Source: International Congress 2014 – Clinical management of interstitial lung diseases and vasculitis
Disease area: Interstitial lung diseases, Thoracic oncology

Abstract

POEMS syndrome is a rare multisystemic disorder characterized by polyneuropathy, organomegaly, monoclonal gammopathy and skin lesions.Here we present a case of Castleman disease variant of atypical POEMS syndrome presented with organomegaly,mediastinal conglomerate lymph nodes,bilateral pulmonary involvement,monoclonal gammopathy,elevated IL-6 levels, anemia and thrombosis but no polyneuropathy.A 40 year-old female was presented with a history of dyspnea, fever and 20 kilograms of weight loss in four months.Thorax computerized tomography revealed bilateral conglomerate hilar and mediastinal lymph nodes,bilateral diffuse parenchymal consolidations and multiple parenchymal spiculated nodules.Fiberoptic bronchoscopy showed no morphological pathology,bronchoscopic biopsy specimens had no specific diagnosis.Diagnostic mediastinoscopy session was terminated because of the increased fibrotic tissue.Hence, patient was undergone thoracotomy and multinodular,dense lymphoplasmocytic infiltration was seen in biopsy specimens.Immunohistochemistry studies showed no specific pathology.The bone scintigraphy and the knee magnetic resonance imaging revealed cortical erosion at distal femur, which showed kappa monoclonal plasma cell infiltration at biopsy.IL-6 level was elevated and electromyography showed no polyneuropathy.On the basis of the findings of conglomerate lymphadenopathy,monoclonal gammopathy, organomegaly,anemia, elevated IL-6 levels and trombocytosis, patient was diagnosed with Castleman disease variant of atypical POEMS syndrome.Atypical and multisystemic rare diseases should be kept in mind in the differential diagnosis of a patients with interstitial lung disease with unusual presentation.

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Z. Isikdogan, A. Baha, N. Kokturk, H. Turktas, N. Akyurek, G. Sucak (Ankara, Turkey). Castleman disease variant of atypical POEMS syndrome: A case presentatIon. Eur Respir J 2014; 44: Suppl. 58, 4511

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