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The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases.

Terrier B, Karras A, Kahn JE, Le Guenno G, Marie I, Benarous L, Lacraz A, Diot E, Hermine O, de Saint-Martin L, Cathébras P, Leblond V, Modiano P, Léger JM, Mariette X, Senet P, Plaisier E, Saadoun D, Cacoub P - Medicine (Baltimore) (2013)

Bottom Line: Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce.Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested.Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Groupe Hospitalier Pitié-Salpetrière, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie, Paris 6, Paris, France.

ABSTRACT
Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce. To analyze the presentation, prognosis, and efficacy and safety of treatments of type I CryoVas, we conducted a French nationwide survey that included 64 patients with type I CryoVas between January 1995 and July 2010: 28 patients with monoclonal gammopathy of unknown significance (MGUS) and 36 with hematologic malignancy.Type I monoclonal CryoVas was characterized by severe cutaneous involvement (necrosis and ulcers) in almost half the patients and high serum cryoglobulin levels, contrasting with a lower frequency of glomerulonephritis than expected. The 1-, 3-, 5-, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively. Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis. Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested. Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.

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Therapeutic management and outcome of patients with MGUS-related CryoVas. Abbreviations: See previous figures; CYC = cyclophosphamide, Lenal = lenalinomide, RFC = RTX/fludarabine/CYC, RR = responder-relapser.
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Figure 4: Therapeutic management and outcome of patients with MGUS-related CryoVas. Abbreviations: See previous figures; CYC = cyclophosphamide, Lenal = lenalinomide, RFC = RTX/fludarabine/CYC, RR = responder-relapser.

Mentions: Therapeutic management of patients with MGUS-related CryoVas is summarized in Table 2 and Figure 4. First-line therapy in patients with MGUS-related CryoVas was mainly based on corticosteroids alone, while second-line therapy included mainly rituximab- and alkylating agents-based regimens. Both of these regimens seem to provide good efficacy, but no comparison can be made between these therapeutic strategies.


The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases.

Terrier B, Karras A, Kahn JE, Le Guenno G, Marie I, Benarous L, Lacraz A, Diot E, Hermine O, de Saint-Martin L, Cathébras P, Leblond V, Modiano P, Léger JM, Mariette X, Senet P, Plaisier E, Saadoun D, Cacoub P - Medicine (Baltimore) (2013)

Therapeutic management and outcome of patients with MGUS-related CryoVas. Abbreviations: See previous figures; CYC = cyclophosphamide, Lenal = lenalinomide, RFC = RTX/fludarabine/CYC, RR = responder-relapser.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4553985&req=5

Figure 4: Therapeutic management and outcome of patients with MGUS-related CryoVas. Abbreviations: See previous figures; CYC = cyclophosphamide, Lenal = lenalinomide, RFC = RTX/fludarabine/CYC, RR = responder-relapser.
Mentions: Therapeutic management of patients with MGUS-related CryoVas is summarized in Table 2 and Figure 4. First-line therapy in patients with MGUS-related CryoVas was mainly based on corticosteroids alone, while second-line therapy included mainly rituximab- and alkylating agents-based regimens. Both of these regimens seem to provide good efficacy, but no comparison can be made between these therapeutic strategies.

Bottom Line: Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce.Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested.Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Groupe Hospitalier Pitié-Salpetrière, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie, Paris 6, Paris, France.

ABSTRACT
Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce. To analyze the presentation, prognosis, and efficacy and safety of treatments of type I CryoVas, we conducted a French nationwide survey that included 64 patients with type I CryoVas between January 1995 and July 2010: 28 patients with monoclonal gammopathy of unknown significance (MGUS) and 36 with hematologic malignancy.Type I monoclonal CryoVas was characterized by severe cutaneous involvement (necrosis and ulcers) in almost half the patients and high serum cryoglobulin levels, contrasting with a lower frequency of glomerulonephritis than expected. The 1-, 3-, 5-, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively. Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis. Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested. Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.

Show MeSH
Related in: MedlinePlus