Immunology: Clinical Case Studies and Disease Pathophysiology

Preface, ix

Contributors, xi

Index of Lessons in Normal Immunity, xiii

INTRODUCTION TO CASE STUDIES: A GUIDE TO USING THIS BOOK, 1

Unit I IMMUNODEFICIENCY DISEASES, 3

INTRODUCTION: ORGANIZATION OF THE IMMUNE SYSTEM AND EVALUATION OF THE IMMUNODEFICIENT PATIENT, 5

1 X-LINKED AGAMMAGLOBULINEMIA (BRUTON’S AGAMMAGLOBULINEMIA), 15

2 SEVERE COMBINED IMMUNODEFICIENCY DISEASE, 27

3 DiGEORGE SYNDROME (CONGENITAL THYMIC APLASIA), 41

4 HYPER-IgM SYNDROME, 53

5 COMMON VARIABLE IMMUNODEFICIENCY DISEASE, 67

6 AUTOIMMUNE LYMPHOPROLIFERATIVE SYNDROME (ALPS), 81

7 X-LINKED LYMPHOPROLIFERATIVE SYNDROME, 91

8 HEREDITARY ANGIOEDEMA, 101

9 ACQUIRED IMMUNODEFICIENCY SYNDROME, 111

Unit II IMMEDIATE HYPERSENSITIVITY AND MAST CELL DISORDERS, 125

INTRODUCTION: WHEN ANTIGENS BECOME ALLERGENS, 127

10 MASTOCYTOSIS, 129

11 ANAPHYLAXIS, 137

12 ASTHMA, 147

13 THE VASCULITIDES, 161

Unit III AUTOIMMUNE DISORDERS, 173

INTRODUCTION: OVERVIEW OF AUTOIMMUNE DISORDERS AND MECHANISMS OF TOLERANCE, 175

14 MYASTHENIA GRAVIS, 183

15 MULTIPLE SCLEROSIS, 195

16 DIABETES MELLITUS TYPE 1, 207

17 CELIAC DISEASE, 219

18 INFLAMMATORY BOWEL DISEASE, 231

19 PSORIASIS, 245

20 IgA NEPHROPATHY AND KIDNEY TRANSPLANTATION, 255

21 RHEUMATOID ARTHRITIS, 269

22 SYSTEMIC LUPUS ERYTHEMATOSUS, 283

23 SCLERODERMA, 299

Unit IV MALIGNANCIES OF THE LYMPHOID SYSTEM, 309

INTRODUCTION: CLASSIFICATION OF LYMPHOID MALIGNANCIES, 311

24 CHRONIC LYMPHOCYTIC LEUKEMIA/SMALL LYMPHOCYTIC LYMPHOMA, 317

25 FOLLICULAR LYMPHOMA, 327

26 PLASMA CELL NEOPLASMS, 337

Unit V 349

LABORATORY TESTS USED IN THE WORKUP OF PATIENTS WITH IMMUNOLOGIC DISEASES, 351

Index, 369