Mediastinal large-cell lymphoma with sclerosis (MLCLS).

In a retrospective analysis encompassing a 14 year period (1978-92), 22 patients (age range 19-71, median 30 years) were identified as having mediastinal large-cell lymphoma with sclerosis on the basis of clinical and pathological features. At presentation, 15/22 had 'bulky' disease and 11...

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Main Authors:	Rohatiner, A. Z., Whelan, J. S., Ganjoo, R. K., Norton, A. J., Wilson, A., Lister, T. A.
Format:	Online
Language:	English
Published:	1994
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1968881/

id	pubmed-1968881
recordtype	oai_dc
spelling	pubmed-19688812009-09-10 Mediastinal large-cell lymphoma with sclerosis (MLCLS). Rohatiner, A. Z. Whelan, J. S. Ganjoo, R. K. Norton, A. J. Wilson, A. Lister, T. A. Research Article In a retrospective analysis encompassing a 14 year period (1978-92), 22 patients (age range 19-71, median 30 years) were identified as having mediastinal large-cell lymphoma with sclerosis on the basis of clinical and pathological features. At presentation, 15/22 had 'bulky' disease and 11/22 had evidence of superior vena caval obstruction. Thirteen patients had stage II disease (6,II; 7,IIE), nine presented with stage IV disease. Complete remission (CR) was achieved in only 4/22 patients with the initial adriamycin-containing regimen. 'Good partial remission' (no clinical evidence of disease, minimal abnormalities of uncertain significance on radiological investigation) was achieved in a further seven patients and 'poor partial remission' (a reduction in measurable disease > 50%) in four, giving an overall response rate of 15/22 (68%). One patient died within 48 h of arrival at the hospital; 16 of the 17 remaining patients in whom anything less than CR was achieved subsequently received additional, alternative treatment (one chemotherapy, six mediastinal radiotherapy, nine both treatment modalities) but in only 2/16 did this result in any further degree of response. With a median follow-up of 5 1/2 years, 10/22 patients remain well without progression between 6 months and 14 years (5/6 in whom CR was eventually achieved and 5/11 in whom only partial remission was ever documented). The seven patients in whom the initial treatment demonstrably failed have all died. These results suggest that a proportion of patients with this rare subtype of high-grade B-cell lymphoma may be cured by chemotherapy alone and that the presence of a residual mediastinal mass after treatment does not necessarily imply treatment failure. However, patients in whom the initial chemotherapy fails have a very grave prognosis. 1994-03 /pmc/articles/PMC1968881/ /pubmed/8123496 Text en
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Rohatiner, A. Z. Whelan, J. S. Ganjoo, R. K. Norton, A. J. Wilson, A. Lister, T. A.
spellingShingle	Rohatiner, A. Z. Whelan, J. S. Ganjoo, R. K. Norton, A. J. Wilson, A. Lister, T. A. Mediastinal large-cell lymphoma with sclerosis (MLCLS).
author_facet	Rohatiner, A. Z. Whelan, J. S. Ganjoo, R. K. Norton, A. J. Wilson, A. Lister, T. A.
author_sort	Rohatiner, A. Z.
title	Mediastinal large-cell lymphoma with sclerosis (MLCLS).
title_short	Mediastinal large-cell lymphoma with sclerosis (MLCLS).
title_full	Mediastinal large-cell lymphoma with sclerosis (MLCLS).
title_fullStr	Mediastinal large-cell lymphoma with sclerosis (MLCLS).
title_full_unstemmed	Mediastinal large-cell lymphoma with sclerosis (MLCLS).
title_sort	mediastinal large-cell lymphoma with sclerosis (mlcls).
description	In a retrospective analysis encompassing a 14 year period (1978-92), 22 patients (age range 19-71, median 30 years) were identified as having mediastinal large-cell lymphoma with sclerosis on the basis of clinical and pathological features. At presentation, 15/22 had 'bulky' disease and 11/22 had evidence of superior vena caval obstruction. Thirteen patients had stage II disease (6,II; 7,IIE), nine presented with stage IV disease. Complete remission (CR) was achieved in only 4/22 patients with the initial adriamycin-containing regimen. 'Good partial remission' (no clinical evidence of disease, minimal abnormalities of uncertain significance on radiological investigation) was achieved in a further seven patients and 'poor partial remission' (a reduction in measurable disease > 50%) in four, giving an overall response rate of 15/22 (68%). One patient died within 48 h of arrival at the hospital; 16 of the 17 remaining patients in whom anything less than CR was achieved subsequently received additional, alternative treatment (one chemotherapy, six mediastinal radiotherapy, nine both treatment modalities) but in only 2/16 did this result in any further degree of response. With a median follow-up of 5 1/2 years, 10/22 patients remain well without progression between 6 months and 14 years (5/6 in whom CR was eventually achieved and 5/11 in whom only partial remission was ever documented). The seven patients in whom the initial treatment demonstrably failed have all died. These results suggest that a proportion of patients with this rare subtype of high-grade B-cell lymphoma may be cured by chemotherapy alone and that the presence of a residual mediastinal mass after treatment does not necessarily imply treatment failure. However, patients in whom the initial chemotherapy fails have a very grave prognosis.
publishDate	1994
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1968881/
_version_	1611399897736544256

Mediastinal large-cell lymphoma with sclerosis (MLCLS).

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