Cohen, Karin Golan, Kfir Lapid, Jonathan Canaani,
Orit Kollet, and Tsvee Lapidot
Chapter 13
Vascular Growth in Health and Disease 126
Janusz Rak
Chapter 14
Principles of Cytokine Signaling 136
Montaser Shaheen and Hal E. Broxmeyer
Chapter 15
Control of Cell Division 147
William MI. Lee and Chi V. Dang
Chapter 16
Cell Death 158
Nika N. Danial and David M. Hockenbery
PART III
IMMUNOLOGIC BASIS OF HEMATOLOGY 171
Chapter 17
Overview and Compartmentalization of the
Immune System 172
Leland D. Powell, Peter Chung, and Linda G. Baum
Chapter 18
B-Cell Development 182
Kenneth Dorshkind and David J. Rawlings
Chapter 19
T-Cell Immunity 193
Shannon A. Carty, Matthew J. Riese, and Gary A. Koretzky
Chapter 20
Natural Killer Cell Immunity 210
Don M. Benson, Jr., and Michael A. Caligiuri
Chapter 21
Dendritic Cell Biology 217
Olivier Manches, Viswanathan Lakshmanan,
Zbigniew M. Szczepiorkowski, and Nina Bhardwaj
Chapter 22
Complement and Immunoglobulin Biology 228
David E. Isenman, Robert Mandie, and Michael C. Carroll
Chapter 23
Tolerance and Autoimmunity 247
Mark J. Shlomchik
PART V
DISORDERS OF HEMATOPOIETIC
CELL DEVELOPMENT 257
Chapter 24
Biology of Erythropoiesis, Erythroid Differentiation,
and Maturation 258
Thalia Papayannopoulou and Anna Rita Migliaccio
Chapter 25
Granulocytopoiesis and Monocytopoiesis 280
Arati Khanna-Gupta and Nancy Berliner
Chapter 26
Thrombocytopoiesis 292
Alan B. Cantor
Chapter 27
Inherited Forms of Bone Marrow Failure 307
Yİgal Dror and Melvin H. Freedman
Chapter 28
Aplastic Anemia 350
Neal S. Young and Jaroslaw P. Maciejewski
Chapter 29
Paroxysmal Nocturnal Hemoglobinuria 373
Robert A. Brodsky
Chapter 30
Acquired Disorders of Red Cell, White Cell,
and Platelet Production 383
Jaroslaw P. Maciejewski and Ramon V. Tiu
PART V
RED BLOOD CELLS 405
Chapter 31
Pathobiology of the Human Erythrocyte and
Its Hemoglobins 406
Martin H. Steinberg, Edward J. Benz, Jr., Adeboye H. Adewoye,
and Benjamin L. Ebert
Chapter 32
Approach to Anemia in the Adult and Child 418
Peter W. Marks
Chapter 33
Pathophysiology of Iron Homeostasis 427
Gary M. Brittenham
Chapter 34
Disorders of Iron Homeostasis: Iron Deficiency
and Overload 437
Gary M. Brittenham
Chapter 35
Anemia of Chronic Diseases 450
Jane A. Little, Edward J. Benz, Jr., and Lawrence B. Gardner
Chapter 36
Heme Biosynthesis and Its Disorders: Porphyrias and
Sideroblastic Anemias 457
Stephen J. Fuller and James S. Wiley
Chapter 37
Megaloblastic Anemias 473
Asok C. Antony
Chapter 38
Thalassemia Syndromes 505
Patricia J. Giardina and Stefano Rivella
Chapter 39
Pathobiology of Sickle Cell Disease 536
Robert P. Hebbel
Chapter 40
Sickle Cell Disease: Clinical Features and
Management 548
Yogen Saunthararajah and Elliott P. Vichinsky
Chapter 41
Hemoglobin Variants Associated With Hemolytic
Anemia, Altered Oxygen Affinity, and Methemoglobinemias 573
Edward J. Benz, Jr., and Benjamin L. Ebert
Chapter 42
Red Blood Cell Enzymopathies 581
