We report on 2 related children, a boy and a girl, from a large Turkish clan. 
Their parents are both first cousins and have several common ancestors. Both 
children have tyrosinase-positive oculocutaneous albinism, recurrent bacterial 
infections, granulocytopenia, intermittent thrombopenia, and microcephaly, a 
protruding midface, rough and projecting hair, and mild mental retardation. 
Chromosomes are normal. Metabolic disorders were excluded. None of 14 well-known 
types of albinism, including Hermansky-Pudlak syndrome and Chediak-Higashi 
syndrome, nor any other genetic syndrome, characterizes our patients 
sufficiently. Thus, this combination of symptoms is considered a new autosomal 
recessive syndrome.