Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Monosomy 7 variant childhood chronic myelogenous leukemia (CML) is a rare, fatal leukemia that usually terminates in blast crisis. We report successful marrow transplantation in a patient with this disease using his one HLA locus mismatched mother. Initially following transplant the patient exhibited mixed hematopoietic chimerism, cytogenetic relapse, and clinical relapse of leukemia. However, following recovery from a period of hydroxyurea-induced aplasia, marrow studies showed elimination of the mixed chimerism, absence of the 45,XY,-7 leukemic clone and full engraftment with donor marrow (46,XX). The ability of hydroxyurea to eliminate mixed chimerism in favor of donor hematopoiesis and to eliminate the persistent leukemic clone in this patient with CML suggests treatment approaches worthy of future investigation.
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PMID:Establishment of donor hematopoiesis after hydroxyurea-induced aplasia following allograft failure in a patient with monosomy 7 variant of childhood chronic myelogenous leukemia. 265 Jul 93

The EUTOS Long-Term Survival score was tested in 350 children with chronic myeloid leukemia in first chronic phase treated with imatinib and registered in the International Registry for Childhood Chronic Myeloid Leukemia. With a median follow up of 3 years (range, 1 month to 6 years) progression and/or death (whichever came first) occurred in 23 patients. For the entire cohort of patients the 5-year progression-free survival rate was 92% (95% CI: 87%-94%) and the 5-year survival accounting for chronic myeloid leukemia deaths was 97% (95% CI: 94%-99%). Of the 309 patients allocated to low (n=199), intermediate (n=68) and high (n=42) risk groups by the EUTOS Long-Term Survival score, events (progression and/or death) occurred in 6.0%, 8.8% and 26.2%, respectively. Estimates of the 5-year progression-free survival rates according to these three risk groups were 96% (95% CI: 92%-98%), 88% (95% CI: 76%-95%) and 67% (95% CI: 48%-81%), respectively. Differences in progression-free survival according to these risk groups were highly significant (P<0.0001, overall). The EUTOS Long-Term Survival score showed better differentiation of progression-free survival than the Sokal (<45 years), Euro and EUTOS scores in children and adolescents with chronic myeloid leukemia and should be considered in therapeutic algorithms. (Trial registered at: www.clinicaltrials.gov NCT01281735).
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PMID:Prognostic discrimination based on the EUTOS long-term survival score within the International Registry for Chronic Myeloid Leukemia in children and adolescents. 2883 93