Elizabeth A. Price, Stavroula Otis, and Stanley L. Schrier
Chapter 43
Red Blood Cell Membrane Disorders 592
Patrick G. Gallagher
Chapter 44
Autoimmune Hemolytic Anemia 614
Ulrich Jager and Klaus Lechner
Chapter 45
Extrinsic Nonimmune Hemolytic Anemias 628
Elizabeth A. Price and Stanley L. Schrier
PART VI
NON-MALIGNANT LEUKOCYTES 639
Chapter 46
Neutrophilic Leukocytosis, Neutropenia, Monocytosis,
and Monocytopenia 640
Lawrence Rice and Moonjung Jung
Chapter 47
Lymphocytosis, Lymphocytopenia, Hypergammaglobulinemia,
And Hypogammaglobulinemia 647
Martha P. Mims
Chapter 48
Disorders of Phagocyte Function 655
Mary C. Dinauer and Thomas D. Coates
Chapter 49
Congenital Disorders of Lymphocyte Function 674
Sung- Yun Pai and Luigi D. Notarangelo
Chapter 50
Histiocytic Disorders 686
Michael B. Jordan and Alexandra Hult Filipovich
Chapter 51
Lysosomal Storage Diseases: Perspectives and Principles 701
Edward H. Schuchman and Melissa P. Wasserstein
Chapter 52
Infectious Mononucleosis and Other Epstein-Barr
Virus-Associated Diseases 708
Carl Allen, Cliona M. Rooney, and Stephen Gottschalk
PART VI
HEMATOLOGIC MALIGNANCIES 721
Chapter 53
Progress in the Classification of Myeloid Neoplasms:
Clinical Implications 722
John Anastasi and Ronald Hoffman
Chapter 54
Conventional and Molecular Cytogenetic Basis
of Hematologic Malignancies 728
Vesna Najfeld
Chapter 55
Pharmacology and Molecular Mechanisms
of Antineoplastic Agents for Hematologic
Malignancies 781
Stanton L. Gerson, Paolo F. Caimi, Erica Campagnaro,
Kapil N. Bhalla, Steven Grant, and Richard J. Creger
Chapter 56
Radiation Therapy in the Treatment of Hematologic Malignancies 844
Andrea K. Ng and Peter M. Mauch
Chapter 57
Pathobiology of Acute Myeloid Leukemia 853
Michael Andreeff and Alfonso Quintas-Cardama
Chapter 58
Clinical Manifestations and Treatment of Acute
Myeloid leukemia 863
Stefan Faderl and Hagop M. Kantarjian
Chapter 59
Myelodysplastic Syndromes: Biology and Treatment 882
Daniel J. DeAngelo and Richard M. Stone
Chapter 60
Allogeneic Hematopoietic Stem Cell
Transplantation for Acute Myeloid Leukemia
and Myelodysplastic Syndrome in Adults 904
John Koreth, Joseph H. Antin, and Corey Cutler
Chapter 61
Acute Myeloid leukemia in Children 913
Michael C. Wei, Gary V. Dahl, and Howard J. Weinstein
Chapter 62
Myelodysplastic and Myeloproliferative Neoplasms
in Children 926
Franklin O. Smith and Mignon L. Loh
Chapter 63
Pathobiology of Acute lymphoblastic Leukemia 935
Alejandro Gutierrez, Scott A. Armstrong, and A. Thomas Look
Chapter 64
Clinical Manifestations and Treatment of Acute lymphoblastic
Leukemia in Children 951
Sima Jeha and Ching-Hon Pui
Chapter 65
Acute lymphoblastic leukemia in Adults 960
Nitin Jain, Sandeep Gurbuxani, Charles Rhee, and Wendy Stock
Chapter 66
Chronic Myeloid Leukemia 981
Ravi Bhatia
Chapter 67
The Polycythemias 998
Marina Kremyanskaya, Vesna Najfeld, John Mascarenhas,
and Ronald Hoffman
