mantle cell lymphoma | Lymphomas of the mantle

Lymphomas of the mantle



Summary
Lymphoma of the mantle is the result of the proliferation of B lymphocytes immature, originating from the area of the mantle on the periphery of the lymph node germinal centers. Cells express the CD5 molecule and pathognomonic way, translocation t (11: 14) which leads to the activation of cyclin D1. Lymphoma of the systemic coat has a very poor prognosis with a median survival of less than 5 years. Skin tumors are almost always associated with deep attacks and can be seen in 2 to 6% of the mantle of systemic lymphomas and in 15% of stage IV [1]. Skin locations can lead to the diagnosis of a systemic form [2], but exclusively dermatological forms are exceptional [1, 3, 4], so that the Lymphoma of the primitively skin coat does not appear in the classification WHO-EORTC of cutaneous lymphomas [5].

Lymphomas of the mantle


* Service of Pathology, CHU, 33000 Bordeaux; University Victor Segalen Bordeaux 2
Under the leadership of Martine Bagot, cutaneous lymphomas - under the aegis of the french study group
Cutaneous lymphomas

196. cutaneous lymphomas - under the aegis of the french study of Cutaneous Lymphoma group
19 very poor prognosis with a median survival of less than 5 years. Them
skin tumors are almost always associated with deep attacks
and can be seen in 2 to 6% of systemic lymphomas of the coat
and in 15% of stage IV [1]. Skin locations can lead
diagnosis of a systemic form [2], but only forms
dermatological are exceptional [1, 3, 4], so that the Lymphoma of the
originally skin coat does not appear in the classifi cation OMSEORTC
Cutaneous lymphomas [5].
Skin clinical presentation
Given the lack of reported cases, it is diffi cult to identify a presentation
evocative clinic of lymphoma of the primitively skin coat.
The lesions have an aspect common to all lymphocyte diseases
infi ltratives: plaques, nodules, sometimes ulcers, more or less grouped and variable size exceeding ten centimeters, hard to the
palpation and red color. It does not appear to exist in preferential locations.
It's the biopsy and immunostaining which lead to the diagnosis.
Pathology and molecular tools presentation
In the classical form, Cutaneous Lymphoma locations
the coat, whether they are primitive or secondary, realize an infi ltrat
dermal dense and monomorphic made up of small cells
average, with irregular nuclei or cleaved with a small nucleolus.
In the variant blastoid [6], the cells are larger, with
a big cleavage core and a prominent nucleolus; the mitotic index is high.
It's the monotonous nature of this proliferation to small lymphocytic B /.
medium-sized cells that should make the diagnosis (fi g. 1). This is confi rmed by Immunohistochemistry and molecular biology. The Immunohistochemistry profile
Lymphoma of the mantle is the following: markers
B-lymphocyte (CD19, CD20), Bcl2 +, CD5 +, CD10-, CD23 - positive
and especially expression of cyclin D1, which testifies to the translocation
t (11; 14) (q13; q32). This translocation between the IgH genes and cyclin D1
(CCND1) is confi reconfirmed by FISH molecular technique [7]. Forms
skin primitives being exceptional, any diagnosis of lymphoma of the
coat on a skin sample should make search a location
extracutanee primitive. The variant blastoid often sign such a localization
Cutaneous secondary [8].