Gracia-Ramos AE, Cruz-Domínguez MP, Vera-Lastra OL

Language: Spanish
References: 20
Page: 464-467
PDF size: 209.70 Kb.

ABSTRACT

Background: Castleman’s disease is an atypical lymphoproliferative disorder which may be compatible with paraneoplastic manifestations of POEMS syndrome.
Clinical case: a 53 year old man with a history of type 2 diabetes, hypothyroidism and Addison’s disease was attended. He started with numbness and weakness in limbs, dyspnea, skin hardening, Raynaud’s phenomenon, weight loss and fatigue appeared. Physical exam showed tachypnea, generalized cutaneous hyperpigmentation and skin hardening of extremities, muscle weakness, hypoesthesia and hyporefl exia. Laboratory showed hyperprolactinemia, low testosterone, hypothyroidism and Addison’s disease. Electrophoresis of proteins showed polyclonal hypergammaglobulinemia. Somatosensory evoked potentials reported peripheral neuropathy and severe axonal polyneuropathy by electromyography. Chest X-rays showed bilateral reticular infi ltrates and mediastinal widening. Echocardiogram displayed moderate pulmonary hypertension. Skin biopsy had no evidence of scleroderma. CT reported axillar, mediastinal and retroperitoneal nodes. The mediastinal lesion biopsy reported hyaline vascular Castleman’s disease, multicentric variety. He was treated with rituximab.
Conclusions: the case meet criteria for multicentric hyaline vascular Castleman’s disease, POEMS variant, treated with rituximab.

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