Chapter 68
Essential Thrombocythemia 1034
Ronald Hoffman, Marina Kremyanskaya, Vesna Najfeld,
and John Mascarenhas
Chapter 69
Primary Myelofibrosis 1053
John Mascarenhas, Vesna Najfeld, Marina Kremyanskaya,
and Ronald Hoffman
Chapter 70
Eosinophilia, Eosinophil-Associated Diseases,
Chronic Eosinophil leukemia, and the
Hypereosinophilic Syndromes 1077
Marina Kremyanskaya, Steven J. Ackerman,
Joseph H. Butterfield, John Mascarenhas, and Ronald Hoffman
Chapter 71
Mast Cells and Systemic Mastocytosis 1095
John Mascarenhas, Animesh Pardanani, Marina Kremyanskaya,
and Ronald Hoffman
Chapter 72
Pathologic Basis for the Classification of Non-Hodgkin
and Hodgkin Lymphomas 1112
Elaine S. Jaffe, Stefania Pittaluga, and John Anastasi
Chapter 73
Origin of Hodgkin Lymphoma 1130
Ralf Küppers
Chapter 74
Hodgkin lymphoma: Clinical Manifestations, Staging,
and Therapy 1138
Dennis A. Eichenauer, Andreas Engert, and Volker Diehl
Chapter 75
Origin of Non-Hodgkin Lymphoma 1157
Matthew S. McKinney and Sandeep S. Dave
Chapter 76
Chronic lymphocytic leukemia 1170
Thomas S. Un, Farrukh T. Awan, and John C. Byrd
Chapter 77
Hairy Cell Leukemia 1192
Farhad Ravandi
Chapter 78
Clinical Manifestations and Treatment of Marginal
Zone Lymphomas (Extranodal/Malt, Splenic,
and Nodal) 1204
Carlos A. Ramos
Chapter 79
Clinical Manifestations, Staging, and Treatment
of Follicular Lymphoma 1215
John G .Gribben
Chapter 80
Mantle Cell Lymphoma 1226
Michael Wang, Liang Zhang, Zhishuo Ou, Madhav V. Desai,
Ken H. Young, Richard E. Champlin, Larry W. Kwak,
and Jorge E. Romaguera
Chapter 81
Diagnosis and Treatment of Diffuse Large B-Cell Lymphoma
and Burkitt Lymphoma 1236
Kieran Dunleavy and Wyndham H. Wilson
Chapter 82
Virus-Associated Lymphoma 1245
Jennifer A. Kanakry and Richard F. Ambinder
Chapter 83
Malignant Lymphomas in Childhood 1255
Kala Y. Kamdar, John T. Sandlund, Jr., and Catherine M. Bollard
Chapter 84
T-Cell Lymphomas 1267
Owen A. O'Connor, Enrica Marchi, Govind Bhagat, Paolo Corradini,
Joan Guitart, Steven T. Rosen, and Timothy M. Kuzel
Chapter 85
Plasma Cell Neoplasms 1303
Nikhil C. Munshi and Sundar Jagannath
Chapter 86
Waldenstrom Macroglobulinemia and Lymphoplasmacytic Lymphoma 1338
Steven P. Treon and Giampaolo Merlini
Chapter 87
Immunoglobulin Light-Chain Amyloidosis (Primary Amyloidosis) 1350
Morie A. Gertz, Francis K. Buadi, Steven R. Zeldenrust, and Suzanne R. Hayman
PART VIII
COMPREHENSIVE CARE OF PATIENTS WITH
HEMATOLOGIC MALIGNANCIES 1375
Chapter 88
Clinical Approach to Infections in the Compromised
Host 1376
Samuel A. Shelburne, Russell E. Lewis, and
Dimitrios P. Kontoyiannis
Chapter 89
Indwelling Access Devices 1391
Franklin W. Huang and Janet L. Abrahm
Chapter 90
Nutritional Issues in Patients With Hematologic Malignancies 1405
Regina S. Cunningham
Chapter 91
Psychosocial Aspects of Hematologic
Disorders 1418
Ruth McCorkle and Elizabeth Cooke
Chapter 92
Pain Management and Antiemetic Therapy
