RESUMEN
To study erythropoiesis and anemia, one must have a firm foundation of indices that accurately measure red blood cell production and destruction. This paper, authored by hematology legends Arno G. Motulsky and Clement A. Finch, provides that foundation. Using methods that would not be approved in today's environment, the authors studied a cohort of normal healthy patients and an equal number of patients with different forms of anemia. The results confirm a reciprocal model of red cell production and destruction, show that anemia can be the result of either underproduction (a regenerative anemia or ineffective erythropoiesis) or increased destruction, and define parameters for distinguishing these 2 possibilities that are still widely used today.
Asunto(s)
Anemia/historia , Eritropoyesis , Hematología/historia , Anemia/fisiopatología , Envejecimiento Eritrocítico , Volumen de Eritrocitos , Historia del Siglo XX , HumanosAsunto(s)
Agammaglobulinemia/etiología , Inmunidad Celular , Inmunodeficiencia Combinada Grave/etiología , Adenosina Desaminasa/deficiencia , Adenosina Desaminasa/genética , Adenosina Desaminasa/inmunología , Agammaglobulinemia/genética , Agammaglobulinemia/inmunología , Preescolar , Consanguinidad , Femenino , Heterocigoto , Historia del Siglo XX , Humanos , Lactante , Inmunodeficiencia Combinada Grave/genética , Inmunodeficiencia Combinada Grave/inmunologíaRESUMEN
After receiving BS and MS degrees from the University of Washington in Seattle, I entered its new medical school in 1947, receiving an MD degree in 1951. After internship and residency, I obtained a 2-year postdoctoral fellowship in hematology under the guidance of Dr Clement Finch. The last 6 months of the fellowship were spent in London, England, at Dr Patrick Mollison's Blood Transfusion Research Unit. There I met and worked with Marie Cutbush (later Crookston) who has been a long-term friend. On returning to Seattle, I joined the faculty of the medical school and became the associate director of the Puget Sound Blood Center. There, I supervised the blood typing and cross-matching laboratory, introducing methods I had learned in London and measuring the effectiveness of various cross-matching procedures. My own research was largely directed toward human genetic polymorphism, and I wrote a textbook published in 1969, describing the biochemical structure, function, inheritance, and geographic distribution of the genetic markers. Subsequently, I discovered that 2 forms of inherited immunodeficiency disease were due to deficiencies of the enzymes adenosine deaminase and purine nucleoside phosphorylase. In 1979, I became the director of the blood center and was shortly afterwards elected to the National Academy of Sciences. I retired in 1987 and have spent most of the intervening years relearning to play the violin and exploring the wonders of chamber music.