The Molecular Basis of Blood Diseases.

Abstract

The recent application of the techniques of molecular biol- ogy to the study of human diseases has made it possible to obtain information unavailable-and, in some instances, unimaginable-a decade ago.Because of the rapidity with which developments have occurred, many clinicians and scientists may be unaware of them and of the insights they provide into the origin of diseases.To address the needs of this audience, four noted authorities in the field, George Stamatoyannopoulos, Arthur Nienhuis, Philip Leder, and Philip Majerus, have recruited 27 of their colleagues to describe what is currently known about the molecular basis of blood diseases.The first chapter introduces the reader to the methods of molecular cloning and analysis of gene structure that have generated much of the information pre- sented subsequently.Several chapters then describe gene structure, expression, and regulation, using the globin genes as a model.After this general introduction, specific groups of diseases are addressed.Each area of classic hematology-the red cell, the white cell (both normal and malignant), and the soluble and cellular components of the hemostatic system (including hemostasis, thrombosis, and fibrinolysis)-is taken up in turn.In addition, three chap- ters present immunological topics-immunoglobulin and T-cell receptor genes (as well as lymphocyte differentia- tion), enzyme deficiencies associated with immunological disorders, and molecular mechanisms of complement ac-