Book Review

Hematology: Thrombosis and Hemorrhage

Hemostasis and Thrombosis: Basic Principles and Clinical Practice

The Hemorrhagic Disorders: A Clinical and Therapeutic Approach

N Engl J Med 1994; 331:1722-1723December 22, 1994

Article

Hematology: Thrombosis and Hemorrhage
Edited by Joseph Loscalzo and Andrew I. Schafer. 1337 pp., illustrated. Boston, Blackwell Scientific, 1994. $225. ISBN: 0-86542-263-X

Hemostasis and Thrombosis: Basic Principles and Clinical Practice
Edited by Robert W. Colman, Jack Hirsh, Victor J. Marder, and Edwin W. Salzman. 1713 pp., illustrated. Philadelphia, J.B. Lippincott, 1994. $195. ISBN: 0-397-51059-4

The Hemorrhagic Disorders: A Clinical and Therapeutic Approach
By Mario Stefanini and William Dameshek. 308 pp., illustrated. New York, Grune and Stratton, 1955. Out of print.

When I was preparing for my fellowship in hematology I bought two books about the blood: Maxwell M. Wintrobe's Clinical Hematology (Philadelphia: Lea & Febiger) and The Hemorrhagic Disorders by Mario Stefanini and William Dameshek. The Wintrobe book, which I bought in its trim, single-author 1951 edition (the third), has recently appeared in its ninth edition, comprising two enormous volumes with 33 contributing authors. The Hemorrhagic Disorders, published in 1955, is now out of print. It is a handsome book, typical of the superior work from the old publishing house of Grune and Stratton. The excellent color plates show graphic examples of petechiae, ecchymoses, the tourniquet test, and the clot-retraction test. The tourniquet test has been abandoned, but both of the modern textbooks under review mention clot retraction. However, only The Hemorrhagic Disorders shows what the phenomenon looks like in a test tube. Its index lists the word “factor” four times; the entry for factor IX reads, “Factor IX. See Plasma thromboplastin component.” By contrast, “factor” takes up more than three columns in the index of Thrombosis and Hemorrhage, and more than eight columns of fine print in the index of Hemostasis and Thrombosis. Astonishingly, the index of Hemostasis and Thrombosis lists 54 mutant variants of factor IX, from factor IX Alabama, through factor IX Hollywood, to factor IX Yemen. The Hemorrhagic Disorders, with less than 350 pages of text, was easily read on a trolley. Hemostasis and Thrombosis and Thrombosis and Hemorrhage, both behemoths, nearly require furniture movers.

Thrombosis and Hemorrhage is well produced and logically organized. It has many excellent diagrams and tables, good photographs, and color plates. The 25-page index is impressive. The design of fibrin strands that heads each of the 63 chapters is a nice touch. The 94 authors include leading authorities, mainly from the United States, almost one quarter of them from the Boston-Cambridge circuit. About one third of the book deals with basic mechanisms of coagulation; of the remainder, approximately two thirds deals with hemorrhagic diseases and one third with thrombotic disorders.

Hemostasis and Thrombosis is also handsome. Its black-and-white diagrams, tables, and photographs equal those in Thrombosis and Hemorrhage. The index of Hemostasis and Thrombosis fills almost 49 pages, and the 129 contributors to its 86 chapters rival the authors of Thrombosis and Hemorrhage. The two books together supply a roster of most of the leading students of hemostasis in the United States.

To compare Thrombosis and Hemorrhage and Hemostasis and Thrombosis, I read what each had to say on four topics: the clinical approach to the patient with a hemorrhagic disorder, the antiphospholipid-antibody syndrome, hypercoagulable states, and the treatment of hemophilia. In Thrombosis and Hemorrhage, Andrew Schafer gives a well-reasoned, thorough, and mature discussion of how an experienced physician approaches the diagnosis in a patient with abnormal bleeding. He appropriately stresses the importance of the history and physical examination, but I think he overemphasizes the importance of the bleeding time. Helpful charts and diagrams illuminate his excellent discussion of other laboratory tests. Five authors -- the four editors and Gilbert C. White II -- wrote the comparable chapter in Hemostasis and Thrombosis. Although it is not as well written as Schafer's, as might be expected of a chapter with so many authors, its excellent tables and the six case studies give it a slight edge. But any physician who receives the dreaded telephone call at 2 a.m. -- “I've got a patient in the operating room with uncontrollable bleeding” -- will find welcome relief in either chapter.

