HODGKIN'S DISEASE: Reed-Sternberg cells. Left/Right: Bone marrow aspirate. (Wright-Giemsa, original magnifi­cation X 400). Right/Left: Lymph node biopsy. (Hematoxylin and eosin, original magnification X 400). (Courtesy of Powers Peterson).

Diseases of the Lymphatic System Diagnosis and Therapy

Edited by David W. Molander Assistant Attending Physician (Medicine), New York Hospital, New York City Associate Attending Physician (Hematology/Lymphoma), Memorial Hospital, New York City Clinical Assistant Professor of Medicine, Cornell University, Medical College, New York City

With Contributions by: Zalmen A. Arlin Donald Armstrong Edward H. Cooper Susanna Cunningham-Rundles Bruno Damascelli Richard D. Fernandez Merril J. Gersten Robert S. Grayson Herbert Hansen D. Geraint James W. Jones Williams Sanford Jay Kempin Benjamin Koziner Ian Magrath Martin H. Markowitz Craig E. Metroka Anne Moore Janet A. Mouradian Renato Musumeci Gorm Pallesen Joseph A. Sonnabend David J. Straus David J. Wolf Lore Zech

Springer-Verlag New York Berlin Heidelberg Tokyo

David W. Molander, M.D. 235 East 67th Street, Suite 205, New York, NY 10021, U.S.A.

Assistant Attending Physician (Medicine), New York Hospital, New York City.

Associate Attending Physician (Hematology/Lymphoma), Memorial Hospital, New York City.

Clinical Assistant Professor of Medicine, Cornell University College of Medicine, New York City.

Consultant (Oncology), Tumor Board, Valley Hospital, Ridgewood, New Jersey.

Consultant (Oncology), Tumor Board, Cape Cod Hospital, Hyannis, Massachusetts.

With 111 Illustrations

Library of Congress Cataloging in Publication Data Main entry under title: Diseases of the lymphatic system.

Includes index. 1. Lymphatics-Cancer. 2. Lymphoma. I. Molander,

David W. II. Arlin, Zalmen A. (Zalmen Amos), 1946-[DNLM: 1. Lymphatic disease-Diagnosis. 2. Lymphatic diseases-Therapy. WH 700 D611] RC280.L9D57 1983 616.99' 446 83-12537

© 1984 by Springer-Verlag New York Inc. Softcover reprint ofthe hardcover 1st edition 1984

All rights reserved. No part of this book may be translated or reproduced in any form without written permission from Springer-Verlag, 175 Fifth Avenue, New York, New York 10010, U.S.A. The use of general descriptive names, trade names, trademarks, etc., in this publication, even if the former are not especially identified, is not to be taken as a sign that such names, as understood by the Trade Marks and Merchandise Marks Act, may accordingly be used freely by anyone.

Typeset by Kingsport Press, Kingsport, Tennessee.

