Agnogenic myeloid metaplasia in children
A. A. Mallouh and A. R. Sa'di
Pediatric Services, Saudi Aramco-Dhahran Health Center, Dhahran, Saudi Arabia.
Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder
characterized by splenomegaly, leukoerythroblastosis, extramedullary
hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone
marrow. It is a disease of adults, with only one case report in the
pediatric literature. The symptoms of the patient in this case clearly fit
the diagnostic criteria of this disease. Myelofibrosis in children is
usually of the acute type, presenting in infancy and running a fulminant,
fatal course with minimal or mild splenomegaly. Red blood cells are usually
normal on morphologic examination. Three infants, including two siblings,
presented at ages 9, 10, and 16 months with clinical and laboratory
findings consistent with agnogenic myeloid metaplasia. The occurrence of
the disease in these siblings is suggestive of an autosomal recessive mode
of inheritance.