Barbara Alving has written an exceptionally good chapter on the antiphospholipid-antibody syndrome for Thrombosis and Hemorrhage. She presents the modern view of the disorder logically, comprehensively, and sensibly. In Hemostasis and Thrombosis, Donald Feinstein includes the discussion of the antiphospholipid-antibody syndrome in his chapter on the immune coagulation disturbances. His review is also authoritative, as would be expected from the coiner of the term “lupus anticoagulant.” It is well reasoned, clear, and thorough, but it was written too soon to mention hexagonal phospholipids, and it refers only briefly to β₂-glycoprotein I. Both are now important aspects of the antiphospholipid-antibody syndrome.

Both books came too late for an exciting advance in the understanding of thrombophilia: hereditary resistance to activated protein C. This new disorder, which is often due to a mutation in the gene for factor V, accounts for up to one third of cases of hitherto “idiopathic” recurrent venous thrombosis. It was recently described in the Journal (Peter J. Svensson and Bjorn Dahlback. “Resistance to Activated Protein C as a Basis for Venous Thrombosis.” 1994;330:517-522). Nevertheless, Jack Hirsh, Martin Prins, and Michael Samama, in Hemostasis and Thrombosis, give comprehensive and valuable lessons on how to evaluate a patient with recurrent thrombosis. Their systematic review contains excellent clinical pointers and good advice on the use of laboratory tests for the diagnosis of this frustrating and difficult group of disorders. Philip Comp's chapter on the topic in Thrombosis and Hemorrhage is briefer, has only 1 table (the chapter in Hemostasis and Thrombosis has 12), and contains no figures. However, some of the material not covered by Comp appears in other chapters.

In Thrombosis and Hemorrhage, David Green and Donna DiMichele write clearly and wisely about the treatment of hemophilia. They present an excellent historical review of the disorder and a comprehensive, detailed analysis of the problem, including its psychosocial and economic aspects. Their literate account reveals a compassionate view of the patient with hemophilia. To find information about inhibitors of factor VIII, however, one must look in another chapter with the clumsy title “Factor VIII and Other Coagulation Factor Inhibitors.” Doreen Brettler and Peter Levine write about clinical aspects of hemophilia for Hemostasis and Thrombosis. They give more detailed information about replacement therapy and the risk of viral infection than Green and DiMichele in Thrombosis and Hemorrhage, but recombinant factor VIII was developed too late for comment (Green and DiMichele mention it). Neither chapter says much about prophylactic therapy (“home therapy”). This is all the more disappointing because both Hemostasis and Thrombosis, published in 1994, and The Hemorrhagic Disorders, published in 1955, show radiographs of advanced hemophilic arthropathy of the knees. The earlier authors hint at an improvement: “It may be said that `continuous protective' management... must await future developments.” The 1994 book says, “Clearly, then, the best therapy for hemophilic arthropathy is prevention,” but offers no other guidance.

As for recommendations, it is hard to decide between Hemostasis and Thrombosis and Thrombosis and Hemorrhage. Both are excellent. A trainee in hematology would not make a mistake with either one, and if money were no object, having both would be fabulous. Hemostasis and Thrombosis is perhaps the more comprehensive of the two, but Thrombosis and Hemorrhage is slightly more up to date. Hematologists in clinical practice might want both; researchers in the field of hemostasis should find a way to obtain both. I myself am lucky enough to have both because I reviewed them for the Journal.

Robert S. Schwartz, M.D.