Presentation Time: 0900-0915
Maryam Bintumar1, Sepideh Mokhtari2 and Robert J.B. Macaulay1
1 Department of Pathology, Moffitt Cancer Center, Tampa FL, USA
2 Department of Neuro-Oncology, Moffitt Cancer Center, Tampa, FL USA
Case 1: Progressive polyneuropathy in a 54 year old man.
This 54 YO man presented to an outside facility with persistent fevers, weight loss, and pancytopenia. Workup disclosed hemophagocytosis eventually necessitating splenic artery embolization and then splenectomy. NK cell expansion was documented in peripheral blood and bone marrow. He also had chronic severe sensorimotor axonal polyneuropathy which evolved concurrently with HLH. He was hospitalized 6 months later for worsening peripheral neuropathy; no HLH activity was detected at this time. Originally the lower extremities were affected but neuropathy progressed to all four extremities along with dysphagia, muffled voice and bilateral lower eyelid droop. He had bilateral foot drop and was able to walk only with braces. He endorsed numbness of his whole face, forehead and hard palate mucosa. Demyelinating and paraneoplastic work up was negative. MRI of brain and spine showed multi-level degenerative spine and disk disease. EMG showed polyneuropathy but no demyelination. He received IVIG 4 mg/kg over 4 days q 3 weeks with no improvement. Three months later he presented to the ER with worsening of neuropathy, decreased ambulation and generalized weakness. He underwent right gastrocnemius muscle and right sural nerve biopsies. He was discharged to hospice, but sought care at the Moffitt Cancer Center.
What is the diagnosis?
What testing should be performed on the biopsy sample?
What are the treatment options?
What is the likely etiology?
One H&E stained slide of sural nerve.
To be added after the meeting.