Second edition. Graham R. Serjeant. 631 pages. Oxford: Oxford University Press; 1992. $59.00.

Sickle Cell Disease: Pathophysiology, Diagnosis, and Management

Vipul N. Mankad and R. Blaine Moore; eds. Westport, Connecticut: Praeger; 1992. $79.95.

Sickle cell disease has been both a triumph and a tragedy for American medicine. Scientific investigations into sickle cell disease ushered in the age of molecular medicine. At the same time, the history of sickle cell disease is also remarkable for the thousands of American children who, over the course of 300 years, died without notice from a disease that remained "undiscovered." The paradox holds to this day, when most of my medical students can describe in frightening detail the genetic abnormality that gives rise to sickle cell disease, but few know how to care for the patients.

In the preface to his 1923 textbook, Downey defined hematology as "the study of the blood and all the organs through which it flows." A former hematology chief of mine, reflecting the hostility that too many physicians harbor for sickle cell patients, once declared that sickle cell disease was not to be considered a hematologic disease. But it is, and more so. The study of sickle cell disease has taken physicians beyond mere blood and organs into the study of anthropology, genetics, molecular biology, and sociology. Sickle cell disease continues to present pathophysiologic and clinical puzzles whose solutions promise to broaden our understanding of medical concerns ranging from the formation of aneurysms to the treatment of pain. It is reasonable to expect that sickle cell disease will be the first major disease that will be cured using modern molecular and genetic techniques. With all this in mind, it is perfectly understandable that Drs. Mankad and Moore would be inclined to state, as they do in the preface to their book Sickle Cell Disease: Pathophysiology, Diagnosis, and Management, that "no single physician can have complete knowledge of a complex problem like sickle cell anemia."

Dr. Graham Serjeant proves them wrong. The second edition of his book Sickle Cell Disease is a masterpiece. This book gives complete and understandable accounts of the biology, pathology, and clinical management of sickle cell disease. Even though he invests major effort in exposing the molecular and genetic details of sickle cell disease, Dr. Serjeant does not ever let us forget that it is a disease of people, not just of cells, and that these people (some of whom do not even live in the United States) have histories and cultures. Along with fascinating and lucid discussions of hemoglobin polymerization and an excellent mini-course in genetics, Dr. Serjeant serves us the original laboratory report on the blood film of Walter Clement (the first patient recognized to have sickle cell disease) and the name of the unheralded intern who first described the findings (a Dr. Irons). In his discussion of antisickling agents, Dr. Serjeant reviews the long history of our attempts to modulate the molecular milieu in patients with the disease. These attempts have ranged from the infusion of urea and inhalation of carbon monoxide to current attempts to increase hemoglobin F concentrations through the use of cytotoxic drugs. In the process, he does not forget the African remedies such as the chewstick made from Fagara zanthoxyloides, which in clinical trials has reduced the frequency of painful crises. The discussions of the clinical pathology and management of patients with sickle cell disease are complete and up-to-date, with superb reviews of cutting-edge areas of pathology such as the mechanism of the cerebrovascular complications of the disease.

One gets the sense throughout this beautifully written book that Dr. Serjeant speaks from personal experience. His narrative style is so rich yet freely flowing that I found myself carrying his book into those smaller rooms of the house usually reserved for reading newspapers and magazines, among other functions. Serjeant's book is not only for the clinician who seeks information about sickle cell disease; it is also for the physician and the trainee who wants to know the breadth of medicine and all the places through which it flows. My test of true greatness for a book lies not in whether I would buy it for myself but whether I would buy it for someone else. Come Christmas, some of my friends will find Dr. Serjeant's book under their trees.

Drs. Mankad and Moore's book is a collection of contributions from distinguished researchers and clinicians, many of whom are associated with the Comprehensive Sickle Cell Center at the University of South Alabama. This book contains in-depth reviews on the science of sickle cell disease, with excellent chapters on pathophysiology that should prove valuable to laboratory researchers and clinicians looking for a detailed discussion of the disease. This book provides greater detail than does Dr. Serjeant's work in such basic science areas as the important interactions between polymerizing hemoglobin S and the erythrocyte membrane and the relation between sickling erythrocytes and vascular endothelium. Excellent clinical reviews on the treatment of vaso-occlusive pain and the management of infectious complications of sickle cell disease are also included.

One interesting feature that this book shares with Dr. Serjeant's is a reliance on the work of Dr. L. W. Diggs. Dr. Diggs, who has published important work on the pathology of sickle cell disease in each of the past eight decades, provided many of the fine anatomic illustrations for Dr. Serjeant's book and wrote a definitive chapter on the anatomic pathology of sickle cell disease for Drs. Mankad and Moore's collection. The latter book apparently reflects its authors' individual research interests and in so doing gives short shrift to some aspects of sickle cell disease that are attracting much attention in medical journals. For example, therapies aimed at increasing fetal hemoglobin levels are mentioned only in passing. Nevertheless, this book will be a worthwhile addition to libraries of hematologists and other physicians who care for patients with sickle cell disease.