Combined systemic and ocular chemotherapy for anterior segment metastasis of systemic mantle cell lymphoma

Abstract

BackgroundMantle cell lymphoma (MCL) is an aggressive subtype of non-Hodgkin’s lymphoma that rarely metastasizes to the iris and the anterior segment. Blastic/pleomorphic morphology is thought to have an adverse effect on prognosis in MCL. MCL is resistant to conventional chemotherapeutic regimens with a tendency for multiple relapses. Management of anterior segment metastasis of systemic MCL has not been described in literature.FindingsA 58-year-old male presented with an aggressive, relapsing, metastatic, systemic blastic variant of MCL with ocular involvement. At the time of initial presentation, large tumor cells were visible in the anterior chamber (AC) along with hypopyon and fibrin. The AC cells stained positively for CD20. The iris was thickened and coated with lymphoma cells. Iris neovascularization was present. Given extensive systemic and ocular involvement, the patient was given combination chemotherapy with systemic ibrutinib and intravitreal injections of methotrexate and rituximab. The disease response was monitored using multimodal imaging, including anterior segment optical coherence tomography and ultrasound biomicroscopy. Following combination of systemic and intraocular chemotherapy, there was a marked decrease in the ocular tumor load and the systemic disease.ConclusionsCombination therapy with intravitreal injections of chemotherapeutic agents targeting monoclonal B-cell population and novel systemic agents may help to achieve remission in anterior segment metastasis of aggressive subtypes of NHL such as blastic variant of MCL. Multimodal imaging may assist in the management of these cases.

The diagnosis and treatment of primary vitreoretinal lymphoma: a review

“…We showed that 1 mg of intravitreal rituximab in 0.1 cc (the 10 mg/cc concentration) not only was safe but also could treat vitreoretinal lymphoma. This was then confirmed by others [ 43 – 45 ]. There are no set protocols that have been verified in larger cohorts of patients.…”

Bilateral mantle cell lymphoma of the ciliary body that responded to a combined local radiotherapy and chemotherapy regimen: a case report

“…Ocular adnexal involvement of all NHL is clinically detectable in about 1.3–5% of cases, of which 5% have been found to be secondary to MCL. Intraocular involvement of MCL, notably, is extremely rare and has only been reported in 7 cases in the literature [5].…”

“…Reid et al reported unilateral granulomatous anterior chamber inflammation and localized undulations of the iris with aberrant vasculature in an elderly male of systemic MCL [9]. Agarwal et al reported another MCL case presented with unilateral anterior chamber hypopyon, thickening of the iris and iris neovascularization [5]. Although malignancy was believed to mainly involve the iris in these cases [5, 7–9], ciliary body was the major site of MCL recurrence in our case as revealed by UBM, despite that a concomitant anterior chamber reaction was also observed.…”

“…Evidences for diagnosis of intraocular MCL were variable in the reported cases. In most of the cases [6–8], intraocular malignancy was established solely on ocular manifestation and past history of systemic MCL, while two cases had more solid flow cytometric evidences [5, 9] and only one patient had cytopathologic confirmation [7]. In our case, intraocular involvement of MCL was confirmed by both liquid-based cytology and gene rearrangement of aqueous humor cells, which to the best of our knowledge has not been reported previously in the clinical setting of intraocular MCL.…”

“…As an aggressive form of NHL, MCL is resistant to conventional chemotherapeutic regimens with a tendency of multiple relapses, which results in a poor median survival of 3–6 years after diagnosis [2–4]. However, systemic metastasis of MCL to eyes is rare with most cases confined to the orbit and adnexa, intraocular involvement is even rarer, and it usually affects the choroid and iris according to the seven cases reported [5–11]. To the best of our knowledge, ciliary body metastasis of systemic MCL has not been reported.…”