Acquisitions Editor: Sonya Seigafuse Product Manager: Kerry Barrett Vendor Manager: Bridgett Dougherty Marketing Manager: Kimberly Schonberger Manufacturing Manager: Ben Rivera Design Coordinator: Stephen Druding Editorial Coordinator: Katie Sharp Production Service: Aptara, Inc © 2012 by Department of Medicine, Washington University School of Medicine Printed in China All rights reserved This book is protected by copyright No part of this book may be reproduced in any form by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews Materials appearing in this book prepared by individuals as part of their official duties as U.S government employees are not covered by the above-mentioned copyright Library of Congress Cataloging-in-Publication Data The Washington manual hematology and oncology subspecialty consult — 3rd ed / editors, Amanda Cashen, Brian A Van Tine p ; cm — (Washington manual subspecialty consult series) Hematology and oncology subspecialty consult Includes bibliographical references and index ISBN 978-1-4511-1424-9 (alk paper) — ISBN 1-4511-1424-9 (alk paper) I Cashen, Amanda II Van Tine, Brian A III Title: Hematology and oncology subspecialty consult IV Series: Washington manual subspecialty consult series [DNLM: Hematologic Diseases—Handbooks Diagnosis, Differential — Handbooks Drug Therapy—methods—Handbooks Neoplasms— Handbooks WH 39] 616.1’5—dc23 2011046133 The Washington Manual™ is an intent-to-use mark belonging to Washington University in St Louis to which international legal protection applies The mark is used in this publication by LWW under license from Washington University Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication Application of the information in a particular situation remains the professional responsibility of the practitioner The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with current recommendations and practice at the time of publication However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions This is particularly important when the recommended agent is a new or infrequently employed drug Some drugs and medical devices presented in the publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice To purchase additional copies of this book, call our customer service department at (800) 638-3030 or fax orders to (301) 223-2320 International customers should call (301) 223-2300 Visit Lippincott Williams & Wilkins on the Internet: at LWW.com Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to pm, EST 10 5 George Ansstas, MD Clinical Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Michael Ansstas, MD Resident Physician Department of Medicine Saint Louis University School of Medicine St Louis, Missouri Kristan M Augustin, PharmD, BCOP Clinical Pharmacist BMT/Leukemia Department of Pharmacy Barnes-Jewish Hospital St Louis, Missouri Leigh M Boehmer, PharmD, BCOP Clinical Pharmacist Department of Pharmacy Barnes-Jewish Hospital St Louis, Missouri Sara K Butler, PharmD, BCOP, BCPS Clinical Pharmacist Department of Pharmacy Barnes-Jewish Hospital St Louis, Missouri Amanda Cashen, MD Assistant Professor of Medicine Section of Leukemia and Stem Cell Transplantation Washington University School of Medicine St Louis, Missouri Yee Hong Chia, MD Clinical Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Kim French, APRN-BC Division of Hematology Washington University School of Medicine St Louis, Missouri Armin Ghobadi Clinical Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Andrea R Hagemann, MD Assistant Professor Department of Obstetrics and Gynecology Washington University School of Medicine St Louis, Missouri Lindsay M Hladnik, PharmD, BCOP Clinical Pharmacist Department of Pharmacy Barnes-Jewish Hospital St Louis, Missouri Xiaoyi Hu, MD, PhD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Amie Jackson, MD Resident Department of Medicine Washington University School of Medicine St Louis, Missouri Ronald Jackups, MD Chief Resident Laboratory and Genomic Medicine Washington University School of Medicine St Louis, Missouri Kian-Huat Lim, MD, PhD Fellow Medical Oncology National Cancer Institute/National Institutes of Health Bethesda, Maryland Daniel J Ma, MD Resident Physician Department of Radiation Oncology Washington University School of Medicine St Louis, Missouri Paul Mehan, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Ali McBride, PharmD, BCPS Clinical Pharmacist Department of Pharmacy Barnes-Jewish Hospital St Louis, Missouri Gregory