in Hematologic Disorders 1429
Kathy J. Selvaggi, Bridget Fowler Scullion, Craig D. Blinderman,
and Janet L. Abrahm
Chapter 93
Palliative Care 1444
Kristen G. Schaefer, Janet L. Abrahm, and Joanne Wolfe
Chapter 94
Late Complications of Hematologic Diseases and Their
Therapies 1452
Wendy Landier and Smita Bhatia
PART IX
CELL-BASED THERAPIES 1469
Chapter 95
Overview and Historical Perspective of Current
Cell-Based Therapies 1470
Leslie E. Silberstein and Helen E. Heslop
Chapter 96
Practical Aspects of Hematologic Stem Cell
Harvesting and Mobilization 1472
Scoff D. Rowley and Michele L. Donato
Chapter 97
Preclinical Process of Cell-Based Therapies 1486
Robert Lindblad, Deborah Wood, Traci Heath Mondora,
and Leslie E. Silberstein
Chapter 98
Graft Engineering and Cell Processing 1492
Adrian P. Gee
Chapter 99
Principles of Cell-Based Genetic
Therapies 1503
David A. Williams
Chapter 100
Mesenchymal Stromal Cells 1513
Edwin M. Horwitz
Chapter 101
T-Cell Therapy of Hematologic Diseases 1521
Gianpietra Doffi and Malcolm K. Brenner
Chapter 102
Natural Killer Cell-Based Therapies 1527
Sarah Cooley, Michael R. Verneris, and Jeffrey S. Miller
Chapter 103
Dendritic Cell Therapies 1534
Karolina Palucka and Jacques Banchereau
PART X
TRANSPLANTATION 1541
Chapter 104
Overview of Hematopoietic Stem Cell
Transplantation 1542
Helen E. Heslop
Chapter 105
Indications and Outcome of Allogeneic
Hematopoietic Cell Transplantation for
Hematologic Malignancies in Adults 1546
Parameswaran Hari and Mary Horowitz
Chapter 106
Unrelated Donor Hematopoietic Cell Transplantation 1557
Effie W. Petersdorf and Claudio Anasetti
Chapter 107
Haploidentical Hematopoietic Cell
Transplantation 1569
Bimalangshu R. Deyand Thomas R. Spitzer
Chapter 108
Unrelated Donor Cord Blood Transplantation for Hematologic Malignancies 1580
Doris M. Ponce and Juliet N. Barker
Chapter 109
Graft-Versus-Host Disease and Graft-Versus-Leukemia Responses 1592
Pavan Reddy and James L.M. Ferrara
Chapter 110
Complications After Hematopoietic Stem Cell Transplantation 1612
Navneet S. Majhail and Daniel J. Weisdorf
PART XI
TRANSFUSION MEDICINE 1627
Chapter 111
Human Blood Group Antigens and Antibodies 1628
Connie M. Westhoff, Jill R. Storry, and Beth H. Shaz
Chapter 112
Principles of Red Blood Cell Transfusion 1642
Melissa M. Cushing and Paul M. Ness
Chapter 113
Principles of Platelet Transfusion Therapy 1653
Richard M. Kaufman
Chapter 114
Human Leukocyte Antigen and Human Neutrophil
Antigen Systems 1659
Ena Wang, Sharon Adams, David F. Stroncek,
and Francesco M. Marincola
Chapter 115
Principles of Neutrophil (Granulocyte)
Transfusions 1676
Ronald G. Strauss
Chapter 116
Principles of Plasma Transfusion: Plasma, Cryoprecipitate,
Albumin, and Immunoglobulins 1683
Matthew S. Karafin, Christopher D. Hillyer, and Beth H. Shaz
Chapter 117
Preparation of Plasma-Derived and Recombinant Human
Plasma Proteins 1695
David B. Clark
Chapter 118
