Essentials of Haematology Essentials of Haematology SSSSSHHHHHIIIIIRRRRRIIIIISSSSSHHHHH MMMMM KKKKKAAAAAWWWWWTTTTTHHHHHAAAAALLLLLKKKKKAAAAARRRRR MD (Pathology) Senior Lecturer in Pathology Govt. Medical College Nagpur (Maharashtra) JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD New Delhi Published by Jitendar P Vij Jaypee Brothers Medical Publishers (P) Ltd EMCA House, 23/23B Ansari Road, Daryaganj New Delhi 110 002, India Phones: +91-11-23272143, +91-11-23272703, +91-11-23282021, +91-11-23245672 Fax: +91-11-23276490, +91-11-23245683 e-mail: [email protected] Visit our website: www.jaypeebrothers.com Branches • 202 Batavia Chambers, 8 Kumara Krupa Road, Kumara Park East, Bangalore 560 001, Phones: +91-80-22285971, +91-80-22382956, +91-80-30614073 Tele Fax: +91-80-22281761 e-mail: [email protected] • 282 IIIrd Floor, Khaleel Shirazi Estate, Fountain Plaza Pantheon Road, Chennai 600 008, Phones: +91-44-28262665, +91-44-28269897 Fax: +91-44-28262331 e-mail: [email protected] • 4-2-1067/1-3, Ist Floor, Balaji Building, Ramkote Cross Road, Hyderabad 500 095, Phones: +91-40-55610020, +91-40-24758498 Fax: +91-40-24758499 e-mail: [email protected] • 1A Indian Mirror Street, Wellington Square, Kolkata 700 013, Phones: +91-33-22456075, +91-33-22451926 Fax: +91-33-22456075 e-mail: [email protected] • 106 Amit Industrial Estate, 61 Dr SS Rao Road, Near MGM Hospital Parel, Mumbai 400 012, Phones: +91-22-24124863, +91-22-24104532, +91-22-30926896 Fax: +91-22-24160828 e-mail: [email protected] • Kamalpushpa, 38 Reshimbag, Opp. Mohota Science College, Umred Road Nagpur 440 009, Phone: +91-0712-3945220 e-mail: [email protected] Essentials of Haematology © 2006, Shirish M Kawthalkar All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the author and the publisher. This book has been published in good faith that the material provided by author is original. Every effort is made to ensure accuracy of material, but the publisher, printer and author will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters to be settled under Delhi jurisdiction only. First Edition: 2006 ISBN 81-8061-633-9 Typeset at JPBMP typesetting unit Printed at Gopsons Paper Ltd, Noida Preface This book is an attempt to present haematology and blood transfusion in a concise and simplified manner and is primarily intended for undergraduate students (second and final year MBBS and BDS). At the same time, it will also be useful for postgraduate students of pathology, medicine, paediatrics, and obstetrics and gynaecology. One comes across a haematological problem frequently in all branches of medicine. Haematology and blood transfusion are closely related and blood bank forms an integral and life-saving support system of a multidisciplinary hospital. A concise textbook of haematology and blood transfusion is needed for undergraduates who have to take on major subjects during their MBBS years. The coverage of haematology in available books is either too extensive or too short for their requirements. I have tried to strike a balance based on my experience in teaching and in diagnostic haematology. This book was published in 1998 as “Essentials of Haematology and Blood Transfusion”. Changes are constantly occurring in haematology especially in molecular diagnostics, classification, and treatment of malignant disorders. I have tried to update each chapter to ensure that current knowledge and practices are reflected. Laboratory investigations play a major role in proper diagnosis and management of blood diseases. Therefore, laboratory aspects have been given relatively more coverage. As the scope of this book is limited, it has not been possible to give treatment of blood disorders in detail, especially dosages and drug schedules. During preparation of this book help has been taken from various well-known textbooks and numerous journals that have been duly acknowledged at the end of each chapter. Figures of blood and bone marrow cells have been presented in a manner that highlights the important morphological details and helps in better understanding. Undoubtedly, there will be errors of omission and commission for which I take the full responsibility. Suggestions and constructive criticism are most welcome. I am indebted to my parents for their constant support, value-based guidance, and blessings that I have received throughout my life. The friendship, love, care, and support of my wife Anjali, herself a gynaecologist, can never be adequately acknowledged and my children Ameya and Ashish, have made everything in life meaningful, worth while, and enjoyable. Essentials of Haematology I am thankful to Dr (Mrs) VS Dani, Dean, Govt Medical College, Nagpur and Dr SK Bobhate, Professor and Head, Dept of Pathology, Govt Medical College, Nagpur for their valuable guidance. I express my appreciation and