H Miday, MD Resident Department of Medicine Washington University School of Medicine St Louis, Missouri Gayathri Nagaraj, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Parag J Parikh, MD Assistant Professor Department of Radiology Oncology Washington University School of Medicine St Louis, Missouri Parvin F Peddi, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Aruna Rokkam, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Cesar Sanchez, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Kristen M Sanfilippo, MD Fellow Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Brian A Van Tine, MD, PhD Assistant Professor of Medicine Division of Medical Oncology Washington University School of Medicine St Louis, Missouri Tzu-Fei Wang, MD Clinical Fellow Department of Medicine Division of Hematology/Oncology Washington University School of Medicine St Louis, Missouri Saiama Waqar, MD Instructor 10 hydroxyurea and, 122–124 hyperhemolytic crises and, 125 incidence rates for, 120 infections from, 126 life expectancy with, 121 management of, 122–124 anesthesia as factor for, 131 through diagnosis, 122 neonatal screening for, 122 through patient visits, 122 through surgery, 131 pathophysiology of, 120 pregnancy and, 128 subacute anemia and, 125 symptoms of, 120–121, 124t acute pain as, 125 transfusion complications alloimmunization in, 132 hyperviscosity syndrome in, 132 iron overload from, 132 transfusion therapy for, 132 treatment of, 122–124 variants of, 123t Sickle cells, 5, 122 anemia from, 31 Sickle cell trait, 121 Sideroblastic anemias, 26 Sigmoidoscopy, for CRC, 240 Sinusoidal obstruction syndrome (SOS), 392 Sipple’s syndrome, 318 Skin ulcers, 131 Small-cell lung cancer (SCLC), 234–236 incidence rates for, 234 palliative care for, 235 paraneoplastic syndromes and, 234 Cushing syndrome, 234 Lambert–Eaton syndrome, 234 705 SIAD, 234 PCI for, 235 staging for, 235 survival rates for, 234 symptoms of, 234 Smoldering MM (SMM), 149 Soft tissue sarcomas, 292–296 biopsies for, 293 chemotherapy for, 294–295 diagnosis for, 292–293 in extremities, 292, 294–295 lab workups for, 293 metastatic, 295–296 pathology of, 292 prognosis after, 296 radiation therapy for, 294 retroperitoneal, 292, 295 staging for, 297t symptoms of, 292 treatment for, 294–296 Solitary extramedullary plasmacytoma (SEP), 151 Solitary plasmacytomas See Plasmacytomas, solitary SOS See Sinusoidal obstruction syndrome Spherocytes, Spinal cord compression, 201 See also Epidural spinal cord compression Spiritual care, 429 Splenectomy, 46 for CIMF, 105 Splenic irradiation, 105 for CIMF, 106 Splenomegaly, 33 Spurious leukocytosis, 17 Squamous cell carcinoma of the head and neck (SCCHN), 279–286 complications from, 285–286 diagnosis of, 280–281 follow-up, 286 incidence rates for, 279 706 management of, 281–283 through imaging studies, 281 pathophysiology for, 279–280 prognosis after, 286 risk factors for, 280 alcohol use as, 280 HPV as, 280 tobacco use as, 280 treatment for, 283–285 Sézary cells, Staging systems for adrenal cortical tumors, 312 of anal cancer, 247t for breast cancer, 212 with TNM system, 213t for cancers, 158–159 clinical, 159 pathologic, 159 TNM classification, 159 for CLL, 368t for CRC, 242–243t for esophageal cancer, 252, 253t for gastric cancer, 258t for HL, 375 with Ann Arbor system, 375, 377t for malignant melanomas, 273–275 for MM, 151 Durie–Salmon, 151 for nasopharyngeal carcinomas, 288t, 289t for NSCLC, 230, 231–232t with TNM system, 231–232t for pancreatic cancer, 261t for renal cell carcinoma, 324, 324t for soft tissue sarcomas, 297t for thyroid carcinoma, 305 Status epilepticus, 443 Steroids 707 for AA treatment, 93 for acquired immune/nonimmune hemolytic anemias, 33 Storage diseases, Streptokinase, 143 Superficial spreading melanoma (SSM), 271 Superior vena cava syndrome (SVCS), 433–434 definition of, 433 diagnosis of, 433–434 symptoms of, 433 treatment for, 434 with radiation therapy, 201, 434 Supportive care, 414–429 See also Pain management; Palliative care for anemia, 426 for anorexia/cachexia, 424–425 for constipation, 419–420 for diarrhea, 420–421 for dyspnea, 425 emotional symptom management as part of, 426–427 for anxiety, 427–428 for delirium, 428 for depression, 426 for insomnia, 428–429 for HSCT, 390–391 for mucositis, 423–424 for nausea and vomiting, 421–423 pain management as part of, 414–418 acute, 415–416 assessment in, 415 chronic, 416 definition of, 414 for pancreatic cancer, 262 for sickle cell disease, 125 