Transfusion Therapy for Coagulation Factor
Deficiencies 1705
Elizabeth Roman, Peter J. Larson, and Catherine S. Manno
Chapter 119
Hemapheresis 1716
Diarmaid Q Donghaile and Harvey G. Klein
Chapter 120
Transfusion Reactions to Blood and Cell Therapy Products 1727
Jacquelyn D. Choate, Robert W. Maitta, Christopher A. Tormey,
YanYun Wu, and Edward L. Snyder
Chapter 121
Transfusion-Transmitted Diseases 1738
Louis M. Katz and Jay E. Menitove
Chapter 122
Transfusion Medicine in Hematopoietic Stem Cell
and Solid Organ Transplantation 1755
Richard M. Kaufman
Chapter 123
Pediatric Transfusion Medicine 1765
Cassandra Josephson and Steven R. Sloan
PART XII
HEMOSTASIS AND THROMBOSIS 1773
Chapter 124
Overview of Hemostasis and Thrombosis 1774
Jeffrey I. Weitz
Chapter 125
The Blood Vessel Wall 1784
Aly Karsan and John M. Harlan
Chapter 126
Megakaryocyte and Platelet Structure 1797
Joseph E. Italiano, Jr., and John H. Hartwig
Chapter 127
Molecular Basis for Platelet Function 1809
Charles S. Abrams and Edward F. Plow
Chapter 128
Molecular Basis of Blood Coagulation 1821
Kathleen Brummel-Ziedins and Kenneth G. Mann
Chapter 129
Regulatory Mechanisms in Hemostasis 1842
Charles T. Esmon and Naomi L. Esmon
Chapter 130
Clinical Approach to the Patient With Bleeding
or Bruising 1847
Catherine P.M. Hayward
Chapter 131
Laboratory Evaluation of Hemostatic and
Thrombotic Disorders 1857
Alvin H. Schmaier
Chapter 132
Acquired Disorders of Platelet Function 1867
Reyhan Diz-Küçükkaya and Jose A. Lopez
Chapter 133
Diseases of Platelet Number: Immune
Thrombocytopenia, Neonatal
Alloimmune Thrombocytopenia, and
Posttransfusion Purpura 1883
Donald M. Arnold, Christopher Patriquin, Lisa J. Toltl
Ishac Nazi, James Smith, and John Kelton
Chapter 134
Thrombocytopenia Caused by Platelet Destruction,
Hypersplenism, or Hemodilution 1895
Theodore E. Warkentin
Chapter 135
Heparin-Induced Thrombocytopenia 1913
Theodore E. Warkentin
Chapter 136
Thrombotic Thrombocytopenic Purpura and the
Hemolytic Uremic Syndrome 1925
Keith R. McCrae, J. Evan Sadler, and Douglas B. Cines
Chapter 137
Hemophilia A and B 1940
Manuel Carcao, Paul Moorehead, and David Lillicrap
Chapter 138
Inhibitors in Hemophilia A and B 1961
Guglielmo Mariani, Barbara A. Konkle, and Craig M. Kessler
Chapter 139
Rare Coagulation Factor Deficiencies 1971
David Gailani and Anne T. Neff
Chapter 140
Structure, Biology, and Genetics of von
Willebrand Factor 1987
Paula James and Natalia Rydz
Chapter 141
Disseminated Intravascular Coagulation 2001
Marcel Levi
Chapter 142
Hypercoagulable States 2013
Julia A. Anderson and Jeffrey I. Weitz
Chapter 143
Antiphospholipid Syndrome 2025
Jacob H. Rand and Lucia R. Wolgast
Chapter 144
Venous Thromboembolism 2039
Wendy Lim
Chapter 145
Mechanical Interventions in Arterial and Venous
Thrombosis 2048
Steven Sauk and Suresh Vedantham
Chapter 146
Atherothrombosis 2057
Roy L. Silverstein
Chapter 147
Stroke 2067
