title: Combined systemic and ocular chemotherapy for anterior segment metastasis of systemic mantle cell lymphoma.
abstract: BACKGROUND
  Mantle 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.
  
  
  RESULTS
  A 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.
  
  
  CONCLUSIONS
  Combination 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.
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. aniruddha.agarwal@unmc.edu.
authors.forename: Aniruddha
authors.initials: A
authors.lastname: Agarwal
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. ali.sadiq@unmc.edu.
authors.forename: Mohammad Ali
authors.initials: MA
authors.lastname: Sadiq
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. william.rhoades@unmc.edu.
authors.forename: William R
authors.initials: WR
authors.lastname: Rhoades
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. loren.jack@unmc.edu.
authors.forename: Loren S
authors.initials: LS
authors.lastname: Jack
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. mostafa.hanout@gmail.com.
authors.forename: Mostafa
authors.initials: M
authors.lastname: Hanout
authors.affiliation: Division of Hematology and Oncology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, USA. pjbierma@unmc.edu.
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Bierman
authors.affiliation: Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA. wwest@unmc.edu.
authors.forename: William W
authors.initials: WW
authors.lastname: West
authors.affiliation: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, 985540 Nebraska Medical Center, Omaha, 68198-5540, NE, USA. quan.nguyen@unmc.edu.
authors.forename: Quan Dong
authors.initials: QD
authors.lastname: Nguyen
keywords: Ibrutinib; Iris; Mantle cell lymphoma; Metastasis; Methotrexate; Rituximab; Ultrasound biomicroscopy; Uveitis