Castleman’s Disease Neuropathies (P3.155)

OBJECTIVE: Define the peripheral neuropathy phenotypes associated with Castlemans disease (CD). BACKGROUND: Castlemans disease (CD) is a rare lymphoproliferative disorder characterized by lymphoid follicle proliferation with involution of germinal centers and endothelial hyperplasia. CD can be associated with POEMS syndrome. METHODS: With IRB approval, we conducted a retrospective chart review for patients with biopsy-proven CD evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy (PN) were identified and divided into two groups: CD with PN without POEMS (CD-PN) and CD with POEMS (CD-POEMS). A cohort of POEMS patients was used as controls. Clinical, electrophysiologic, and laboratory characteristics were collected and compared among patient subgroups. RESULTS: There were 7 patients with CD-PN, 20 with CD-POEMS and 122 with POEMS. Patients with CD-PN had the mildest neuropathy with mainly sensory symptoms, less pain and weakness, and mild distal sensory deficits (median Neuropathy Impairment Score (NIS) of 7 points). Although both CD-POEMS and POEMS patients had a severe, painful, sensorimotor neuropathy, CD-POEMS patients were less affected (median NIS of 33 and 66 points respectively). The degree of severity was also reflected on electrodiagnostic testing where CD-PN patients demonstrated a mild degree of axonal loss, followed by CD-POEMS and then POEMS. Demyelinating features were present in 43[percnt] of CD-PN group, 78[percnt] of CD-POEMS and 86[percnt] of POEMS group. CD-POEMS and POEMS groups had a similar number of patients with sclerotic lesions and the associated monoclonal protein was almost always of the lambda, non-IgM subtype. CONCLUSIONS: There is a spectrum of demyelinating peripheral neuropathies associated with Castlemans disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe, painful, sensorimotor neuropathy. Compared to POEMS cohort, the CD-POEMS has a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study. Disclosure: Dr. Naddaf has nothing to disclose. Dr. Dispenzieri has nothing to disclose. Dr. Mandrekar has nothing to disclose. Dr. Mauermann has nothing to disclose.