treatments for, 415–416, 417t spiritual, 429 Surgery for breast cancer, 214–215 for cancers, 166–167 708 for cervical cancer, 335 for CRC, 244 for esophageal cancer, 252 for essential thrombocythemia, 100 for hepatocellular carcinoma, 265 intraoperative blood recovery during, 131 for NSCLC, 230 for pancreatic cancer, 262 for parathyroid carcinomas, 308 for pheochromocytomas, 316 for polycythemia vera, 100 for prostate cancer, 322 for rectal cancer, 244 sickle cell disease and, 131 for testicular cancer, 327 for thyroid carcinoma, 305 SVCS See Superior vena cava syndrome SVC syndrome, 229 Syndrome of inappropriate antidiuresis (SIAD), 234, 438–439 causes of, 439 management of, 439 symptoms of, 439 T Tamoxifen, for breast cancer, 217, 218 Tamponade, 431–433 causes, 431 symptoms for, 431 treatment for, 433 Target cells, Taxanes, 188t Teardrop cells/dacryocytes, Tenecteplase, 144t Testicular cancer, 325–327 causes of, 325 diagnosis of, 325–326 with serum tumor markers, 326 709 follow-up of, 327 germ cell tumors and, 325 incidence rates for, 325 from nonseminomal tumors, 325 treatment of, 326–327 pathology for, 325 prognosis after, 327 risk factors for, 325 from seminomal tumors, 325 symptoms of, 325 treatment for, 326–327 with chemotherapy, 326 with surgery, 327 Thalassemias, 26–27 alpha, 27 beta, 26 Thalassemias definition of, 26 epidemiology of, 26 pathophysiology of, 26–27 treatment for, 27 Thalidomide, 105 for CIMF, 105 Thalidomide/dexamethasone (TD), 150 Therapies See Adjunctive therapy; Adjuvant therapy; Androgen deprivation therapy; Antimicrobial therapy; Chemotherapy; Gene therapy; Hormonal therapy; Neoadjuvant therapy; Radiation therapy Thrombic thrombocytopenic purpura (TTP), 37 Thrombin time (TT), 48 Thrombocythemia, essential, 101–103 definition of, 101 diagnostic criteria for, 98t, 101–102 incidence rates for, 101 pathophysiology of, 101 pregnancy and, 103 prognosis after, 103 risk factors for, 99t 710 symptoms of, 101 treatment of, 102–103 Thrombocytopenia, 36–47 See also Heparin-induced thrombocytopenia (HIT); Idiopathic thrombocytopenic purpura (ITP) causes of, 36 classification, 36 definition, 36 DIC, 40–42 associated conditions, 41 definition of, 40 diagnosis of, 42 epidemiology, 41 management of, 42 pathophysiology of, 41, 41t peripheral smears for, 42 symptoms of, 42 thrombosis and, 40 treatment for, 42 differential diagnosis for decreased platelet production, 36 increased platelet destruction, 36–37 direct/indirect Coombs tests for, 31 HIT, 42–44 classification for, 42 diagnosis of, 43–44 epidemiology and etiology of, 43 management of, 43 pathophysiology of, 43 risk factors, 43 treatment for, 44 HUS, 37–40 ADAMTS 13 and, 37, 38t diagnosis of, 39–40 etiology for, 39t findings for, 39t pathophysiology, 37 peripheral blood smears for, 39 711 plasma exchange for, 40 remission of, 40 treatment for, 40 ITP, 45–46 chronic refractory, 46 classification of, 45 definition of, 45 diagnosis for, 45 pathophysiology of, 45 treatment for, 46 MAHA and, 37 TTP, 37–40 ADAMTS 13 and, 37, 38t causes for, 37 diagnosis of, 39–40 etiology for, 39t findings for, 39t incidence rates for, 37 management of, 39–40 pathophysiology of, 37 peripheral blood smears for, 39 plasma exchange for, 40 remission of, 40 symptoms of, 39 treatment for, 40 Thrombocytosis, 46–47 essential, 9, 47 reactive, 47 Thrombolytics, 63, 134–146 alteplase, 144t and fibrinolytics, 143, 144t streptokinase, 144t tenecteplase, 144t teteplase, 144t urokinase, 144t Thrombophilia, 66–70 causes of, 66–68 712 activated protein C resistance/factor V Leiden as, 67 antithrombin deficiency as, 67–68 elevated factor VIII levels, 68 hereditary thrombotic dysfibrinogenemia as, 68 protein deficiencies as, 68 prothrombin G20210A as, 67 management of, 68–69 treatment of, 69–70 Thrombosis, 46–47 Thrombotic disease, 58–72 See also Arterial thromboembolism; Thrombophilia acute recurrent, 64 arterial thromboembolism, 70–72 anticoagulants and, 71 causes of, 70 risk factors for, 70 symptoms of, 70 Budd–Chiari syndrome and, 65 cancer and, 66 of cerebral veins/sinuses, 64–65 DVT, 58–66 causes of, 59t complications from, 66 definition of, 58 diagnosis of, 59–61 Doppler ultrasonography for, 60 incidence rates for, 58 management of, 59–61 symptoms of, 59 treatment of, 61–64 of upper extremities, 65 mesenteric, 65 PE, 58–66 causes of, 59t complications from, 66 diagnosis of, 59–61 Doppler ultrasonography for, 60 713 incidence rates for, 58 management of, 59–61 PIOPED trials for, 