Abstract Details

Title Combined Central and Peripheral Demyelination in the Context of Chronic Lymphocytic Leukemia

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-100

Objective We report the first described case of seronegative combined central and peripheral demyelination (CCPD) in the setting of untreated chronic lymphocytic leukemia (CLL), and successful treatment with dual-indication B-cell depleting therapy.

Background CCPD has been used to describe an uncommon syndrome where demyelinating lesions are seen concurrently or in rapid sequence in both the central and peripheral nervous systems. The pathophysiology underlying this disorder, and particularly cases arising in the context of cancer, has not been fully defined. CLL is associated with immune dysregulation and autoimmune phenomena.

Design/Methods

Not applicable

Results A 57-year-old man with untreated CLL developed progressive lower extremity weakness and was diagnosed with chronic inflammatory demyelinating neuropathy (CIDP). After an initial response to intravenous immunoglobulin (IVIG) and steroids, over several months his neuropathy rapidly worsened, and he was found to have an expansile ring-enhancing cerebellar mass. Brain biopsy and cerebrospinal fluid testing showed no definitive evidence of CLL involvement and were consistent with demyelination. Extensive antibody testing was negative. He underwent plasmapheresis followed by dual-indication obinutuzumab B-cell depleting therapy. The patient experienced marked clinical and radiographic improvement over the following months.

Conclusions Seronegative CCPD in the context of hematologic malignancy has not previously been reported. This case expands the spectrum of neurological complications in CLL beyond the established association with CIDP. B-cell depletion with enhanced antibody-dependent cellular cytotoxicity may be beneficial in patients with both oncologic and neurologic indications.

Authors/Disclosures
Adaadinchezo Oguejiofor, MD PRESENTER	Dr. Oguejiofor has nothing to disclose.
Ashley E. Aaroe, MD (MD Anderson Cancer Center)	Dr. Aaroe has received personal compensation in the range of $10,000-$49,999 for serving as a Delphi Panel Consultant with Advi.
Shekhar Khanpara	Shekhar Khanpara has nothing to disclose.

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