Abstract
Type I cryoglobulinemia is associated with B cell proliferative diseases, whereas essential mixed cryoglobulinemia is classically associated with infections, malignancy, and autoimmune diseases, but may be idiopathic. Prognosis in patients with grave manifestations and renal involvement is often poor. We report a case of a 40-year-old woman, 2 weeks post-partum for pre-eclampsia who was hospitalized with nephritic syndrome and acute renal failure. The patient harbored type I and type II cryoglobulinemia. Renal and cutaneous biopsies confirmed the diagnosis; however, an underlying etiology was not established. A bone marrow biopsy suggested monoclonal gammopathy of undetermined source (MGUS). Despite therapy with intravenous cyclophosphamide, rituximab, plasmapheresis, dialysis, and bortezomib, the patient succumbed after 8 months of hospitalization. We suggest that an overlap entity of types I and II cryoglobulinemia with severe multi-organ involvement not only is rare but also may be resistant to conventional therapy and fatal.
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References
Damoiseaux J (2014) The diagnosis and classification of the cryoglobulinemic syndrome. Autoimmun Rev 13:359–362
Trejo O, Ramos-Casals M, García-Carrasco M et al (2001) Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore) 80:252–262
Visentini M, Tinelli C, Colantuono S, Monti M, Ludovisi S, Gragnani L, Mitrevski M, Ranieri J, Fognani E, Piluso A, Granata M, de Silvestri A, Scotti V, Mondelli MU, Zignego AL, Fiorilli M, Casato M (2015) Efficacy of low-dose rituximab for the treatmen of mixed cryoglobulinemic vasculitis: phase II clinical trial and systematic review. Autoimmun Rev 14:889–896
Cacoub P, Comarmond C, Domont F, Savey L (2015) Cryoglobulinemia vasculitis. Am J Med 128:950–955
Houssiau FA, Vasconcelos C, D’Cruz D, Sebastiani GD, Garrido ER, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R (2002) Immunosuppressive therapy in lupus nephritis: the Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide. Arthritis Rheum 46:2121–2131
Muchtar E, Magen H, Gertz MA (2017) How I treat cryoglobulinemia. Blood 129:289–298
Terrier B, Krastinova E, Marie I, Launay D, Lacraz A, Belenotti P, de Saint-Martin L, Quemeneur T, Huart A, Bonnet F, le Guenno G, Kahn JE, Hinschberger O, Rullier P, Diot E, Lazaro E, Bridoux F, Zenone T, Carrat F, Hermine O, Leger JM, Mariette X, Senet P, Plaisier E, Cacoub P (2012) Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. Blood 119:5996–6004
Terrier B, Launay D, Kaplanski G, Hot A, Larroche C, Cathébras P, Combe B, de Jaureguiberry JP, Meyer O, Schaeverbeke T, Somogyi A, Tricot L, Zénone T, Ravaud P, Gottenberg JE, Mariette X, Cacoub P (2010) Safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis: data from the French Autoimmunity and Rituximab registry. Arthritis Care Res 62:1787–1795
Wink F, Houtman PM, Jansen TL (2001) Rituximab in cryoglobulinaemic vasculitis, evidence for its effectivity: a case report and review of literature. Clin Rheumatol 30:293–300
Colantuono S, Mitrevski M, Yang B, Tola J, Carlesimo M, de Sanctis GM, Fiorilli M, Casato M, Visentini M (2017) Efficacy and safety of long-term treatment with low-dose rituximab for relapsing mixed cryoglobulinemia vasculitis. Clin Rheumatol 36:617–623
Perez-Alamino R, Espinoza LR (2014) Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach. Curr Rheumatol Rep 16:420
Terrier B, Carrat F, Krastinova E, Marie I, Launay D, Lacraz A, Belenotti P, de Saint Martin L, Quemeneur T, Huart A, Bonnet F, le Guenno G, Kahn JE, Hinschberger O, Rullier P, Hummel A, Diot E, Pagnoux C, Lzaro E, Bridoux F, Zenone T, Hermine O, Leger JM, Mariette X, Senet P, Plaisier E, Cacoub P (2013) Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey. Ann Rheum Dis 72:374–380
Mazzaro C, Maso LD, Mauro E et al (2018) Survival and prognostic factors in mixed cryoglobulinemia: data from 246 cases. Diseases 3:6
Cordonnier D, Martin H, Groslambert P et al (1975) Mixed IgG-IgM cryoglobulinemia with glomerulonephritis. Immunochemical, fluorescent and ultrastructural study of kidney and in vitro cryoprecipitate. Am J Med 59:867–872
Frankel AH, Singer DR, Winearls CG, Evans DJ, Rees AJ, Pusey CD (1992) Type II essential mixed cryoglobulinaemia: presentation, treatment and outcome in 13 patients. Q J Med 82:101–124
Terrier B, Marie I, Launay D, Lacraz A, Belenotti P, de Saint-Martin L, Quemeneur T, Huart A, Bonnet F, le Guenno G, Kahn JE, Hinschberger O, Rullier P, Diot E, Lazaro E, Bridoux F, Zénone T, Carrat F, Hermine O, Léger JM, Mariette X, Senet P, Plaisier E, Cacoub P (2014) Predictors of early relapse in patients with non-infectious mixed cryoglobulinemia vasculitis: results from the French nationwide CryoVas survey. Autoimmun Rev 13:630–634
Ghetie D, Mehraban N, Sibley CH (2015) Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances. Rheum Dis Clin N Am 41:93–108
Rockx MA, Clark WF (2010) Plasma exchange for treating cryoglobulinemia: a descriptive analysis. Transfus Apher Sci 42:247–251
Madore F, Lazarus JM, Brady HR (1996) Therapeutic plasma exchange in renal diseases. J Am Soc Nephrol 7:367–386
Guillevin L, Pagnoux C (2003) Indications of plasma exchanges for systemic vasculitides. Ther Apher Dial 7:155–160
De Vita S, Quartuccio L, Isola M et al (2012) A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum 64:843–853
Ramirez GA, Campochiaro C, Salmaggi C, Pagliula G, D’Aliberti T, Marcatti M, Tresoldi M, Praderio L (2015) Bortezomib in type I cryoglobulinemic vasculitis: are we acting too late? Intern Med 54:1119–1123
Spizzo G, Mitterer M, Gunsilius E (2010) Bortezomib for the treatment of refractory type I cryoglobulinaemia. Br J Haematol 150:235–237
Talamo G, Claxton D, Tricot G, Fink L, Zangari M (2008) Response to bortezomib in refractory type I cryoglobulinemia. Am J Hematol 83:883–884
Besada E, Vik A, Koldingsnes W, Nossent JC (2013) Successful treatment with bortezomib in type I cryoglobulinemic vasculitis patien after rituximab failure: a case report and literature review. Int J Hematol 97:800–803
Bazari H, Mahindra AK, Farkash EA (2014) Case records of the Massachusetts General Hospital. A 61-year-old woman with gastrointestinal symptoms, anemia, and acute kidney injury. N Engl J Med 370:362–373
Hsu JH, Fang YW, Yang AH, Tsai MH (2018) Mixed cryoglobulinemic membranoproliferative glomerulonephritis due to monoclonal gammopathy of undetermined significance: a case report. Medicine (Baltimore) 97(37):e12416
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Tocut, M., Rozman, Z., Biro, A. et al. The complexity of an overlap type resistant cryoglobulinemia: a case report and review of the literature. Clin Rheumatol 38, 1257–1262 (2019). https://doi.org/10.1007/s10067-018-04423-y
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DOI: https://doi.org/10.1007/s10067-018-04423-y