Biologic features and treatment outcome of secondary acute lymphoblastic leukemia--a review of 101 cases.

Ann Oncol. 2008 May 7;

Authors: Shivakumar R, Tan W, Wilding GE, Wang ES, Wetzler M

BACKGROUND: Secondary acute lymphoblastic leukemia (sALL) is a rare disease and its biologic features are not well characterized. PATIENTS AND METHODS: We describe a cohort of seven patients and discuss 94 additional cases from the literature for whom biological parameters were described. Cases with incomplete data were excluded. RESULTS: Hodgkin's disease (HD) was more common in the 18-59 age group while breast and prostate cancers were prevalent only in the >/=18-year-old patients. The time interval to develop sALL was similar among all age groups but was significantly longer for HD and neuroblastoma primary diagnoses and sALL with complex karyotype. T-cell immunophenotype was more common in the <18 age group. Complete remission was infrequent in the >/=60 age group. The overall survival was poor for all sALL regardless of age, primary diagnoses, cytogenetic subgroups, or immunophenotype. Allogeneic transplantation most probably represents the only chance of cure. CONCLUSION: Better identification of prognostic factors to prevent the occurrence of sALL is indicated.

PMID: 18467310 [PubMed - as supplied by publisher]

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Pathology of the facial nerve.

Neuroimaging Clin N Am. 2008 May;18(2):309-20

Authors: Veillon F, Taboada LR, Eid MA, Riehm S, Debry C, Schultz P, Charpiot A

A good examination in facial nerve imaging (CT or MR imaging) depends on a good knowledge of anatomy. Two clinical situations must be considered: imaging of patients with or without facial palsy. CT and MR imaging are very useful when the symptoms are atypical or progressive: MR imaging gives very good information about the facial nerve inflammation but may also discover a schwannoma, a hemangioma, a meningioma, or a primitive or secondary cholesteatoma. In malignant tumors of the parotid gland, a study of the fallopian canal must always be performed to delineate an extension in the mastoid, tympanic, or intrameatic parts. In some rare cases, a metastasis in the temporal bone may occur, especially in the region of the geniculate ganglion. Particular attention must be paid to children with facial palsy, considering the possibility of a histiocytosis or metastasis of a neuroblastoma.

PMID: 18466834 [PubMed - in process]

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Mutations and treatment outcome in cytogenetically normal acute myeloid leukemia.

N Engl J Med. 2008 May 1;358(18):1909-18

Authors: Schlenk RF, Döhner K, Krauter J, Fröhling S, Corbacioglu A, Bullinger L, Habdank M, Späth D, Morgan M, Benner A, Schlegelberger B, Heil G, Ganser A, Döhner H,

BACKGROUND: Mutations occur in several genes in cytogenetically normal acute myeloid leukemia (AML) cells: the nucleophosmin gene (NPM1), the fms-related tyrosine kinase 3 gene (FLT3), the CCAAT/enhancer binding protein alpha gene (CEPBA), the myeloid-lymphoid or mixed-lineage leukemia gene (MLL), and the neuroblastoma RAS viral oncogene homolog (NRAS). We evaluated the associations of these mutations with clinical outcomes in patients. METHODS: We compared the mutational status of the NPM1, FLT3, CEBPA, MLL, and NRAS genes in leukemia cells with the clinical outcome in 872 adults younger than 60 years of age with cytogenetically normal AML. Patients had been entered into one of four trials of therapy for AML. In each study, patients with an HLA-matched related donor were assigned to undergo stem-cell transplantation. RESULTS: A total of 53% of patients had NPM1 mutations, 31% had FLT3 internal tandem duplications (ITDs), 11% had FLT3 tyrosine kinase-domain mutations, 13% had CEBPA mutations, 7% had MLL partial tandem duplications (PTDs), and 13% had NRAS mutations. The overall complete-remission rate was 77%. The genotype of mutant NPM1 without FLT3-ITD, the mutant CEBPA genotype, and younger age were each significantly associated with complete remission. Of the 663 patients who received postremission therapy, 150 underwent hematopoietic stem-cell transplantation from an HLA-matched related donor. Significant associations were found between the risk of relapse or the risk of death during complete remission and the leukemia genotype of mutant NPM1 without FLT3-ITD (hazard ratio, 0.44; 95% confidence interval [CI], 0.32 to 0.61), the mutant CEBPA genotype (hazard ratio, 0.48; 95% CI, 0.30 to 0.75), and the MLL-PTD genotype (hazard ratio, 1.56; 95% CI, 1.00 to 2.43), as well as receipt of a transplant from an HLA-matched related donor (hazard ratio, 0.60; 95% CI, 0.44 to 0.82). The benefit of the transplant was limited to the subgroup of patients with the prognostically adverse genotype FLT3-ITD or the genotype consisting of wild-type NPM1 and CEBPA without FLT3-ITD. CONCLUSIONS: Genotypes defined by the mutational status of NPM1, FLT3, CEBPA, and MLL are associated with the outcome of treatment for patients with cytogenetically normal AML.

PMID: 18450602 [PubMed - indexed for MEDLINE]

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Fibrin glue application in the management of refractory chylous ascites in children.

J Pediatr Gastroenterol Nutr. 2008 Apr;46(4):478-81

Authors: Zeidan S, Delarue A, Rome A, Roquelaure B

The purpose of this retrospective review of the charts of 6 children who underwent surgical treatment of chylous ascites refractory to conservative measures between 1993 and 2006 was to evaluate the efficiency of fibrin glue application for control of lymph leakage. Five children had postoperative chylous ascites (neuroblastoma, 4; cystic lymphangioma, 1) and 1 had a congenital malformation. Surgical exploration revealed large areas of diffuse lymphatic leakage in all of the patients. Lymphatic fistula was not identified intraoperatively in any patient. Ingestion of lipophilic dye in a concentrated fatty meal was not helpful in locating a lymph fistula. Absorbable mesh was used in association with glue application in the last 3 patients treated. Control of ascites was achieved immediately in 2 patients and within 3 weeks in 2 patients. Repeat surgery was required in the remaining 2 patients. The mean follow-up time was 4.3 years. One patient died of tumor recurrence 12 months after surgical treatment without relapse of the ascites. Two mild late recurrences were observed at 6 and 11 months after surgery and were managed conservatively. The findings of this study show that fibrin glue application on absorbable mesh after dissection of the leakage zones is easy, safe, and effective. We recommend that surgery with glue application be repeated until control of ascites is achieved. We suggest fibrin glue application as a preventive measure against postoperative chylous ascites.

PMID: 18367970 [PubMed - indexed for MEDLINE]