POEMS SYNDROME WITH CASTLEMAN DISEASE - AN UNUSUAL PRESENTATION

Abstract

Chronic Inflammatory Demyelinating Polyneuropathy(CIDP) is an acquired immune mediated disorder presenting as symmetric proximal and distal sensory motor polyneuropathy. It is treatable with immunosuppressant. CIDP may be associated with HIV-1 infection, SLE, para neoplastic disorders and Paraproteinemias. Paraproteinemias is abnormal monoclonal antibody or light chain in blood secondary to plasma cell disorder like multiple myeloma, Waldenstrom macroglobulinemia, monoclonal gammopathy of undetermined significance (MUGS), POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, monoclonal plasma cell proliferative disorder and Skin Changes) syndrome and Castleman disease with or without POEMS syndrome. These account for approximately 10% of CIDP. Diagnosing Paraproteinemic neuropathy is necessary to prevent delay in diagnosis, start appropriate treatment and estimate prognosis of the condition. POEMS syndrome is due to clonal proliferation of plasma cells. Castleman disease (CD), also known as angiofollicular or giant lymph node hyperplasia, is a rare non-neoplastic lymphoproliferative disorder . We present a Case report of relatively young male with right parotid swelling with chronic sensorimotor quadriparesis with wasting, with no predominant neuropathic pain and cranial nerve involvement. On Nerve Conduction Studies it was found to have severe sensori-motor neuropathy which was not associated with pain, an unlikely feature of either CIDP or Castleman disease; on further evaluation was found to have POEMS syndrome. Thus high index of suspicion is necessary to diagnose this condition. This case report emphasizes the fact that patients with Castleman disease-POEMS Syndrome may present at younger age than usual with short duration of illness, with severe sensorimotor neuropathy which may not be associated with pain. High index of suspicion and appropriate investigations may lead to early diagnosis and prompt treatment.