Abstract:

A 73-year-old male with recently diagnosed mantle cell lymphoma (MCL) was transferred to our facility for ongoing work-up of progressive weakness. He was originally diagnosed with bone marrow biopsy after presenting with night sweats, weight loss, and generalized weakness. His MCL was positive for CD19, CD20, and CD5. He received one round of chemotherapy, bendamustine and rituximab, however treatment was complicated by pancytopenia. He then developed progressive weakness of all extremities leading to near paralysis. Upon dermatologic exam, he had infiltrated red-violaceous plaques on the face, trunk, and upper extremities that began a few weeks after initiating chemotherapy. Work-up for his weakness was unrevealing at an outside hospital, and his symptoms did not respond to treatment with dexamethasone and plasma exchange. Further work-up at our facility including a lumbar spine MRI showed abnormal contrast enhancement in the dural sac which raised suspicion for neurolymphomatosis. Skin biopsy from the left arm showed an infiltrate of monomorphous, medium-large atypical cells throughout the entire dermis. Immunohistochemical stains were positive for CD20, CD5, and cyclin D1. These findings were consistent with cutaneous involvement of MCL. MR-CHOP therapy was initiated, however the patient developed aspiration pneumonia and unfortunately passed soon after transfer back to his local hospital. This patient’s rapidly progressive symptoms including neurolymphomatosis and cutaneous involvement indicate high grade disease. MCL accounts for up to 10% of cases of non-Hodgkin’s lymphoma and usually has a poor prognosis. Cutaneous MCL is oftentimes due to extension of systemic disease and has rarely been reported to primarily affect the skin. Cyclin D1 positivity is due to a t(11;14) translocation that is useful in making this diagnosis. This is the first reported case of MCL presenting with neurolymphomatosis and cutaneous involvement.

Financial Disclosure:
No current or relevant financial relationships exist.

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Margaret Ann Kreher