9 8 7 6 5 432 1

ISBN-13: 978-1-4612-9733-8 e-ISBN-13: 978-1-4612-5198-9 DOl: 10.1007/978-1-4612-5198-9

Contents

Preface

Contributors

Plates

Abbreviations

1 Hodgkin's Disease Benjamin Koziner

2 Non-Hodgkin's Lymphoma David J. Wolf

3 Angioimmunoblastic Lymphadenopathy: A Clinical View David J. Straus

vii

ix

xii

xvii

1

27

41

4 Acute and Chronic Lymphocytic Leukemia 51 Martin H. Markowitz, Janet A. Mouradian, and Anne Moore

5 Philadelphia Chromosome-Positive Chronic Myelogenous Leukemia: Diagnosis and Treatment 71 Zalmen A. Arlin

6 Burkitt's Lymphoma: Diagnostic and Taxonomic Aspects 89 Gorm Pallesen

7 Burkitt's Lymphoma: Clinical Aspects and Treatment 103 Ian Magrath

8 Sarcoidosis of the Lymph Nodes 140 D. Geraint James and W. Jones Williams

v

VI Contents

9 Biochemical and Immunologic Disorders in Diseases of the Lymphoid Tissue 157 Edward H. Cooper

10 Hematologic Aspects of Malignant Lymphoproliferative Disease 181 Sanford Jay Kempin

11 Cell Kinetics and the Classification of Lymphocytic Lymphomas 208 Herbert Hansen

12 Infections in Lymphoma Patients 228 Merril Gersten and Donald Armstrong

13 Radiologic Approach to Diseases of the Lymph Nodes 245 Renato Musumeci and Bruno Damascelli

14 Chromosome Abnormalities in Malignant Diseases of the Lymphatic System 269 Lore Zech

15 Generalized Lymphadenopathy in Homosexual Men 296 Craig E. Metroka, Susanna Cunningham-Rundles, Joseph A. Sonnabend, Richard D. Fernandez, and Janet A. Mouradian

16 Psychological Care of Patients with Cancer 306 Robert S. Grayson

Index 333

Preface

This volume presents a practical approach for clinicians who work with patients afflicted with diseases of the lymphoid system and bone marrow. Associated biochemical, hematological, and chromosomal abnormalities, as well as infectious complications, are covered because of their importance in managing patients with lymphatic diseases. In addition, cell kinetics and the classification of lym­phocytic lymphomas are included because familiarity with these subjects leads to a better understanding of the physiology of the disease.

Radiological techniques that aid the clinician in the diagnosis and management of diseases of the lymphoid tissue are discussed. The explosive interest in patients with generalized acquired (auto) immune deficiency syndrome (AIDS) prompted the inclusion of a chapter exclusively devoted to this topic. Finally, the chapter dealing with psychological aspects of patients with cancer allows the reader to become familiar with useful techniques.

Distilled from a vast array of data and representing the most up-to-date material available, this volume is intended to be of interest to internists, oncolo­gists, hematologists, radiotherapists, nurses, and anyone in an associated pa­ramedical field who may be involved in the care of patients with diseases of the lymphatic system.

Acknowledgements: I am indebted to the following for their help in making this volume possible: Mr. Rudy De Angelo and the Hudson County Chapter of the Pack Medical Foundation, Mrs. Louis Berkoff, Mrs. Frances Goldman, The Pack Medical Foundation, Mr. and Mrs. Julius Schure and Mr. Richard Schure.

DAVID W. MOLANDER, M.D.

vii

Contributors

Zalmen A. Arlin, M.D., Chief, Division of Neoplastic Diseases, Professor of Medi­cine, New York Medical College, Valhalla, NY 10595, U.S.A.

Donald Armstrong, M.D., Chief, Infectious Diseases Service, Director, Microbiol­ogy Laboratory, Memorial Hospital, Professor of Medicine, Cornell University Medical College, 1275 York Avenue, New York, NY 10021, U.S.A.

Edward H. Cooper, M.D., Dsc, F.R.C.P., Mackintosh Professor of Cancer Re­search, The Unit for Cancer Research, University of Leeds, Leeds, England.

Susanna Cunningham-Rundles, Ph.D., Laboratory of Clinical Immunology, Memorial Hospital, Research Associate, Sloan Kettering Institute for Cancer Re­search, 1275 York Avenue, New York, NY 10021, U.S.A.

Bruno Damascelli, M.D., Clinical Assistant Professor of Diagnostic Radiology, National Cancer Institue, Special Procedures Section, Milan, Italy.

Richard D. Fernandez, M.D., Fellow in Surgical Pathology, Cornell University Medical Center, 525 East 68th Street, New York, NY 10021, U.S.A.

Merril J. Gersten, M.D., Developmental Biology Laboratory, Salk Institute, San Diego, CA 92037, U.S.A.

Robert S. Grayson, M.D., Clinical Associate Professor of Psychiatry, Cornell University Medical College, New York, NY 10021, U.S.A.; Consultation-Liaison Psychiatrist, Oncology Service, New York Hospital, 525 East 68th Street, New York, NY 10021, U.S.A.

Herbert Hansen, M.D., Assistant Attending Physician, Memorial Hospital, Assis­tant Professor of Clinical Medicine, Cornell University Medical College, 1275 York Avenue, New York, NY 10021, U.S.A.

