[Cryoglobulinemia].

Rev Prat

Laboratoire d'hématologie, hôpital Necker-Enfants malades, Paris.

Published: January 2006

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Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.

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This study describes a 64-year-old female with a history of hepatitis C and cryoglobulinemia, who presented with respiratory symptoms, including dry cough, shortness of breath, and fever, alongside joint pain and fatigue. Initial workup revealed interstitial pneumonia, supported by chest imaging, and the patient was treated for pneumonia with standard antibiotic therapy. Despite no renal involvement, a hallmark of cryoglobulinemia, further testing confirmed elevated serum cryoglobulin levels.

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We report a case of coexisting cold agglutinin and cryoglobulin in a patient with severe anemia following COVID-19 infection, in whom direct antiglobulin testing revealed C3d positivity and immunoglobulin G negativity. There was no evident hemolytic anemia, thrombosis, or clinically significant IgM monoclonal gammopathy. The anemia improved with folic acid supplementation alone accompanied by a decrease of the cold agglutination titer, and the direct antiglobulin test became negative.

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Article Synopsis
  • Type I and mixed cryoglobulinemic vasculitis have different causes, symptoms, and treatment responses, with a reported case of refractory vasculitis linked to ischemic non-obstructive coronary artery disease.
  • The patient exhibited severe symptoms including dyspnea, abdominal pain, purpura, and renal failure requiring dialysis, with diagnostic markers suggesting mixed cryoglobulinemia.
  • Despite various treatments failing initially, bortezomib and dexamethasone successfully led to clinical improvement and cryoglobulin negativity, indicating bortezomib's potential as an effective therapy for this challenging condition.
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Introduction: Hepatitis C virus (HCV) infection represents a significant global health burden, particularly due to its extrahepatic immune-mediated manifestations, such as mixed cryoglobulinemia, associated vasculitis (CryoVas), and non-Hodgkin B-cell lymphoma (B-NHL), which pose significant challenges. The advent of direct-acting antiviral (DAA) has changed the therapeutic landscape for HCV-related complications.

Areas Covered: This review explores the evolving epidemiology and management of HCV extrahepatic manifestation and lymphoproliferative disorders in the era of DAAs.

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