60 treatment of, 61–64 portal venous, 65 during pregnancy, 64 in renal veins, 65 thrombophilia, 66–70 causes of, 67–68 management of, 68–69 treatment of, 69–70 Thrombotic thrombocytopenic purpura (TTP), 37–40 ADAMTS 13 and, 37, 38t causes for, 37 diagnosis of, 39–40 etiology for, 39t findings for, 39t incidence rates for, 37 management of, 39–40 peripheral blood smears for, 39 plasma exchange for, 40 remission of, 40 symptoms of, 39 treatment for, 40 Thyroid carcinoma, 303–307 anaplastic, 306 diagnosis of, 303–305 histologic subtypes, 304t incidence rates for, 303 prognosis after, 307 staging of, 305 treatment of, 305–306 with adjuvant therapy, 306 with hormonal therapy, 306 with radiation/chemotherapy, 305–306 with radioiodine therapy, 305–306 with surgery, 305 714 variants of, 303 Thyroid carcinoma follow-up of, 307 prognosis, 307 risk factors for, 303 Tirofiban, 139 Tobacco use lung cancer from, 228 SCCHN from, 280 Topoisomerase inhibitors, 186t Total iron binding capacity (TIBC), 24 Toxic granulation, TRALI See Transfusion-related acute lung injury Transferrin saturation (Tsat), 24 Transfusion-related acute lung injury (TRALI), 118t Transfusions, 110–118 blood products, 111, 113–114t modifications of, 111, 115t of CMV-negative products, 115t complications, 116 management of, 117–118t donors for, 110 of gamma-irradiated products, 115t of granulocytes, 115t hepatitis and, 111t HIV/AIDS and, 111t infection risks from, 114t for leukoreduction, 115t of platelets, 116 complications from, 116 dosing for, 113t products for, 113t pretransfusion testing, 110–111 antibody screening as, 110 for compatibility, 110–111, 112t crossmatching in, 111 donor screening, 110 715 for infectious diseases, 111t emergency release of blood products, 111 of RBCs bacterial contamination from, 117t dosing for, 113t graft-versus-host disease from, 115t hemolytic reactions from, 117t indications for, 113t iron overload from, 132 packed, 113t products of, 113–114t TRALI from, 118t viral infections from, 114t for sickle cell disease, 131 of whole blood, 111 Trastuzumab, 222 Trousseau syndrome, 65 TT See Thrombin time TTP See Thrombotic thrombocytopenic purpura Tumor lysis syndrome See Acute tumor lysis syndrome Tumor markers, 165 Tumor metastasis, 177–178 Tumor suppressor genes, 175–176 Turcot syndrome, 240 U Unsaturated iron binding capacity (UIBC), 24 Urokinase, 144t Uterine cancer, 332 management of, 332 symptoms of, 332 Uterine leiomyomas, 332 V Vaginal bleeding, persistent, 330–331 von Willebrand disease and, 331 Venography, 65 716 Venous thromboembolism (VTE), 58 causes of, 59t during pregnancy, 64 risk factors for, 58 treatment of, 62 Ventilation/perfusion scans (V/Q scans), 60 Viruses, 174 HIV/AIDS, 14, 111t HPV, 246 tumorigenesis and, 174 Vitamins, 29 B12 deficiencies of, anemias as result of, 29 K, 79, 145 as coagulant, 145 deficiency disorders and, 79 Vomiting See Nausea and vomiting Von Willebrand disease (vWD), 52, 74–77 classification of, 75t diagnosis of, 75–76 factor VIII assay for, 53–54 management of, 74–77 pathophysiology of, 74 persistent vaginal bleeding and, 331 platelet function and, 54 with lab evaluation, 56t subtypes of, 75t symptoms of, 75 treatment for, 76–77 Von Willebrand factor (VWF), 74 antigen concentration (vWF:Ag), 55 multimer assay, 57 W Waldenström macroglobulinemia (WM), 154–156 diagnosis of, 154–155 incidence rates for, 154 717 prognosis after, 155 symptoms of, 154–155 treatment of, 155 Warfarin, 139–140 adverse effects of, 140 dosage for, 139 for DVT, 64 for HIT, 44 reversal of, 140t Werner’s syndrome, 317 White blood cells (WBCs) See also Leukocytosis; Leukopenia abnormalities in, 6–7 auer rods as, blast cells as, hairy cells as, Pelger–Huet anomaly, Sézary cells as, average adult count for, 11t basophils in, disorders in, 10–21 leukocytosis, 14–16 leukopenia, 14 eosinophils in, gender differences in, 10 lymphocytes in, monocytes in, neutrophils in, 5–6 hypersegmented, 5–6 in peripheral smears, analysis of, WHO See World Health Organization WM See Waldenström macroglobulinemia World Health Organization (WHO) AML classification under, 358t lymphoma classification under, 374 for HL, 375t for NHL, 380t MDS classifications under, 84, 85t 718 MPD classifications under, 95, 96t 719 [...]