FAMILIAL INFANTILE MYELOFIBROSIS AS AN AUTOSOMAL RECESSIVE DISORDER: Preponderance among Children from Saudi Arabia

摘要

Familial infantile myelofibrosis is a rare clonal myeloproliferative stem cell disorder also known as agnogenic myeloid metaplasia (AMM) and acute panmyelosis with myelofibrosis (APMF). The classic triad of this disorder consists of myelofibrosis, extramedullary hematopoiesis, and leucoerythrob-lastosis with dacrocytes. The mode of inheritance remains unproven. Sieff and Malleson reported a British family with 2 affected among a total of 3 children, a brother and sister, and postulated an autosomal recessive mode of inheritance [1].