Multicentric hyaline vascular Castleman´s disease. A POEMS type variant
Main Article Content
Keywords
Giant lymph node hyperplasia, POEMS syndrome
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 hyporeflexia. 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 infiltrates 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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