Hepatoma and Peliosis Hepatis Developing in a Patient with Fanconi's Anemia

Abstract
FANCONI'S anemia is a rare genetic disorder in which pancytopenia develops — usually in childhood. The most frequent nonhematologic manifestations include increased pigmentation, skeletal deformities, mental retardation, dwarfism, microcephaly, renal anomalies and hypogenitalism. Death from hemorrhage, anemia or sepsis within one to five years after onset was the natural history in the past. Therapy with testosterone and corticosteroids1 and more recently with oxymetholone2 has produced life-prolonging remissions. Patients with Fanconi's anemia and members of their families have a very high rate of acute leukemia3 4 5; the development of squamous-cell carcinoma of the skin6 or gingivae7 in such cases has been . . .

This publication has 7 references indexed in Scilit: