Abstract

ALFONSO VALDES, María Elena et al. Immunological disorders in Castleman's disease. Rev Cubana Hematol Inmunol Hemoter [online]. 2014, vol.30, n.2, pp. 184-189. ISSN 0864-0289.

Castleman'sdisease is a rare disorder characterized by angiofollicularlymphoid hyperplasia. There are two main histological patterns of the disease: the hyaline vascular type and the plasma cell type; andtwo clinical forms: localized and multicentric. We report a case of a 19-year-old female patient with hyaline vascular typeCastleman's disease withcervical lymphadenopathies and medical history of repetitive infectious diseases before diagnosis. The inmunohistochemical study reported CD 20, normal pattern of CD3 and Ki 67 positive only in lymphoidfollicles. The immunological study showed low levels of CD3+ CD4+ lymphocytes, slightly low levels of CD3+ CD8+ lymphocytes, and the presence of antinuclear antibodies (ANA). The humoral immunity was not affected.Other findings were IgG antibodies against CMV and EBV positive, and VIH 1,2 antibodies negative. Clinical history suggesting Immunodeficiency and laboratory findingssupport the hypothesis that deregulation of the immune system could lead to the pathogenesis of the proliferative disease. Also, the immunodeficiency state promotes the onset of opportunistic infectionswhich may increase immune and proliferative disorders. A favorable response to immunostimulant treatment was observed.

Keywords : Castleman's disease; follicular lymphoid hyperplasia; immunological disorders.

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