Clinical spectrum of Castleman disease-associated neuropathy.
OBJECTIVE
To define the peripheral neuropathy phenotypes associated with Castleman disease.


METHODS
We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls. Clinical, electrodiagnostic, 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 characterized by predominant sensory symptoms with no pain and mild distal sensory deficits (median Neuropathy Impairment Score of 7 points). Although both patients with CD-POEMS and patients with POEMS had a severe sensory and motor neuropathy, patients with CD-POEMS were less affected (median Neuropathy Impairment Score of 33 and 66 points, respectively). The degree of severity was also reflected on electrodiagnostic testing in which patients with CD-PN demonstrated a mild degree of axonal loss, followed by patients with CD-POEMS and then those with POEMS. Demyelinating features, defined by European Federation of Neurologic Societies/Peripheral Nerve Society criteria, were present in 43% of the CD-PN, 78% of the CD-POEMS, and 86% of the POEMS group.


CONCLUSIONS
There is a spectrum of demyelinating peripheral neuropathies associated with Castleman disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Compared to the POEMS cohort, those with CD-POEMS neuropathy have a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study.
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN.
Elie
E
Naddaf
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN.
Angela
A
Dispenzieri
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN.
Jay
J
Mandrekar
From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN. mauermann.michelle@mayo.edu.
Michelle L
ML
Mauermann
D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D005871:Castleman Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D015331:Cohort Studies; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D009460:Neurologic Examination; D016878:POEMS Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology*; D010641:Phenotype; D012720:Severity of Illness Index
D015415:Biomarkers