Case
A 26-year-old female was diagnosed about a year ago with stage IIA ALK+ ALCL with right axillary and cervical lymphadenopathy and was initiated on CHOEP (cyclophosphamide, doxorubicin, etoposide, vincristine and prednisone) chemotherapy. She achieved complete remission following three cycles of treatment. Soon thereafter, she developed sudden loss of vision in both eyes associated with fever, and night sweats. Vision loss started in the left eye then progressed to the right eye, with severity at a loss of light perception level. She was transferred to our institution. Significant clinical findings include loss of vision bilaterally with limited ability to appreciate light and dark stimulus. Pupils were dilated bilaterally both with a positive direct and indirect light reflex. She had left ocular dysmotility consistent with CN6 palsy. No focal weakness or sensory deficit bilaterally in the face and extremities. She had no lymphadenopathy, or hepatosplenomegaly. Serum lactate dehydrogenase enzyme level was elevated at 406 U/L (122-222U/L). MRI brain imaging showed bilateral nodular leptomeningeal enhancements in the posterior fossa and enhancement around both optic nerves (Fig.1 Before treatment) consistent with neurolymphomatosis. CSF cytology was positive for atypical large cells consistent with ALCL (Fig.2). Bone marrow biopsy did not show any involvement with lymphoma. She did not have any clinical evidence of neurolymphomatosis involving peripheral nerves. Review of her outside right axillary lymph node biopsy (Fig.3) confirmed the diagnosis of ALCL with the proliferation of large, atypical lymphocytes with horseshoe-shaped nuclei on hematoxylin and eosin stain and strong expression of ALK-1, CD5, and CD30, and negative expression of PAX 5, CD20, or CD3 by immunohistochemistry studies.
She was initially treated with dexamethasone 40 mg/day for four days followed by a taper and the Ferrari regimen with high-dose methotrexate 3500mg/m2 and high-dose cytarabine 2000mg/m2 with intrathecal methotrexate 12.5mg. This was followed with10 sessions of radiation to the bilateral orbits and optic nerves, a total cumulative dose of 3000cGy. There was no significant improvement in her vision after the initial treatment. She continued with the Ferrari regimen every 3 weeks for a total of 4 cycles. After 2 cycles there was no radiological evidence of residual lymphoma on follow up brain MRI imaging (Fig.1 Post treatment). Furthermore, repeat CSF cytology was negative for malignant cells. The patient subsequently underwent high-dose chemotherapy with BCNU and thiotepa followed by autologous stem cell transplant. One year after her transplant, she continues to be in complete remission for both systemic and CNS disease, and had small improvement in light and colour perception. In spite of her CNS disease being responsive to treatment and achieving complete remission her vision never improved significantly.