KRIOGLOBULINEMIČNI VASKULITIS KAO MANIFESTACIJA PARANEOPLASTIČKOG SINDROMA  PRIKAZ BOLESNIKA

Jasminka Milas-Ahić, Višnja Prus, Nela Šustić, Roberta Višević, Ivana Kovačević, Željka Kardum

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 -yearold
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 diff erentiated prostate adenocarcinoma was diagnosed and antiandrogen treatment was initiated. One year aft er 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.

Krioglobulinemija predstavlja prisutnost cirkulirajućih krioglobulina u serumu koji mogu dovesti do organskih oštećenja te sistemskog odgovora karakteriziranog umorom, artralgijama, purpurom, neuropatijom i glomerulonefritisom. Bolest uglavnom zahvaća male i srednje krvne žile i uzrokuje vaskulitis zbog stvaranja imunokompleksa koja sadrže krioglobuline. Miješane krioglobulinemije (tip I. i II.) najčešće su povezane s infekcijama, naročito hepatitisom C (HCV), ali se mogu javljati u sklopu limfoproliferativnih bolesti te autoimunih bolesti. Prikazali smo 75-godišnjeg bolesnika s akrocijanozom i nekrozom distalnih falangi 2. i 4. prsta lijeve šake te purpuroznim promjenama donjih ekstremiteta. Od 2001. bolesnik je liječen po hematologu zbog non-Hodgkinova limfoma želuca (MALT), a od 2002. godine kontrolira se zbog primarnog Sjogrenova sindroma. Učinjenom opsežnom obradom nađena je krioglobulinemija tip II., ali bez dokaza HCV-infekcije ili relapsa limfoproliferativne bolesti. Naknadnom obradom dokazan je slabo diferencirani adenokarcinom prostate i započeto liječenje antiandrogenom. Oko godinu dana nakon pojave simptoma akrocijanoze i digitalne nekroze kontrolnom endoskopskom obradom dokazan je i adenokarcinom želuca, bez tumorskih presadnica, te je provedeno kirurško liječenje. Međutim, postoperativno se razvija kliničko pogoršanje uz pojavu novih digitalnih nekroza na rukama te ubrzo dolazi do smrtnog ishoda pod slikom višesustavnog organskog zatajenja. Povezanost krioglobulinemije i solidnih tumora rijetko je opisana u literaturi, stoga je od izuzetnog značenja detaljna dijagnostička obrada radi što ranijeg otkrivanja osnovne bolesti.