D. Geraint James, M.D., F.R.C.P., Dean and Senior Physician, Royal Northern Hospital, London, England; Consultant Opthalmic Physician, St. Thomas' Hospi-

ix

x

tal, London, England; Consulting Physician to Royal Navy; Adjunct Professor of Medicine and Epidemiology, School of Medicine, University of Miami, Miami, FL 33136, U.S.A.

W. Jones Williams, M.D., F.R.C.P., F.R.C. Path., Clinical Teacher, Welsh Na­tional School of Medicine, Llandough Hospital, Penarth S, Glam, United King­dom.

Sanford Jay Kempin, M.D., Memorial Hospital, Associate Professor of Clinical Medicine, Cornell University Medical College, New York, NY 10021, U.S.A.

Benjamin Koziner, M.D., Associate Attending Physician [Hematology/Lym­phoma], Memorial Hospital, Associate Professor of Clinical Medicine, Cornell University Medical College, 1275 York Avenue, New York, NY 10021, U.S.A.

Ian Magrath, M.B.B.S., M.R.C.P., Visiting Scientist and Attending Physician, Pediatric Branch, National Cancer Institute, National Institutes of Health, Build­ing 10, Room 13N240, Bethesda, Maryland 20205, U.S.A.

Martin H. Markowitz, M.D., Fellow, Division of Hematology/Oncology, Depart­ment of Medicine, Cornell University Medical College, New York Hospital, 525 East 68th Street, New York, NY 10021, U.S.A.

Craig E. Metroka, M.D., Division of Hematology/Oncology, Department of Medi­cine, Cornell University Medical College, 525 East 68th Street, New York, NY 10021, U.S.A.

Anne Moore, M.D., Associate Professor of Clinical Medicine, Department of Medicine, Cornell University Medical College, New York Hospital, 525 East 68th Street, New York, NY 10021, U.S.A.

Janet A. Mouradian, M.D., Clinical Associate Professor of Pathology, Cornell University Medical College, New York Hospital, 525 East 68th Street, New York, NY 10021, U.S.A.

Renato Musumeci, M.D., Vice Director of Diagnostic Radiology, Chief Section of Urologic Radiology and Lymphology, National Cancer Institute of Milan, Associate Professor of Radiology, University of Milan, Italy.

Gorm Pallesen, M.D., University Institute of Pathology, Aarhus Kommunehospi­tal, University of Aarhus, DK-8000 Aarhus C, Denmark.

Joseph A. Sonnabend, M.D., 49 West 12th Street, New York, NY 1O01l, U.S.A.

David J. Straus, M.D., F.A.C.P., Associate Attending Physician [Hematology/ Lymphoma], Memorial Hospital, Associate Professor of Clinical Medicine, Cornell University Medical College, 1275 York Avenue, New York, NY 10021, U.S.A.

David J. Wolf, M.D., Clinical Instructor of Medicine, Division of Hematology/ Oncology, Cornell University Medical College, New York Hospital, 525 East 68th Street, New York, NY 10021, U.S.A.

Lore Zech, Ph.D., Associate Professor, Department of Medical Cell Genetics, Medical Nobel Institute, Karolinska Institute, Stockholm, Sweden.

Contributors

Contributors Xl

Photograph Contributors

Daniel A. Philippa, M.D., Associate Attending Pathologist, Memorial Hospital, Sloan-Kettering Cancer Center, New York, NY 10021; Assistant Professor of Pa­thology, Cornell University Medical College, New York, NY 10021, U.S.A.

Powers Peterson, M.D., Assistant Attending Pathologist, The New York Hospital, Assistant Professor of Pathology, Cornell University Medical College, 525 East 68th Street, New York, NY 10021, U.S.A.

Plates

Ten examples of the histology of malignant Lymphoma, including Hodgkin's and non­Hodgkin's lymphoma. Sections hematoxylin and eosin stain. (Courtesy of Daniel A. Filippa, M.D.)