Michelle Canavan, Emer McGrath, and Martin O'Donnell
Chapter 148
Acute Coronary Syndromes 2076
John W. Eikelboom and Jeffrey I. Weitz
Chapter 149
Atrial Fibrillation 2086
Stavros Apostolakis and Gregory Y.H Lip
Chapter 150
Peripheral Artery Disease 2094
Reena L. Pande and Mark A. Creager
Chapter 151
Antithrombotic Drugs 2102
Jeffrey I. Weitz
Chapter 152
Disorders of Coagulation in the Neonate 2120
Christine A. Macartney, Nethnapha Paredes,
and Anthony K. C. Chan
PART XIII
CONSULTATIVE HEMATOLOGY 2131
Chapter 153
Hematologic Changes in Pregnancy 2132
Caroline Cromwell
Chapter 154
Hematologic Manifestations of Childhood Illness 2144
Arthur Kim Ritchey, Frank G. Keller, and Sarah H. O'Brien
Chapter 155
Hematologic Manifestations of Liver Disease 2167
Andrea Lee and Wendy Lim
Chapter 156
Hematologic Manifestations of Systemic Disease: Renal Disease 2173
Peter W. Marks, Rachel Rosovsky, and Edward J. Benz, Jr.
Chapter 157
Hematologic Manifestations of Cancer 2176
Gerald A. Soft, David L. Green, and Lawrence B. Gardner
Chapter 158
Integrative Therapies in Patients With Hematologic Diseases 2183
David S. Rosenthal, Kara M. Kelly, and Donald I. Abrams
Chapter 159
Hematologic Manifestations of HIV/AIDS 2191
Howard A. Liebman and Anil Tulpule
Chapter 160
Hematologic Aspects of Parasitic Diseases 2208
David J. Roberts
Chapter 161
Hematologic Problems in the Surgical Patient:
Bleeding and Thrombosis 2234
Mark T. Reding and Nigel S. Key
Chapter 162
The Spleen and Its Disorders 2252
Nathan T. Connell, Susan B. Shurin, and Fred J. Schiffman
Chapter 163
Hematology in Aging 2266
Andrew S. Artz and William B. Ershler
Chapter 164 (online only)
Resources for the Hematologist: Interpretive
Comments and Selected Reference Values for
Neonatal, Pediatric, and Adult Populations
Andrea M. Sheehan and Donald L. Ye
Inherited disorders of platelets membrane glycoprotein disorders
İstanbul Bilim Üniversitesi, Tıp Fakültesi.Platelet membrane glycoproteins play a key role in hemostasis and thrombosis. Although disorders of platelet membrane glycoproteins are rare, their effects on the lives of those affected are very important. Severe deficiencies manifest themselves early during childhood with mucocutaneous bleeding. Mild deficiencies may not be diagnosed until adulthood or until the hemostatic system is stressed by surgery or trauma. The diagnosis of these disorders requires detailed laboratory investigation. Management of bleeding in patients with inherited platelet disorders requires both preventive measures and the treatment of individual bleeding episodes according to severity. The study of platelet membrane disorders also has yielded important insights into the functions of affected proteins, information that has produced some of the most successful antithrombotic drugs currently in use
High risk of systemic lupus erythematosus development in patients with ITP: antiphospholipid syndrome is also a concern.
