The chronic myeloproliferative disorders consist of chronic myelogenous leukemia, polycythemia vera (p. vera), primary myelofibrosis, essential thrombocythemia, chronic neutrophilic leukemia, and chronic eosinophilic leukemia. All of these disorders involve dysregulation at the multipotent hematopoietic stem cell (CD34), with one or more of the following shared features:
Overproduction of one or several blood elements with dominance of a transformed clone.
Hypercellular marrow/marrow fibrosis...
Added text to state that in a study of adolescents with stage III primary mediastinal large B-cell lymphoma treated with FAB/LMB-96 therapy, the 5-year event-free survival (EFS) was 66%, versus 85% for adolescents with nonmediastinal diffuse large B-cell lymphoma (cited Gerrard et al. as reference 24 and level of evidence 2A). Also added text about a single-arm study in adults that showed excellent disease-free survival utilizing the DA-EPOCH-R regimen (dose-adjusted etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone, and rituximab; usually six cycles) with filgrastim and no radiation therapy. The 5-year EFS was 93% and overall survival (OS) was 97% (cited Dunleavy et al. as reference 25 and level of evidence 2A).
Revised text to state that the genetic hallmark of adult follicular lymphoma, the translocation of t(14;18)(q32;q21) involving BCL2, is typically not detectable in pediatric follicular lymphoma (cited Liu et al. as reference 51). Also added text to state that molecular alterations observed in pediatric follicular lymphoma include translocations of the immunoglobulin locus and IRF4, losses of regions of chromosome 1p, and mutations of TNFSFR14 on chromosome 1p (cited Launay et al. as reference 52).
Revised text to state that pediatric follicular lymphoma predominantly occurs in males, is associated with a high proliferation rate, and is more likely to be localized disease; in contrast, adult follicular lymphoma usually presents as disseminated disease with a relatively low proliferation rate.
Treatment Option Overview
Added text to state that the International Working Group has updated their response criteria for malignant lymphoma to include positron emission tomography, immunohistochemistry, and flow cytometry data.
Added Bakhshi et al. as reference 21.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.
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September 04, 2014
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