[Transitional type of Castleman's disease manifested as the POEMS syndrome].

BACKGROUND

Castleman's disease is an atypical lymphoproliferative disorder characterized by angiofollicular hyperplasia of lymph nodes. Histologically, it can be classified into a hyaline-vascular type, plasma-cell type, and transitional (mixed-cell) type, while clinically localized type has been classified as unicentric, or generalized (multicentric) form of the disease.

CASE REPORT: This paper presents a 21 year old male patient with multicentric Castleman's disease, a transitional (mixed-cell) type. The disease was manifested by fever, generalized enlargement of peripheral lymph nodes, breast glands enlargement, hyperemia of the face, and weakness of the lower extremities. We found mediastinal lymphadenopathy, pleural and pericardial effusions, sensorimotor peripheral neuropathy and polyclonal hypergammaglobulinemia. The simultaneous presence of these manifestations of the disease (sensomotor peripheral neuropathy, lymphadenopathy, effusions, endocrinopathy, polyclonal gammaglobulinemia and skin changes) is indentified as POEMS syndrome. The diagnosis of Castleman's disease was based on the results of histopathologic analysis of mediastinal lymph node biopsies after thoracotomy. The patient was treated with corticosteroids (prednisone 80 mg daily for 2 weeks followed by 60 mg daily). A partial response was achieved after 4 months of treatment.

CONCLUSION

A transitional type of multicentric Castleman's disease may be present itself as POEMS syndrome. The effect of corticosteroid therapy in this form of the disease is unpredictable.