... directly applied at the bedside and in outpatient settings to improve patient care Victoria J Fraser, MD Dr J William Campbell Professor Interim Chairman of Medicine Co-Director of the Infectious Disease Division Washington University School of Medicine 12 13 e are pleased to present the third edition of The Washington Manual Hematology and Oncology Subspecialty Consult The field of medical oncology continues...Division of Medical Oncology Washington University School of Medicine St Louis, Missouri Israel Zighelboim, MD Assistant Professor Department of Obstetrics and Gynecology Division of Obstetrics and Gynecology Oncology Washington University School of Medicine St Louis, Missouri Imran Zoberi, MD Assistant Professor Department of Radiation Oncology Washington University School of Medicine St Louis,... Henderson and Tom De Fer, who have worked tirelessly to produce another outstanding edition of this manual I would also like to thank Dr Melvin Blanchard, Chief of the Division of Medical Education in the Department of Medicine at Washington University School of Medicine, for his advice and guidance I believe this Subspecialty Manual will meet its desired goal of providing practical knowledge that... include house officers, fellows, and attendings at Washington University School of Medicine and Barnes-Jewish Hospital Their commitment to patient care and education is unsurpassed, and their efforts and skill in compiling this manual are evident in the quality of the final product In particular, I would like to acknowledge our editors, Drs Amanda Cashen and Brian A Van Tine, and the series editors, Drs... Most of the authors are hematology oncology fellows or internal medicine residents, the physicians who have recent experience with the issues and questions that arise in the course of training in these subspecialties Primary care practitioners and other health care professionals also will find this manual useful as a quick reference source in hematology and oncology As the practice of hematology and oncology. .. dosing and indications for chemotherapy and targeted therapies will occur, and staging systems will be modified We recommend a handbook of chemotherapy regimens and an oncology staging manual to complement the information in this book And of course, clinical judgment is imperative when applying the principles presented here to the care of individual patients We appreciate the effort and expertise of everyone... who contributed to this edition of the Hematology and Oncology Subspecialty Consult In particular, we would like to thank the authors for their enthusiastic efforts to distill volumes of medical advances into a concise, useable format We also recognize the faculty in the divisions of hematology, oncology, bone marrow transplantation, radiation 14 oncology, and gynecologic oncology at Washington University... pleasure to present the new edition of The Washington Manual Subspecialty Consult Series: Hematology/ Oncology Subspecialty Consult This pocket-size book continues to be a primary reference for medical students, interns, residents, and other practitioners who need ready access to practical clinical information to diagnose and treat patients with a wide variety of disorders Medical knowledge continues to increase... stages of normoblasts known as basophilic, chromatophilic, and orthochromic The nucleus gradually condenses, and the cytoplasm gradually takes on the pinkish hue of Hgb found in mature red cells Bone marrow core biopsies are fixed in a buffered formaldehydebased solution and then embedded in paraffin or plastic Biopsies are used to assess the cellularity of the bone marrow and the presence of neoplasias,... acquired Congenital causes are usually suggested by family history Most cases of neutropenia are acquired and related to decreased granulocyte production and, less often, increased destruction Pseudo-neutropenia may be obtained by analyzing blood several hours old and in the presence of paraproteinemia and certain anticoagulants that can cause clumping Lower ANCs occur in African Americans as a result of ... Professor of Medicine Division of Medical Oncology Washington University School of Medicine St Louis, Missouri Tzu-Fei Wang, MD Clinical Fellow Department of Medicine Division of Hematology/ Oncology. .. of Medicine Co-Director of the Infectious Disease Division Washington University School of Medicine 12 13 e are pleased to present the third edition of The Washington Manual Hematology and Oncology. .. Library of Congress Cataloging-in-Publication Data The Washington manual hematology and oncology subspecialty consult — 3rd ed / editors, Amanda Cashen, Brian A Van Tine p ; cm — (Washington manual