Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Associated With Castleman Disease: A Common Misdiagnosis

Abstract

Castleman disease is a rare lymphoproliferative disorder. Co-presentation with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS syndrome) has been documented in 11-30% of Castleman disease cases. POEMS syndrome is a rare paraneoplastic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Not all features are required to make the diagnosis. We report a case of a woman who presented with a 1-year history of a left-side supraclavicular swelling associated with constitutional symptoms and symmetrical paresthesia of the lower limbs. In addition, she had skin hyperpigmentation, multiple supra and infra-diaphragmatic lymphadenopathies, hepatosplenomegaly and osteosclerotic lesions. Serum immunofixation was positive for immunoglobulin G-kappa gammopathy. A lymph node excisional biopsy was compatible with Castleman disease. The diagnosis of POEMS syndrome associated with Castleman disease was made. Our patient started treatment with a combination of bortezomib, cyclophosphamide, and dexamethasone with clinical and analytical improvement. Current treatment of POEMS syndrome associated with Castleman disease is focused on the management of POEMS syndrome. Early diagnosis requires a high index of suspicion and is crucial to reduce morbidity and mortality. This case report aims to raise awareness about this rare entity.

Keywords: Castleman disease; Monoclonal gammopathy; POEMS syndrome; Polyneuropathy.

Figure 1

Histological findings in the surgical specimen. (a) A germinal center with lymphocyte depletion; small dark lymphocytes concentrically arranged in the mantle zone (hematoxylin and eosin (H&E), × 400). (b) Interfollicular regions expanded by sheets of plasma cells and vascular proliferation (H&E, × 100). (c) Immunohistochemical staining for human herpes virus-8 (HHV-8, × 400).

Figure 2

Histological findings in the bone marrow biopsy specimen. (a) Elements from the three hematopoietic lineages with normal density, location and maturation (hematoxylin and eosin (H&E), × 100). (b, c) Positive immunohistochemical staining for plasma cells accounting for about 5% of the total cell population ((b) CD138, × 400); (c) MUM1, × 400). (d) Negative immunohistochemical staining for human herpes virus-8 (HHV-8, × 400).

Figure 3

Bone windows of computed tomography scan showing: (a) diffuse sclerotic lesions and (b) sclerotic lesion in right ischium.