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Written2002-01Antonio Cuneo
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

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Atlas_Id 2116

Clinics and Pathology

Disease Splenic marginal zone B-cell lymphoma (MZBCL)
Phenotype / cell stem origin CD19+; CD5-; CD22+; CD23-; CD10-; CD25-/+; CD38+; CD11c-; CD103-; FMC7+; surface Ig bright+. The transformed cell represents an IgM+/IgD+ B-lymphocyte deriving from the marginal zone.
Epidemiology The translocation is rare (<1% of B-cell chronic lymphoid neoplasias)
Clinics These patients may feature a relatively aggressive clinical course as compared with other cases of splenic MZBCL
Cytology Usually, the abnormal lymphocytes in the PB smear are morphologically heterogeneous. The majority of cells are >14 mm in size, with pleomorphism of nuclear shapes, fine chromatin structure and distinct nucleoli. Some lymphocytes with short villi may be present along with larger lymphoid cells and rare prolymphocyte-like cells.
Pathology The bone biopsy may show B-lymphoid cells of intermediate size with an interstitial infiltration pattern. An intrasinusoidal pattern of growth was also noted.
The spleen specimens display a nodular lymphoid infiltrate of the white pulp by small lymphocytes mixed with larger blast cells with clear cytoplasm, centered on polyclonal follicle centers. Red pulp involvement may occur.
Treatment The patients may show partial responses to alkylating agent and to multiagenet chemotherapy.
Evolution Transformation into high-grade lymphoma was reported.


Cytogenetics Morphological The tranlocation was described as a balanced t(11;14)(p11;q32).
Cytogenetics Molecular To detect the 14q32 break, the cos-Ca1, cosIg6 and cos3/64 and YAC Y6 were used. Splitting of cosmid signals and Y6 signals has been detected, indicating a break downstream of the IgVH sequences, in the region flanked by cos3/64 and Y6. The gene involved at the 11p11 band is presently unknown.
Additional anomalies del(7)(q22q32); +12; del(17)(p12). In one case a 7q- chromosome was the primary anomaly, the t(11;14) having found at the time of histologic transformation into high grade lymphoma


Nonrandom chromosome abnormalities in lymphoma.
Bloomfield CD, Arthur DC, Frizzera G, Levine EG, Peterson BA, Gajl-Peczalska KJ
Cancer research. 1983 ; 43 (6) : 2975-2984.
PMID 6850608
A novel recurrent translocation t(11;14)(p11;q32) in splenic marginal zone B cell lymphoma.
Cuneo A, Bardi A, Wlodarska I, Selleslag D, Roberti MG, Bigoni R, Cavazzini F, De Angeli C, Tammiso E, del Senno L, Cavazzini P, Hagemeijer A, Castoldi G
Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 2001 ; 15 (8) : 1262-1267.
PMID 11480569
The Non-Hodgkin's Lymphomas. Pp 277-308.
WhangPeng J, Knutsen T, in: Magrath I ed
Arnold, London. 1997.


This paper should be referenced as such :
Cuneo, A
Atlas Genet Cytogenet Oncol Haematol. 2002;6(2):127-127.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Translocations implicated (Data extracted from papers in the Atlas)


External links

Mitelman databaset(11;14)(p11;q32)
arrayMap (UZH-SIB Zurich)[select an item]
REVIEW articlesautomatic search in PubMed
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