부산대학교 도서관

Castleman disease (CD) is a rare lymphoproliferative disorder. Two main variants have been described: unicentric or multicentric (UCD/MCD). MCD can be idiopathic or related to human herpesvirus 8 (HHV8) infection in the setting of immune deficiency. MCD is more severe than UCD and is usually accompanied by other systemic symptoms. Peripheral neuropathy can be associated with MCD in isolation or as part of a wider spectrum of symptoms, the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes). Compared with POEMS-neuropathy, CD-neuropathy is often milder, sensory, and not painful. There are few reports of CD-neuropathy with cranial nerve involvement, only affecting the optic and trigeminal nerves. We present a 50-year-old patient who developed bilateral facial weakness, numbness of hands and feet, sensory ataxia, and progressive walking difficulties over 3 weeks. Nerve conduction studies showed sensory-motor axonal symmetric neuropathy with signs of demyelination in cranial segments. He had history of human immunodeficiency virus infection, well controlled under antiretroviral treatment. Upon admission, he developed fever and pancytopenia, and diffuse lymphadenopathies were detected. An axillary lymph node biopsy was performed showing HHV8-positive small follicles, lymphodepletion, and penetrating vessels with interfollicular polyclonal plasmocytosis, supporting the diagnosis of CD, and adjacent focus of HHV8+ Kaposi sarcoma. He was treated with R-CHP (rituximab, cyclophosphamide, doxorubicin, and prednisone) chemoimmunotherapy regimen. The neuropathy progressively resolved, both clinically and electrophysiologically, over a period of 6 months in parallel with the remission of CD. CD-associated neuropathy may present as bifacial weakness and limb paraesthesia, mimicking the variant of Guillain-Barré syndrome. The neurological symptoms may have a subacute progression leading to important disability. The outcome is good with chemoimmunotherapy, with parallel recovery of the neuropathy and the CD.