OBJECTIVE: To correlate clinical presentation and therapeutic outcomes in @entity1 with a diagnosis of @entity1778 . METHODS: The medical records of 14 @entity1 who fulfilled the International @entity1778 Working Group Criteria for a diagnosis of @entity1778 ( @entity1778 ) presenting to a single institution were reviewed, and their clinical status at September 2000 was documented. RESULTS: The most common presenting features were @entity828 and @entity3329 . Fifty per cent of cases presented in the first year of life. Nine of 14 @entity1 initially received chemotherapy otherwise used in the treatment of @entity981 with no apparent benefit. All six @entity1 who received conditioning therapy with chemotherapy alone, followed by allogeneic bone marrow transplant (BMT), are in complete remission at a median follow-up duration of 12 months (range 5-91 months). Five of six @entity1 surviving post-allogeneic BMT received marrow from an unrelated donor. Only one of seven @entity1 who did not receive BMT survived long-term. CONCLUSION: @entity1 with a diagnosis of @entity1778 should be treated with allogeneic BMT as soon as a suitable donor is found. The role of anti-leukaemic therapy in this disease, prior to BMT, requires further investigation in the context of a multicentre clinical trial.

Allogeneic bone marrow transplant improves outcome for XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Children'] @entity3329 :: ('MESH:D008206', 'Disease') :: ['lymphadenopathy'] @entity981 :: ('MESH:D054198', 'Disease') :: ['acute myeloid or lymphoblastic leukaemia'] @entity828 :: ('MESH:C535727', 'Disease') :: ['hepatosplenomegaly'] @entity1778 :: ('MESH:D007938', 'Disease') :: ['juvenile myelomonocytic leukaemia', 'Juvenile Myelomonocytic Leukaemia', 'JMML']
@entity1778 :: (MESH:D007938,Disease) :: ['juvenile myelomonocytic leukaemia']
