Ida Bianco Silvestroni. From red cell osmotic fragility to molecular biology of globin genes. A long, productive life with thalassemia.

نویسنده

  • M Cazzola
چکیده

his issue of Haematologica contains an interesting paper by Ida Bianco (Figure 1) and co-workers on the molecular diagnosis of a-thalassemia, one of the innumerable contributions (by no means the last) that this gentle, inexhaustible lady has made to our present knowledge of thalassemia. I met her last spring in Naples, of course at a meeting on thalassemia, and, like the rest of the audience, I was fascinated by her up-to-date competency and enthusiasm. Ida Bianco was born in Rome on July 30, 1917, and received her M.D. from the University of Rome School of Medicine in 1941. While doing post-doctoral work with Dr. Ezio Silvestroni (later her husband) (Figure 2), she decided to reproduce some experiments by a Czech investigator, Hoffman, who had described a decrease in osmotic fragility in some patients with cancer (presumably because of the associated iron deficiency anemia). Bianco and Silvestroni employed the Simmel test in 50 cancer patients and 50 normal controls. They found that 2 patients and 4 controls presented a clear decrease in osmotic fragility and erythrocytosis at the same time. Based on these preliminary findings, they decided to use a modified Simmel test (the Silvestroni-Bianco red cell fragility test) for a screening of the normal population. Within a group of 400 normal individuals, 7 were found to have decreased osmotic fragility, anemia, microcytosis, hypochromia and additional red cell morphological abnormalities. Most importantly, this condition was shown to be hereditary since in the 3 cases studied at least one of the parents was shown to carry the same disorder. On November 26, 1943, Bianco and Silvestroni presented their work on Microcythemia as an oral communication at a meeting of the Rome Medical Society. They also recognized that microcythemia shared many similarities with the Rietti-Greppi-Micheli syndrome (hemolytic jaundice with decreased red cell fragility). Thomas Cooley had described his series of cases of splenomegaly in children, with anemia IDA BIANCO SILVESTRONI. FROM RED CELL OSMOTIC FRAGILITY TO MOLECULAR BIOLOGY OF GLOBIN GENES. A LONG, PRODUCTIVE LIFE WITH THALASSEMIA editorial Haematologica 1996; 81:385-6

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عنوان ژورنال:
  • Haematologica

دوره 81 5  شماره 

صفحات  -

تاریخ انتشار 1996