Pediatric primary cutaneous marginal zone B-cell lymphoma treated with intralesional rituximab

ARTICLE

Auteur(s) : Min Young Park¹, Hyun Joo Jung², Jun Eun Park², You Chan Kim¹

¹Department of Dermatology
²Department of Pediatrics, Ajou University School of Medicine, 5 Wonchon-Dong, Yeongtong-Gu, Suwon 443-721, South Korea

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is an indolent lymphoma which is extremely rare in children. Recently, intralesional therapy with anti-CD20 monoclonal antibodies (rituximab) has shown favorable results for the treatment of PCMZL. We present a pediatric case of PCMZL that was successfully treated with intralesional rituximab injection.

An 11-year-old boy presented with a 3-month history of an erythematous, non-tender nodule on the left cheek (figure 1A). There was no history of fever, weight loss or night sweats and no hepatosplenomegaly or lymphadenopathy. A skin biopsy specimen showed a dense infiltrate of small to medium sized lymphoid cells in the dermis but the epidermis was spared (figure 1C). The cells had pale cytoplasm, irregular and hyperchromatic nuclei and small nucleoli (figure 1D). The lymphocytes were positive for CD20 (figure 1E), CD79a, and bcl-2 (figure 1F), however negative for CD5 and bcl-6.

Serological tests for the human immunodeficiency virus, hepatitis B and C viruses, and Helicobacter pylori were negative; however, indirect immunofluorescence assay and Westen blot tests for Borrelia burgdorferi were positive, but showed negative results in the tests repeated after one month. There were no abnormalities in the bone marrow aspirate, biopsy, or on immunophenotyping. The PET-CT demonstrated no evidence of systemic involvement. Polymerase chain reaction analysis for immunoglobulin heavy chain genes showed B-cell monoclonality only in the skin biopsy tissue.

The treatment options considered included surgical excision and radiotherapy, however, the cosmetic outcome was a concern. Therefore, intralesional rituximab therapy was attempted after informed consent was obtained. The patient received local injections of 0.5 mL stock rituximab solution, twice weekly for the first two weeks and then once weekly for 18 consecutive injections. The lesion resolved at 6 months and the treatment was then discontinued. This treatment was safe and effective and there was only moderate pain related to the injection. Our patient has remained in complete remission for 26 months to date (figure 1B).

Until now, many treatment options have been suggested for PCMZL. For a single lesion, surgical excision or local irradiation has commonly been used [1]. For multiple lesions, interferon-alpha or systemic chemotherapy has been used, and rituximab has also been considered as an alternative strategy [2]. However, systemic administration can affect normal B-cells and immunoglobulins. Therefore, for a few lesions, intralesional administration has been attempted, showing favorable results [3]. Kyrtsonis et al. [4] reported two patients with PCMZL, who showed a complete response to intralesional rituximab therapy and no recurrence at 36 to 44 months.

Intralesional rituximab treatment has some advantages compared to other modalities; it is much less expensive, and has fewer adverse effects compared to intravenous administration. In addition, because intralesional injection seldom causes scarring, compared to surgical excision or radiotherapy, it provides a more cosmetically acceptable outcome, especially when areas such as the face are involved.

In the present case, the blood test confirmation of Borrelia burgdorferi took a long time; therefore, we could not wait for the results, and attempted treatment with rituximab injections first, rather than with antibiotics. In fact, the positive test results were unexpected, because Asia is not an endemic area of Borrelia burgdorferi [5]. In addition, the following laboratory test showed a negative result. Therefore, we could not be sure that the infection was associated with PCMZL in this patient. Moreover, eradication of Borrelia burgdorferi would not guarantee regression of the PCMZL [6].

The present case shows that intralesional rituximab injections were a safe and effective treatment for PCMZL, even in a pediatric patient. Further investigations with controlled multi-center studies are needed to evaluate the appropriate dose of the injected rituximab and the possible recurrence risks.

Acknowledgements

References

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3 Fink-Puches R, Wolf IH, Zalaudek I, Kerl H, Cerroni L. Treatment of primary cutaneous B-cell lymphoma with rituximab. J Am Acad Dermatol 2005; 52: 847-53.

4 Kyrtsonis MC, Siakantaris MP, Kalpadakis C, et al. Favorable outcome of primary cutaneous marginal zone lymphoma treated with intralesional rituximab. Eur J Haematol 2006; 77: 300-3.

5 Li C, Inagaki H, Kuo TT, Hu S, Okabe M, Eimoto T. Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathologic study of 24 asian cases. Am J Surg Pathol 2003; 27: 1061-9.

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