Marginal Zone B Cell Lymphoma and Non-HCV Associated Type II Cryoglobulinemic Vasculitis: A Rare Presentation
Bildiriler > Tam Metin Bildiri · 2023
Yazarlar: Ayris ÖZTÜRK, Batuhan TURAL, Irmak ÇAPRAK, Banu YAMAN, Nilay DUMAN, Can CEYLAN
32nd Congress of the European Academy of Dermatology and Venereology, Almanya, Berlin, Uluslararası, Poster, Tam Metin Bildiri, 2023-2023, cilt -, ss ---
Özet
<p><strong>Introduction & Objectives:</strong></p><p>Cryoglobulins are immunoglobulins (Ig) or antibodies composed of Ig and complement components that precipitate below 37°C and dissolve upon rewarming. Cryoglobulinemia (CG) usually leads to a systemic inflammatory syndrome characterized by malaise, arthralgia, vasculitis, ulcers, neuropathy, and/or glomerulonephritis. In this case report, we present a 72-year-old HCV-seronegative 72-year-old woman with Marginal Zone B Cell Lymphoma and Type II Cryoglobulinemic Vasculitis (CV).</p><p><strong>Materials & Methods:</strong></p><p>The patient presented to our clinic with complaints of abdominal pain, fatigue, weight loss for 2 years, and painful sores that started on the legs 4 months ago and spread to both lower extremities. Comorbid hypertension, type 2 diabetes mellitus, polyneuropathy, and hypothyroidism were present. She was taking atorvastatin, methylprednisolone (MP) 15 mg, levothyroxine, perindopril arginine, indapamide, amlodipine, vildagliptin, metformin, insulin, colchicine, pregabalin, alpha lipoic acid. Physical examination revealed infected ulcers with necrotic crusts on both lower extremities.</p><p><strong>Results:</strong></p><p>Laboratory tests revealed elevated C reactive protein, sedimentation, cryoglobulin, rheumatoid factor, low complement 4, and normal complement 3. Normochrome normocytic anemia, lymphopenia, and thrombocytosis were present. Viral serology was compatible with previous hepatitis B (HBV). Hepatitis C virus (HCV) and human immunodeficiency virus antibodies were negative. Antinuclear antibody, anti-histone, anti-polymyositis- scleroderma antibody, and cold agglutinin were positive. IgM was high, IgA, and IgG was normal. Histopathology was consistent with CV (Figure 1).</p><p>The patient was diagnosed as type II CG due to a history of HBV, laboratory and HP findings, concomitant sensorial neuropathy, and leg ulcers and was started on MP 500 mg for 3 days and then 60 mg daily. Biopsy of suspicious lymph nodes found in malignancy screening was compatible with nodal marginal zone lymphoma (NMZL) with intense plasmacytic differentiation and bone marrow aspiration biopsy was consistent with B-cell lymphoma infiltration. No pathology was detected in other malignancy scans. A rituximab-bendamustine regimen for NMZL was planned. Entecavir treatment was started for prophylaxis of hepatitis B reactivation. Significant clinical response was obtained with treatment.</p><p><strong>Conclusion:</strong></p><p>Type I CG consists of monoclonal IgM or IgG; Type II consists of monoclonal IgM/polyclonal IgG immune complexes; Type III consists of polyclonal IgM and IgG. In Type II and III Mixed Cryoglobulinemia (MC), the IgM fraction has RF activity. Type I CG is usually associated with monoclonal gammopathies, whereas MC is associated with infections (HCV, HBV), autoimmune diseases, B-cell lymphomas, or solid neoplasms.</p><p>Typical laboratory findings of MC, which usually presents with the triad of purpura, malaise, and arthralgia, are elevated RF, hypocomplementemia, and hypergammaglobulinemia. CV is difficult to manage due to severe organ involvement and high mortality. Treatment of the underlying malignancy, glucocorticoids, cyclophosphamide, rituximab, and plasmapheresis are among the treatment options. This case report emphasizes this rare association between HCV-seronegative B-cell lymphoma and CV and aims to raise clinician awareness.</p>