Blood, 1955, Vol. 10, No. 6, pp. 623-632.
© 1955 American Society of Hematology, Inc.
New Data on Microdrepanocytic Disease
E. SILVESTRONI 1 and
I. BIANCO 1
1 Istituto di Clinica Medica Generale e Terapia Medica (Director, Professor G.
Di Guglielmo), University of Rome, Italy, and the Istituto di Genetica (Director, Professor G. Montalenti), University of Naples, Italy.
1. Geographic, clinical and genetic data of 35 new cases of microdrepanocytic
disease are presented.
2. These data suggest that sickle cell trait and microdrepanocytic disease are
not infrequent in the eastern district of Sicily, perhaps with some small foci of
sicklemic subjects.
3. The clinical history of adult patients and the personal examination of some
cases of microdrepanocytic disease in children show the existence of mild forms
of the disease and of forms appearing late in life.
4. From the clinical viewpoint, this series shows several interesting findings
in the patients with microdrepanocytic disease: painful osteoarticular crises are
inconstantly present; splenomegaly may undergo a spontaneous lessening during
the course of the disease, analogous to that which occurs in sickle cell anemia; in
women with microdrepanocytic disease miscarriages are frequent and successful
pregnancies and births are extremely rare.
5. Hematologic studies reveal hypochromic anemia, leukocytosis, nucleated
erythrocytes in the circulating blood, marked abnormalities in the morphology
of the erythrocytes, and decreased cell fragility to hypotonic saline.
6. Genetic studies of the new cases again show the simultaneous presence in
the patients with microdrepanocytic disease of the genes responsible for microcythemia and sickle cell trait.
Submitted on March 18, 1954
Accepted on March 31, 1954
