Rossbach H C, Grana N H, Chamizo W, Barrios N J, Barbosa J L
Division of Pediatric Hematology/Oncology, All Children's Hospital, St. Petersburg, Florida 33701, USA.
J Pediatr Hematol Oncol. 1996 May;18(2):213-5. doi: 10.1097/00043426-199605000-00025.
Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by marrow fibrosis, extramedullary hematopoiesis, splenomegaly, and leukoerythroblastosis with abnormalities of red blood cell morphology. This is rarely encountered in children. No conventional curative therapy is known; however, allogeneic bone marrow transplantation (BMT) may eradicate the underlying stem cell abnormality with subsequent normal hematopoiesis.
We report a 3-year-old Arab boy who had AMM and who had normal marrow hematopoiesis and markedly reduced fibrosis after high-dose chemotherapy and matched sibling BMT.
Allogeneic BMT offers a potential cure for patients with agnogenic myeloid metaplasia. A preparatory regimen containing busulfan and cyclophosphamide appears promising for patients with the disease.
