S2512 A Devastating Case of Hepatitis C-Induced Mixed Cryoglobulinemia

Abstract

INTRODUCTION: Hepatitis C induced Mixed Cryoglobulinemia leading to rapidly progressive gangrene of all four extremities, necessitating amputations, is a rare presentation of cryoglobulinemia. It is postulated that precipitation of cold sensitive globulins in the presence of decreased vascular reserve are responsible for the occurrence of gangrene in cryoglobulinemia. CASE DESCRIPTION/METHODS: A 55-year-old male with treatment naive Hepatitis C (genotype 1a) presented to the emergency room with bilateral hand pain and diffuse purpuric rash. Physical examination showed palpable purpura and extensive gangrene affecting bilateral upper and lower extremities. Laboratory investigation revealed positive rheumatoid factor (>1,200 IU/mL), hypocomplementemia (C4 < 8.0 mg/dL), HCV RNA 2,390,000 IU/mL, and positive cryoglobulins. Cutaneous biopsy showed infiltrate with scattered small to mid-sized vessels with features of fibrin thrombi and associated vasculitis. He was diagnosed with mixed cryoglobulinemia complicated by severe cutaneous vasculitis with ischemic limbs. Plasmapheresis was initiated, however, gangrene continued to progressively worsen. He was transitioned to immunosuppressive therapy with high-dose pulse steroids followed by prednisone 60 mg daily for four weeks in combination with Rituximab. Sofosbuvir/velpatasvir was started for treatment of chronic Hepatitis C. HCV viral load was undetectable (<15 IU/mL) after one week and there was no further spread of gangrene. However, the necrotic limbs were unsalvageable, requiring serial limb amputations. DISCUSSION: There is a wide spectrum of manifestations associated with cryoglobulinemia with severe and life-threatening presentations being relatively rare (< 15% of cases). This case exemplifies a rare and devastating presentation of Hepatitis C induced mixed cryoglobulinemia. Upon literature review, this is one of the most aggressive cases of mixed cryoglobulinemia vasculitis reported thus far. There have been no other cases reported that have resulted in multiple limb amputations secondary to this extent of cryoglobulinemia. As such, this case serves to increase clinician awareness of the severe gangrene that may rapidly develop in mixed cryoglobulinemia. These cases require early recognition and aggressive treatment to prevent progression to the extent of limb amputation and even death. This highlights the necessity of universal screening for Hepatitis C and portrays the devastating effects that delayed screening and therefore delayed treatment may have.Figure 1.: Patient's left hand on initial presentation.Figure 2.: Patient's left hand weeks later, demonstrating progression of gangrene.Figure 3.: Bilateral gangrenous lower extremities.