Successful allogeneic bone marrow transplantation for agnogenic myeloid metaplasia in a 3-year-old boy.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Rossbach
H C
HC
Division of Pediatric Hematology/Oncology, All Children's Hospital, St. Petersburg, Florida 33701, USA.
Grana
N H
NH
Chamizo
W
W
Barrios
N J
NJ
Barbosa
J L
JL
D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D006410:Hematopoiesis / Q000502:physiology; D006801:Humans; D008297:Male; D055728:Primary Myelofibrosis / Q000601:surgery*