Book Review

Hematology: Landmark Papers of the Twentieth Century

N Engl J Med 2001; 344:1562May 17, 2001

Article

Hematology: Landmark Papers of the Twentieth Century
Edited by Marshall A. Lichtman, Jerry L. Spivak, Laurence A. Boxer, Sanford J. Shattil, and Edward S. Henderson. 1065 pp., illustrated. San Diego, Calif., Academic Press, 2000. $149.95. ISBN: 0-12-448510-3

Napoleon once observed that history is the version of past events that people have decided to agree on. And so it is with Hematology: Landmark Papers of the Twentieth Century. This anthology of 86 papers was assembled from an initial list of 450 articles by sequential votes of the five editors, who “focused on articles that pioneered the clinical description, pathogenesis, diagnosis, or treatment of disease.” One could quibble with the decision to exclude basic sciences and “pathological insights” because they had such a key role in the evolution of hematology from a descriptive clinical specialty to a discipline firmly grounded in biologic mechanisms. Omitting these kinds of articles means that readers lose a chance to see the scientific underpinnings of such topics as clinical bone marrow transplantation and clonal proliferation in lymphomas. They also miss some remarkable insights, such as those that led to the concepts of myeloproliferative and lymphoproliferative diseases — both of which are in daily use by hematologists the world over.

Nevertheless, many of these absentees do receive mention in the informative commentaries that precede the reproductions of the original papers. For example, the discovery of the Epstein–Barr virus and the link between this virus and certain lymphomas is well described in the note concerning the first description of atypical lymphocytes in the blood of patients with infectious mononucleosis. Even so, given the choice between the landmark 1968 paper that linked Epstein–Barr virus to infectious mononucleosis and Felty's description of splenomegaly and neutropenia in rheumatoid arthritis, I would have picked the former. But since disagreement is the lifeblood of hematology, the editors deserve credit for making the hard choices that make this book so fascinating.

Another quibble is the organization of the entries into four sections: “Descriptions of Hematologic Diseases or Syndromes,” “Insights into the Pathophysiology of Hematologic Disorders,” “Advances in Treatment of Hematologic Diseases,” and “Diagnostic or Clinical Laboratory Innovations.” This scheme is perhaps logical, but it disrupts historical continuity. Readers will have to hunt through many pages of the book to connect the discovery that feeding large amounts of liver causes a remission of pernicious anemia with Castle's observation that normal gastric juice contains a factor that prevents the disease, Wintrobe's description of the mean corpuscular volume, the first description of tropical macrocytic anemia, and Herbert's report of the development of macrocytic anemia when he put himself on a folate-deficient diet. The same difficulty appears when one attempts to trace the histories of transfusion medicine, sickle cell anemia, hemorrhagic diseases, and other disorders. Exacerbating the problem is the frequent discontinuity between the commentary and the article: 372 pages separate the annotation concerning the one-stage clotting test for hemophilia and the paper itself. This layout is not reader-friendly.

These criticisms boil down to debatable points about style and should not detract from the value of the papers chosen for reproduction, which are of considerable interest (those not published in English are offered in the original version and in translation). The first entry, Osler's description of polycythemia vera, published in 1903, is worth the price of admission. It is a combination of sharp clinical observation, keen analysis of the facts, and trenchant commentary on the literature. I would urge every trainee in hematology to read this jewel of a paper, which rejects jargon and fearlessly declaims the first person singular. The last entry, an article by Kan et al. that reports the first example of the prenatal diagnosis of sickle cell anemia (the commentary appears on page 45; the article starts on page 985), foretells the future. Much of what physicians do now — and will be doing increasingly — to counsel patients about heritable diseases, whether they are due to mutations in a single gene or are multigenic in origin, rests on this work. The giant leap from Osler's remarkable synthesis of clinical findings in polycythemia vera to the emergence of molecular biology from the laboratory to the hematology clinic is an event of historic significance that will occupy the attention of historians of medicine and philosophers of science for many years. This book, which contains some of the primary data supporting that claim, merits the attention of all hematologists who want to know the origin of their specialty.

Robert S. Schwartz, M.D.