Central nervous system (CNS) involvement in like skin involvement with epidermolysis. A Ki-1/CD30 lymphoma is extremely rare, in con-lymph node biopsy was compatible with Ki-1/ trast to the frequent involvement in other types CD30 anaplastic large cell lymphoma (ALCL). of pediatric non-Hodgkin's lym
Prognostic significance of p80 and visceral involvement in childhood CD30 anaplastic large cell lymphoma (ALCL)
✍ Scribed by Massimino, M. ;Spreafico, F. ;Luksch, R. ;Giardini, R.
- Publisher
- John Wiley and Sons
- Year
- 2001
- Tongue
- English
- Weight
- 163 KB
- Volume
- 37
- Category
- Article
- ISSN
- 0098-1532
- DOI
- 10.1002/mpo.1176
No coin nor oath required. For personal study only.
✦ Synopsis
Abstract
Background
Between 1976 and 1998, CD30+anaplastic large cell lymphoma (ALCL) was diagnosed in 44 children (28 males, 16 females, age range 2.7–16.1 years, median 10). Up to 1993, 32 such children were treated according to a common chemotherapeutic protocol that stratified patients according to stage, without considering presenting features. Thereafter, patients presenting with visceral (lung, spleen, liver, gastro‐intestinal tract) or mediastinal involvement were assigned to a high‐risk treatment protocol with induction intensification. The generation of these two risk‐groups was the result of a retrospective analysis of clinical risk factors for therapy failure as previously reported [Massimino M, Gasparini M, Giardini R, Ann Oncol 1995;6:915–920]. Considering the whole cohort of patients divided into group A—21/22 evaluable patients with visceral/mediastinal involvement, and group B—22 evaluable patients, with other ALCL location—disease‐free survival (DFS) and survival (S) at 5 years were 57 and 58% for group A, and 83 and 100% (94% at 6 years) for group B, respectively.
Procedure
We tested 15/21 cases of group A, and 18/22 of group B for p80 immunoreactivity in order to investigate a possible correlation between ALCL locations and NPM‐ALK expression.
Results
Thirteen of 15 specimens in group A and 17/18 in group B were positive for p80.
Conclusions
It is impossible to conclude anything about p80 positivity based on a series of 33/44 patients with childhood ALCL, neither about over‐all prognosis nor about the role of visceral involvement. In adults, NPM‐ALK protein expression is a favourable prognostic factor. Med Pediatr Oncol 2001;37:97–102. © 2001 Wiley‐Liss, Inc.
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## Abstract The t(2;5)(p23;q35) translocation results in the formation of a unique chimeric NPM‐ALK protein (p80). Expression of this protein is considered to be one of the clinical features of anaplastic large cell lymphoma (ALCL). Recently recognized as one clinical subtype of ALCL, the small cel