Indian Journal of Pathology and Microbiology
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Year : 2013  |  Volume : 56  |  Issue : 3  |  Page : 325-327
Primary cutaneous marginal zone lymphoma (PCMZL) presenting with heterochronous biclonal lesions

1 Department of Pathology, Medical School, Aristotle University of Thessaloniki, Greece
2 Laboratory of Pathology, General Hospital of Xanthi, Greece

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Date of Web Publication24-Oct-2013

How to cite this article:
Koletsa TD, Beretouli EA, Mavropoulou SD, Kostopoulos IS. Primary cutaneous marginal zone lymphoma (PCMZL) presenting with heterochronous biclonal lesions . Indian J Pathol Microbiol 2013;56:325-7

How to cite this URL:
Koletsa TD, Beretouli EA, Mavropoulou SD, Kostopoulos IS. Primary cutaneous marginal zone lymphoma (PCMZL) presenting with heterochronous biclonal lesions . Indian J Pathol Microbiol [serial online] 2013 [cited 2022 Jan 23];56:325-7. Available from: https://www.ijpmonline.org/text.asp?2013/56/3/325/120418


Primary cutaneous marginal zone lymphoma (PCMZL) is a B-cell lymphoma, which, by definition, belongs to MALT (Mucosa associated lymphoid tissue) lymphoma. [1] Chronic inflammatory or autoimmune processes can play a role in the development of MALT, where it is normally not found. The diagnosis of PCMZL is often established either by immunohistochemical light chain restriction or by molecular studies. A case of PCMZL that showed different light chain restrictions in two heterochronous lesions is presented.

A 43-year-old male patient presented in 2004 with a solitary cutaneous nodule in the anterior surface of the right tibia, which was totally excised, and a paraffin block with embedded tissue of the lesion was sent for consultation to our department. Hematoxylin and eosin (H-E)-stained sections showed diffuse dermal infiltration by small-to medium-sized neoplastic lymphoid cells with plasmacytoid features [Figure 1]a, expressing B-cell markers and λ light chains [Figure 1]b. The findings were consistent with PCMZL. Blood laboratory tests were within normal ranges and no other therapy was performed.

In 2011, a new cutaneous nodule, located in the right ankle, was observed [Figure 1]c. H-E-stained sections revealed a dense dermal and subcutaneous infiltration by small to medium-sized neoplastic lymphoid cells [Figure 1]d, many of which had plasmacytoid features. These cells presented the following immunophenotype: CD20+, CD45RA+, CD79α+, PAX5+, IgG+, κ+ [Figure 1]e, CD45RO-, CD3-, CD138-, CD10-, BCL6-, IgA-, IgM- and λ- [Figure 1]f. A significant number of IgG/κ-restricted plasma cells, positive to CD38, CD138 and MUM1 antibodies, were also apparent. The existence of IGH monoclonal population was demonstrated by polymerase chain reaction (PCR) analysis.
Figure 1: First biopsy: Cutaneous infi ltrati on by lymphoid cells (a) and λ light chain-restricted plasma cells (b). A biopsy of erythematous nodule (c) revealed similar histologic characteristi cs to those seen in the fi rst biopsy (d), but the plasma cells are κ restricted (e: κ, f: λ). [a: Hematoxylin
and eosin (H-E) ×100, b: immunohistochemistry (IHC) ×400, d: H-E ×100, e-f: IHC ×400]

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The histological findings set the diagnosis of PCMZL, with λ light chain restriction in the first lesion and κ in the second one. Tissue from the first biopsy was not available for PCR analysis. Blood laboratory studies and chest and abdominal computerized tomography scans revealed no evidence of systemic involvement by lymphoma. The patient denied bone marrow biopsy. He had undergone radiotherapy and is followed up every 6 months.

Although heavy chain isotype switching is well known in B cell ontogeny and lymphomas, [2] the finding of both κ and λ light chain-restricted B cell populations in PCMZL, happening synchronous or metachronous, is unusual and has only recently been reported. [3],[4],[5] Whether lesions arising in different places or times are clonally related or not is a question to be investigated. The two lesions of this particular case are considered clonally different. According to the molecular biology of B cell development, the IGK rearrangements occur first and, if they succeed to be productive, the IGL genes will remain in germline configuration. If IGK rearrangements fail to be productive, the IGL genes will rearrange. If the IGL genes fail as well, then the cell will die by apoptosis. Hence, a lymphoma B cell can express both κ and λ light chains and can switch from making κ to λ, but switching from λ to κ expression is not possible. Finally, cases similar to this presentation could be considered as different neoplastic diseases, having the same immunomorphology and probably a common causative immune stimulator.

   References Top

1.Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 1
2.Aarts WM, Willemze R, Bende RJ, Meijer CJ, Pals ST, van Noesel CJ. VH gene analysis of primary cutaneous B-cell lymphomas: Evidence for ongoing somatic hypermutation and isotype switching. Blood 1998;92:3857-64.  Back to cited text no. 2
3.Edinger JT, Kant JA, Swerdlow SH. Cutaneous marginal zone lymphomas have distinctive features and include 2 subsets. Am J Surg Pathol 2010;34:1830-41.  Back to cited text no. 3
4.Edinger JT, Lorenzo CR, Breneman DL, Swerdlow SH. Primary cutaneous marginal zone lymphoma with subclinical cutaneous involvement and biclonality. J Cutan Pathol 2011;38:724-30.   Back to cited text no. 4
5.Ferrara G, Cusano F, Robson A, Stefanato CM. Primary cutaneous marginal zone B-cell lymphoma with anetoderma: spontaneous involution plus de novo clonal expansion. J Cutan Pathol 2011; 38:342-5.  Back to cited text no. 5

Correspondence Address:
Triantafyllia D Koletsa
Department of Pathology, Medical School, Aristotle University of Thessaloniki, University Campus, 54124 Thessaloniki
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.120418

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