gratitude to M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi for their sensible and valuable advice during publication of this book and also for bringing out the book in an excellent, easy to read format. Shirish M Kawthalkar vi Haematopoietic Stem Cell Transplantation Contents SSSSSeeeeeccccctttttiiiiiooooonnnnn IIIII::::: PPPPPhhhhhyyyyysssssiiiiiooooolllllooooogggggyyyyy ooooofffff BBBBBlllllooooooooooddddd 1. Overview of Physiology of Blood....................................................................................................3 SSSSSeeeeeccccctttttiiiiiooooonnnnn IIIIIIIIII::::: DDDDDiiiiisssssooooorrrrrdddddeeeeerrrrrsssss ooooofffff RRRRReeeeeddddd BBBBBlllllooooooooooddddd CCCCCeeeeellllllllllsssss (((((AAAAAnnnnnaaaaaeeeeemmmmmiiiiiaaaaasssss))))) 2. Approach to Diagnosis of Anaemias............................................................................................55 3. Anaemias due to Impaired Red Cell Production........................................................................75 4. Anaemias due to Excessive Red Cell Destruction................................................................... 121 SSSSSeeeeeccccctttttiiiiiooooonnnnn IIIIIIIIIIIIIII::::: DDDDDiiiiisssssooooorrrrrdddddeeeeerrrrrsssss ooooofffff WWWWWhhhhhiiiiittttteeeee BBBBBlllllooooooooooddddd CCCCCeeeeellllllllllsssss 5. Acute Leukaemias........................................................................................................................ 223 6. Myelodysplastic Syndromes....................................................................................................... 261 7. Chronic Myeloproliferative Disorders ...................................................................................... 268 8. Chronic Lymphoid Leukaemias................................................................................................. 284 9. Plasma Cell Dyscrasias................................................................................................................ 295 10. Malignant Lymphomas ............................................................................................................... 316 11. Quantitative and Qualitative Disorders of Leucocytes .......................................................... 330 12. Haematopoietic Stem Cell Transplantation............................................................................. 354 SSSSSeeeeeccccctttttiiiiiooooonnnnn IIIIIVVVVV::::: DDDDDiiiiisssssooooorrrrrdddddeeeeerrrrrsssss ooooofffff HHHHHaaaaaeeeeemmmmmooooossssstttttaaaaasssssiiiiisssss 13. Approach to the Diagnosis of Bleeding Disorders.................................................................. 363 14. Bleeding Disorders Caused by Abnormalities of Blood Vessels (The Vascular Purpuras)............................................................................................................. 379 15. Bleeding Disorders Caused by Abnormalities of Platelets ................................................... 382 16. Disorders of Coagulation............................................................................................................. 396 vii Essentials of Haematology SSSSSeeeeeccccctttttiiiiiooooonnnnn VVVVV::::: BBBBBlllllooooooooooddddd TTTTTrrrrraaaaannnnnsssssfffffuuuuusssssiiiiiooooonnnnn 17. Blood Group Systems .................................................................................................................. 431 18. Serologic and Microbiologic Techniques ................................................................................. 437 19. Collection of Donor Blood, Processing and Storage................................................................ 447 20. Whole Blood, Blood Components and Blood Derivatives...................................................... 455 21. Transfusion of Blood to the Recipient....................................................................................... 467 21. Adverse Effects of Transfusion.................................................................................................. 472 23. Autologous Transfusion .............................................................................................................. 484 24. Alternatives to Blood Transfusion............................................................................................. 487 Appendices Appendix A: Reference Ranges ................................................................................................. 491 Appendix B: Selected CD Antigens........................................................................................... 494 Index ................................................................................................................................................ 499 viii Essentials of Haematology 2