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Bone Marrow Failure Syndromes
by Young, Neal S., MD Young
From The New England Journal of Medicine, November 16, 2000
Primary and secondary bone marrow-failure disorders have disparate causes, yet many of them have similar clinical presentations. The features of many of these disorders are frequently so nonspecific on routine studies of peripheral blood and bone marrow that it is difficult to establish the correct diagnosis without the aid of one or more definitive cytogenetic, phenotypic, immunologic, or molecular genetic studies. Cognizant of this problem, Young, who is chief of the Hematology Branch of the National Heart, Lung, and Blood Institute of the National Institutes of Health, and 12 colleagues have compiled descriptions of the 10 most common bone marrow-failure syndromes in separate chapters in a compendium just over 200 pages long. Included in these chapters are detailed considerations of acquired, congenital, and hereditary disorders such as aplastic anemia, primary and secondary myelodysplasia, myelofibrosis, several types of agranulocytosis, amegakaryocytic thrombocytopenia, bone marrow failure related to infection with the human immunodeficiency virus, Fanconi's anemia, paroxysmal nocturnal hemoglobinuria, T-cell large-granular-lymphocyte lymphoproliferative disorder, and various forms of pure red-cell aplasia. The explicit role of the hematopoietic stem cell and its various committed forms in the evolution of various bone marrow-failure disorders is an important part of many of the discussions. The chapters are of similar composition and each includes sections on the history of the disease; epidemiologic features; causes and pathogenesis; clinical and laboratory features; and therapy and prognosis. Each chapter is extremely well referenced, and each ends with a concise conclusion. Most of the chapters contain excellent diagrams of pathogenesis, and many include extensive tables or figures presenting possible causative agents, differential diagnosis, or the results of therapy. The color photomicrographs of peripheral blood and bone marrow are of limited value, since the prints are too small to show clearly the specific features of individual cells.
The book is distinguished by its comprehensive coverage of the pathophysiologic features, essential laboratory studies, differential diagnosis, and clinical classifications of bone marrow-failure disorders. Perhaps the most remarkable feature of the book, however, is the manner in which clear explanations of pathophysiology are expertly blended into descriptions of the clinical course of each disorder and its rational therapy. To accomplish this goal, the authors of each chapter used their expertise not only in clinical hematology but also in cytogenetics, immunology, pharmacology, and molecular genetics. Especially notable in this respect are the chapters dealing with Fanconi's anemia, paroxysmal nocturnal hemoglobinuria, myelodysplastic syndromes, and acquired aplastic anemia.
The book is well organized and is readable. The highly specialized nature of its content eminently qualifies it for the bookshelves of hematologists and oncologists and for all medical libraries. Internists, pediatricians, residents, and medical students will also find it very useful as an up-to-date teaching resource and guide for the diagnosis and management of bone marrow-failure disorders in their patients.
Stuart C. Finch, M.D.
Copyright © 2000 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
From Book News, Inc.
Researchers from the National Institutes of Health in Bethesda, Maryland, along with a few other contributors, explore ten disorders that may be linked only by resulting in a lowered blood count that can be traced to the failure of the bone marrow. They are acquired aplastic anemia, Fanconi's anemia, myelodysplastic syndromes, paroxysmal nocturnal hemoglobinuria, myelofibrosis, pure red cell aplasia, agranulocytosis, acquired amegakaryocytic thrombocytopenic purpura, bone marrow failure related... read more
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