L Lukes classification. WF working formulation classification Rap. Rappaport classification Kiel Kiel classification

1 poorly differentiated lymphocytic type, nodular (Rap.) low grade, centroblastic/ centrocytic, follicular (Kiel) FCC, small cleaved cell, follicular (L) low grade, follicular, small cleaved cell (WF) (a-40X; b-430X)

2 well differentiated lymphocytic type, diffuse (Rap.) low grade, lymphocytic (B-CLL) (Kiel) small lymphocyte (L) low grade, small lymphocytic consistent with CLL (WF) (430X)

3 mixed lymphocytic and histiocytic type, diffuse (Rap.) low grade, centroblastic/centrocytic, diffuse (Kiel) FCC, small cleaved cell, diffuse (L) intermediate grade, diffuse, mixed, small cleaved and large cell (WF) (430X)

4 histiocytic type, diffuse (Rap.) low grade, centroblastic/centrocytic, diffuse (Kiel) FCC, large cleaved cell, diffuse (L) intermediate grade, diffuse, large cell, cleaved (WF) (430X)

5 histiocytic type, diffuse (Rap.) high grade, immunoblastic (Kiel)

xii

FCC, large non-cleaved cell, diffuse (L) intermediate grade, diffuse, large cell, non-cleaved (WF) (430X)

xiii

6 histiocytic type, diffuse (Rap.) high grade, immunoblastic, plasmacytic (Kiel) immunoblastic sarcoma (L) high grade, immunoblastic, plasmacytic (WF) (430X)

7 lymphoblastic, convoluted (Rap.) high grade, lymphoblastic convoluted (Kiel) convoluted lymphocytic (L) high grade lymphoblastic, convoluted cell (WF) (430X)

8 undifferentiated, diffuse (Rap.) high grade lymphoblastic, B cell type (Kiel) FCC, small non-cleaved cell (L) high grade, diffuse small non-cleaved cell (WF) (430X)

9 mixed lymphocytic and histiocytic type, diffuse (Rap.) ? T -zone lymphoma (Kiel) ? immunoblastic sarcoma, T type (L) high-grade immunoblastic, polymorphous (WF) (430X)

10 Hodgkin's disease (430X)

xiv

Abbreviations

AA ABP ACOMLA AGP APRP BACOP

BCND BCOP 132M CAT CCSG CHOP CHOP-BLEO C-MOPP COPP CNS COM CMV COMA COMLA COP + Ara C

COP-BLAM

COP CVP CRP CSF CSF CT DIC EORTC

ESR HCL HDL HOP

amyloid A Adriamycin, bleomycin, prednisone COMLA and adriamycin acid arglycoprotein acute phase reactant proteins Bleomycin, adriamycin, cyclophosphamide, vincristine, prednisone 1,3-bis (2 chloroethyl) 1 nitrosurea BCND, cyclophosphamide, vincristine, prednisone 132-microglobulin Cytosine arabinoside, adriamycin, 6-thioguanine Children's Cancer Study Group Cyclophosphamide, adriamycin, vincristine, prednisone CHOP + bleomycin Cyclophosphamide, vincristine, procarbazine, prednisone central nervous system cyclophosphamide, vincristine, methotrexate cytomegalovirus Cyclophosphamide, vincristine, methotrexate, cytosine arabinoside Cyclophosphamide, vincristine, prednisone, cytosine arabinoside Cyclophosphamide, vincristine, prednisone, bleomycin, adriamycin, matulane Cyclophosphamide, vincristine, prednisone C-reactive protein cerebrospinal fluid colony-stimulating factor computerized tomography disseminated intravascular coagulation European Organization for Research on the Treatment of Cancer erythrocyte sedimentation rate hairy cell leukemia high-density lipoprotein Adriamycin, vincristine, prednisone

xvii

xviii

LACE

LDH LI L2

MBA COD MBA COP MCV NCI NHL PWM SAA SACE SCE TACE TRIKE

bronchoalveolar lavage fluid levels of angiotensin-converting enzyme lactate dehydrogenase labeling index Cyclophosphamide, prednisone, vincristine, daunorubicin, cytosine arabinoside, thioguanine, L-asperaginase, BCNU MBACOP, Dexamethasone replaces prednisone BACOP and methotrexate modal cell volume National Cancer Institute non-Hodgkin's lymphoma pokeweed mitogen serum amyloid A serum levels of angiotensin-converting enzyme sister chromatid exchanges tissue levels of angiotensin-converting enzyme cyclophosphamide, vincristine, methotrexate, cytosine arabinoside

Abbreviations