title: Secondary B-cell acute lymphoblastic leukemia following Wilms' tumor: clinical and in vitro chemosensitivity studies.
abstract: We report the clinical features and in vitro chemosensitivity assay findings of a 13-year-old girl who developed secondary B-cell acute lymphoblastic leukemia (ALL) 7 years after a diagnosis of Wilms' tumor. The patient was treated using the Berlin - Frankfurt - Muenster (BFM) ALL chemotherapy protocol with poor response to initial therapy before succumbing to sepsis. An in vitro chemosensitivity assay on her peripheral blood lymphoblasts was performed while she was undergoing induction therapy and showed a high level of resistance to drugs commonly used for ALL therapy, e.g. steroids, anthracyclines, vincristine and L-asparaginase. The mechanism of chemoresistance was not elicited, but was probably not related to P-glycoprotein (P-gp) over-expression. We believe that the in vitro chemosensitivity assay is a good indicator of cellular response to chemotherapy and may provide reliable information for the basis of the selection of drugs to be used for the treatment of similarly rare patients rather than relying on "standard" protocols.
authors.affiliation: Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Malaysia. hany@um.edu.my
authors.forename: H
authors.initials: H
authors.lastname: Ariffin
authors.forename: T
authors.initials: T
authors.lastname: Muthukkumaran
authors.forename: J
authors.initials: J
authors.lastname: Stanslas
authors.forename: A R
authors.initials: AR
authors.lastname: Sabariah
authors.forename: N
authors.initials: N
authors.lastname: Veerasekaran
authors.forename: H P
authors.initials: HP
authors.lastname: Lin
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002051:Burkitt Lymphoma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002470:Cell Survival / Q000187:drug effects; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D019008:Drug Resistance, Neoplasm; D004354:Drug Screening Assays, Antitumor; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D066298:In Vitro Techniques; D007680:Kidney Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D016609:Neoplasms, Second Primary / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D018805:Sepsis / Q000188:drug therapy; D009396:Wilms Tumor / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
chemical_list: D000970:Antineoplastic Agents