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Case Reports
. 2018 Mar;52(1):61-65.
doi: 10.4314/gmj.v52i1.9.

Sjögren's and plasma cell variant Castleman disease: a case report

Yvonne A Dei-Adomakoh  1 , Leslie Quarcoopome  2 , Afua D Abrahams  3 , Catherine I Segbefia  4 , Dzifa I Dey  5
Affiliations
Case Reports

Sjögren's and plasma cell variant Castleman disease: a case report

Yvonne A Dei-Adomakoh et al. Ghana Med J. 2018 Mar.

Abstract

Castleman disease is a rare cause of lymphoid hyperplasia and may result in localized symptoms or an aggressive, multisystem disorder. It can mimic other diseases like lymphoma or tuberculosis. It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites including the lungs, larynx, parotid glands, pancreas, meninges, and muscles. In HIV and HHV8-negative patients with idiopathic multi-centric Castleman disease, pathogenesis may involve autoimmune mechanisms. We highlight and report a case of a 34-year-old Ghanaian female who was successfully diagnosed and managed for Sjögren's as well as plasma cell variant Castleman disease with combination chemotherapy and rituximab followed by eighteen months maintenance therapy with pulse chlorambucil and prednisolone and three monthly rituximab.

Keywords: Plasma Cell Variant Castleman Disease; Rituximab; Sjögren's syndrome; chemotherapy; lymphadenopathy.

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Conflict of interest statement

Conflict of interest: None declared

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