| 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. |
| 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. |
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