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Published in: Clinical Rheumatology 5/2019

01-05-2019 | Vasculitis | Case Based Review

The complexity of an overlap type resistant cryoglobulinemia: a case report and review of the literature

Authors: Milena Tocut, Ziv Rozman, Alexander Biro, Asher Winder, Amir Tanay, Gisele Zandman-Goddard

Published in: Clinical Rheumatology | Issue 5/2019

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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.
Literature
1.
go back to reference Damoiseaux J (2014) The diagnosis and classification of the cryoglobulinemic syndrome. Autoimmun Rev 13:359–362CrossRefPubMed Damoiseaux J (2014) The diagnosis and classification of the cryoglobulinemic syndrome. Autoimmun Rev 13:359–362CrossRefPubMed
2.
go back to reference 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) 80:252–262CrossRef 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) 80:252–262CrossRef
3.
go back to reference 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 14:889–896CrossRefPubMed 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 14:889–896CrossRefPubMed
4.
go back to reference Cacoub P, Comarmond C, Domont F, Savey L (2015) Cryoglobulinemia vasculitis. Am J Med 128:950–955CrossRefPubMed Cacoub P, Comarmond C, Domont F, Savey L (2015) Cryoglobulinemia vasculitis. Am J Med 128:950–955CrossRefPubMed
5.
go back to reference 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 46:2121–2131CrossRefPubMed 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 46:2121–2131CrossRefPubMed
7.
go back to reference 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 119:5996–6004CrossRefPubMed 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 119:5996–6004CrossRefPubMed
8.
go back to reference 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 62:1787–1795CrossRef 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 62:1787–1795CrossRef
9.
go back to reference Wink F, Houtman PM, Jansen TL (2001) Rituximab in cryoglobulinaemic vasculitis, evidence for its effectivity: a case report and review of literature. Clin Rheumatol 30:293–300CrossRef Wink F, Houtman PM, Jansen TL (2001) Rituximab in cryoglobulinaemic vasculitis, evidence for its effectivity: a case report and review of literature. Clin Rheumatol 30:293–300CrossRef
10.
go back to reference 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 36:617–623CrossRefPubMed 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 36:617–623CrossRefPubMed
11.
go back to reference Perez-Alamino R, Espinoza LR (2014) Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach. Curr Rheumatol Rep 16:420CrossRefPubMed Perez-Alamino R, Espinoza LR (2014) Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach. Curr Rheumatol Rep 16:420CrossRefPubMed
12.
go back to reference 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 72:374–380CrossRefPubMed 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 72:374–380CrossRefPubMed
13.
go back to reference Mazzaro C, Maso LD, Mauro E et al (2018) Survival and prognostic factors in mixed cryoglobulinemia: data from 246 cases. Diseases 3:6 Mazzaro C, Maso LD, Mauro E et al (2018) Survival and prognostic factors in mixed cryoglobulinemia: data from 246 cases. Diseases 3:6
14.
go back to reference 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 59:867–872CrossRefPubMed 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 59:867–872CrossRefPubMed
15.
go back to reference 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 82:101–124PubMed 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 82:101–124PubMed
16.
go back to reference 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 13:630–634CrossRefPubMed 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 13:630–634CrossRefPubMed
17.
go back to reference Ghetie D, Mehraban N, Sibley CH (2015) Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances. Rheum Dis Clin N Am 41:93–108CrossRef Ghetie D, Mehraban N, Sibley CH (2015) Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances. Rheum Dis Clin N Am 41:93–108CrossRef
18.
go back to reference Rockx MA, Clark WF (2010) Plasma exchange for treating cryoglobulinemia: a descriptive analysis. Transfus Apher Sci 42:247–251CrossRefPubMed Rockx MA, Clark WF (2010) Plasma exchange for treating cryoglobulinemia: a descriptive analysis. Transfus Apher Sci 42:247–251CrossRefPubMed
19.
go back to reference Madore F, Lazarus JM, Brady HR (1996) Therapeutic plasma exchange in renal diseases. J Am Soc Nephrol 7:367–386PubMed Madore F, Lazarus JM, Brady HR (1996) Therapeutic plasma exchange in renal diseases. J Am Soc Nephrol 7:367–386PubMed
20.
go back to reference Guillevin L, Pagnoux C (2003) Indications of plasma exchanges for systemic vasculitides. Ther Apher Dial 7:155–160CrossRefPubMed Guillevin L, Pagnoux C (2003) Indications of plasma exchanges for systemic vasculitides. Ther Apher Dial 7:155–160CrossRefPubMed
21.
go back to reference 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 64:843–853CrossRefPubMed 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 64:843–853CrossRefPubMed
22.
go back to reference 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 54:1119–1123CrossRefPubMed 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 54:1119–1123CrossRefPubMed
23.
go back to reference Spizzo G, Mitterer M, Gunsilius E (2010) Bortezomib for the treatment of refractory type I cryoglobulinaemia. Br J Haematol 150:235–237PubMed Spizzo G, Mitterer M, Gunsilius E (2010) Bortezomib for the treatment of refractory type I cryoglobulinaemia. Br J Haematol 150:235–237PubMed
24.
go back to reference Talamo G, Claxton D, Tricot G, Fink L, Zangari M (2008) Response to bortezomib in refractory type I cryoglobulinemia. Am J Hematol 83:883–884CrossRefPubMed Talamo G, Claxton D, Tricot G, Fink L, Zangari M (2008) Response to bortezomib in refractory type I cryoglobulinemia. Am J Hematol 83:883–884CrossRefPubMed
25.
go back to reference 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 97:800–803CrossRefPubMed 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 97:800–803CrossRefPubMed
26.
go back to reference 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 370:362–373CrossRefPubMed 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 370:362–373CrossRefPubMed
27.
go back to reference 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):e12416CrossRef 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):e12416CrossRef
Metadata
Title
The complexity of an overlap type resistant cryoglobulinemia: a case report and review of the literature
Authors
Milena Tocut
Ziv Rozman
Alexander Biro
Asher Winder
Amir Tanay
Gisele Zandman-Goddard
Publication date
01-05-2019
Publisher
Springer London
Published in
Clinical Rheumatology / Issue 5/2019
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-018-04423-y

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