Biclonal splenic marginal zone lymphoma with T cell-rich background and aggressive transformation to large cell lymphoma
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Marginal zone B cell lymphomas (MZL) are biologically heterogeneous, rarely demonstrating biclonality, complex cytogenetic abnormalities, or T cell predominance. We report a case of biclonal splenic MZL, T cell-rich variant with an abnormal karyotype that progressed to large B cell lymphoma. A 74-year-old female presented with pancytopenia, weight loss, fever, and splenomegaly. Microscopically, the spleen revealed an extensive, vaguely nodular lymphoid proliferation, composed of small lymphocytes, majority of which were reactive T cells. B cells were mostly small and < 5% of total lymphocytes. Focal follicular dendritic cell networks were present, but germinal centers were absent. Flow cytometric analysis revealed two distinct CD5 and CD10 negative B cell clones, one kappa positive and one lambda positive. Conventional cytogenetic analysis revealed the following abnormal karyotype: 47,XX,add(7)(q36),del(7)(q22),add(21)(p11.2),+mar/46,XX. Immunoglobulin heavy chain gene rearrangement showed two monoclonal peaks of different magnitude, consistent with biclonality. Overall, these features favored a low-grade splenic MZL. The staging bone marrow biopsy was normocellular with few interstitial lymphoid aggregates, composed of small lymphocytes, consistent with minimal involvement by low-grade B cell lymphoma. The patient improved without adjuvant chemotherapy; however, 12 months later, she developed anemia and lymphocytosis. Subsequent bone marrow biopsy showed extensive involvement by a large B cell lymphoma and complex karyotype with the previously identified abnormalities, as well as additional numerical and structural aberrations, consistent with cytogenetic evolution and biclonal gene rearrangement. These findings were consistent with transformation. This case demonstrates a unique pathological presentation of splenic MZL with disease progression, highlighting the importance of an integrated approach for lymphoma classification and the difficulties in diagnosing such cases.
KeywordsMarginal zone lymphoma T cell-rich variant
We would like to acknowledge Dr. Mazen Toushan for his help with taking the gross pictures.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
- 1.Zinzani PL (2012) The many faces of marginal zone lymphoma. ASH Educ Progr B 1:426–432Google Scholar
- 3.Miranda RN, Cousar JB, Hammer RD, Collins RD, Vnencak-Jones CL (1999) Somatic mutation analysis of IgH variable regions reveals that tumor cells of most parafollicular (monocytoid) B-cell lymphoma, splenic marginal zone B-cell lymphoma, and some hairy cell leukemia are composed of memory B lymphocytes. Hum Pathol 30:306–312CrossRefGoogle Scholar
- 6.Swerdlow SH, Campo E, Harris NL et al (2017) Splenic marginal zone lymphoma. In: Swerdlow SH, Campo E, Harris NL et al (eds) WHO classification of tumours of haematopoietic and lymphoid tissues, revised 4th edn. International Agency for Research on Cancer, Lyon, pp 223–225Google Scholar
- 11.Delville JP, Heimann P, Housni HE, Boutriaux M, Jeronnez A, Remmelink M, Lasudry J, Pradier O, Kentos A (2007) Biclonal low grade B- celllymphoma confirmed by both flow cytometry and karyotypic analysis, in spite of a normal kappa/lambda Ig light chain ratio. Am J Hematol 82:473–480CrossRefGoogle Scholar
- 13.O’Reilly RA (1998) Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 1963 to 1995: 449 patients. West J Med 169:88–97Google Scholar
- 16.Dungarwalla M, Appiah-Cubi S, Kulkarni S, Saso R, Wotherspoon A, Osuji N, Swansbury J, Cunningham DC, Catovsky D, Dearden CE, Matutes E (2008) High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. Br J Haematol 143:71–74CrossRefGoogle Scholar