Palpable mantel cell lymphoma in the beast

Citation
N. Boullanger et al., Palpable mantel cell lymphoma in the beast, PRESSE MED, 30(4), 2001, pp. 163-165
Citations number
12
Categorie Soggetti
General & Internal Medicine","Medical Research General Topics
Journal title
PRESSE MEDICALE
ISSN journal
07554982 → ACNP
Volume
30
Issue
4
Year of publication
2001
Pages
163 - 165
Database
ISI
SICI code
0755-4982(20010203)30:4<163:PMCLIT>2.0.ZU;2-L
Abstract
BACKGROUND: Extranodal involvement is not unusual in mantle cell lymphoma ( MCL) which accounts for 6% of non-Hodgkin's lymphomas, Simultaneous localiz ation in the eyelid and in the breast, as observed in our case, is however exceptional. CASE REPORT: Chronic lymphoid leukemia (CLL) was suspected in a 71-year-old woman with asthenia, a cervical and axillary nodal enlargement and elevate d lymphocyte count Blood immunoflow cytometry analysis, occurrence of rapid ly growing tumors involving the two breasts and eyelids and cytogenetic and molecular features led to the diagnosis of MCL. A very good partial remiss ion was obtained with second-line polychemotherapy composed of cytarabin, c isplatin and dexamethasone, but lasted only 3 months after the end of 6 cyc les. DISCUSSION: Primary breast and eyelid lymphomas are rare. Such localization s are exceptional in MCL and are signs of aggressive disease. Before extra- nodal involvement, MCL. may simulate banal CLL. Therefore, systematic immun ohistochemistry and if necessary molecular analysis are useful for early di agnosis of MCF. Prognosis is particularly poor. Conventional chemotherapy c annot provide cure of MCL and median survival is 48 months. For this reason , high-dose chemotherapy with stem cell graft has to be discussed in young patients. MCL is currently characterized by Bcl 1 rearrangement t(11-14) tr anslocation and cyclin D 1 overexpression among small B-cell lymphomas in r ecent REAL- and WHO-classifications.