Correction: Inhibition of IGF-1R Prevents Ionizing Radiation-Induced Primary Endothelial Cell Senescence
İstanbul Bilim Üniversitesi, Tıp Fakültesi.Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and fetal mortality. Thrombotic microangiopathy (TMA) is an important histological finding in catastrophicAPS (CAPS) and in APS patients with nephropathy. Analysis of familial thrombotic thrombocytopenic purpura patients showed that there are mutations in the ADAMTS-13 gene that lead to functional defects in the ADAMTS-13 enzyme. The aim of this study was to investigate the prevalence of the aforementioned mutations in APS, as well as to evaluate the level and activity of the ADAMTS-13 enzyme in patients with APS. C365del, Q449stop codon, P475S, and C508Y mutations were analyzed in APS patients. Transcriptions were analyzed using real-time PCR, and the level and activity of ADAMTS-13 were analyzed via fluorogenic assay. None of the mutations tested were present in the patient or control groups. The level of ADAMTS-13 mRNA in the patient group was 50% lower than that in the control group. Although a significant difference in ADAMTS-13 activity was not observed between the patient and control groups, a significant association was observed with the level of ADAMTS-13 (p<0.0001). The level and activity of ADAMTS-13 were not associated with thrombotic complications, thrombocytopenia, or pregnancy complications in the patients with APS.Antifosfolipid sendrom (AFS), tekrarlayan tromboz ve fetal kayıplar ile karakterize olan otoimmün bir hastalıktır. Trombotik mikroanjiyopati (TMA), katastrofik AFS ve nefropatinin eşlik ettiği AFS olgularında önemli bir histolojik bulgudur. Ailesel trombotik trombositopenik purpura olgularının incelenmesi sonucu, ADAMTS-13 enziminin işlev bozukluğuna yol açan mutasyonların varlığını göstermiştir. Bu çalışmada ADAMTS-13 mutasyonları ile, enzimin aktivite ve miktarının AFS’ye katkısının olup olmadığının araştırılması amaçlanmıştır. Bu kapsamda C365del, Q449stop kodonu, P475S ve C508Y mutasyonları analiz edildi. Transkripsiyon aşaması gerçek zamanlı polimeraz zincir reaksiyonu, enzimin aktivite ve miktarı ise florojenik bir yöntem ile incelendi. Varlığı araştırılan mutasyonlar kontrol ve hasta gruplarında tespit edilmedi. ADAMTS-13 mRNA ve protein miktarının hasta grubunda kontrol grubundaki düzeyin yarısı kadar olduğu saptandı. (p<0.0001). Enzim aktivitesi açısından ise iki grup arasında istatistik olarak anlamlı bir fark gözlenmedi. Sonuç olarak, ADAMTS-13 enzim aktivitesi ve miktarının AFS hastalarında trombotik komplikasyonlar, trombositopeni ve gebelik komplikasyonları ile ilişkili olmadığı saptandı
Simultaneous occurrence of immune tyhrombocytopenic purpura and bullous pemphigoid: A case report
Otoimmün hastalıklar, immun toleransın bozulması sonucu gelişirler. Herhangi bir otoimmun hastalığı olan bir kişide, beraberinde başka bir otoimmun hastalığın ortaya çıkma ihtimali yüksektir. Bu yazıda idiyopatik otoimmun trombositopeni ve büllöz pemfigoidin birlikte ortaya çıktığı bir olgu sunulmaktadır. Kırkbeş yaşındaki kadın hasta bir yıldan beri ellerde kaşıntı ve su toplanmaları, 6 aydır ise hipermenore ve küçük travmalarla kolay morarma şikayetleri ile başvurdu. Fizik muayene, periferik yayma ve kemik iliği aspirasyonu yapılıp trombositopeni yapan diğer nedenler dışlanarak idiyopatik otoimmun trombositopeni tanısı; deri biyopsisi ve bazal membranda antikor birikimi ile de büllöz pemfigoid tanısı konuldu. Her iki hastalık da steroid tedavisine iyi cevap verdi.Otoimmune diseases develop due to disordered regulation in immune tolerence. In a patient who had developed disorder an autoimmune disorder, the chance of developing another autoimmune disorder is higher than in normal population. In this paper we report a case who had both autoimmune idiopathic thrombocytopenic purpura and bullous pemphigoid simultaneously. A 45-year-old female was admitted to the hospital with complaints of itching and blistering on hands, easy bruising, and hypermenorrhea. Idiopathic thrombocytopenic purpura was diagnosed by medical history, physical examination, peripheral blood smear and bone marrow aspiration, and bullous pemphigoid was confirmed by skin biopsy and immunoglobulin deposition in basal membrane of epidermis. Both diseases responded well to steroid treatment
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