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KRIOGLOBULINEMICNI VASKULITIS KAO MANIFESTACIJA PARANEOPLASTICKOG SINDROMA--PRIKAZ BOLESNIKA #MMPMID26891578
Milas-Ahic J; Prus V; Sustic N; Visevic R; Ivana Kovacevic; Kardum Z
Reumatizam 2015[]; 62 (1): 27-30 PMID26891578show ga
Cryoglobulinemia refers to the presence of circulating cryoglobulins in the serum which may lead to organ damage and systemic response characterized by fatigue, arthralgias, purpura, glomerulonephritis and neuropathy. The disease mainly affects small and medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Mixed cryoglobulinemia (type I and II) are most often associated with infections, especially hepatitis C virus (HCV), but may occur as part of lymphoproliferative and autoimmune diseases. We present a 75-year-old male patient with acrocyanosis and digital necrosis of the left hand and purpura of the lower extremities. Since 2001 the patient was treated for Non-Hodgkin's lymphoma of the stomach (MALT) and since 2002 for Sjogren's syndrome. Extensive diagnostic procedures revealed cryoglobulinemia type II, but without evidence of HCV infection or relapse of lymphoproliferative disorder. Furthermore, poorly differentiated prostate adenocarcinoma was diagnosed and antiandrogen treatment was initiated. One year after the onset of symptoms acrocyanosis and digital necrosis, gastric adenocarcinoma was diagnosed, without metastatic disease, and surgical treatment was performed. However, postoperatively recurrence of new digital necrosis of the hands occurred along with clinical deterioration and multiple organ failure leading to lethal outcome. Association of cryoglobulinemia and solid tumors is rarely described in the literature, so it is very important to perform comprehensive diagnostic evaluation and detect potential underlying disease as soon as possible.