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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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Efficacy of alkylating agents-based regimen in patients with type I CryoVas according to its use in first or second line. Abbreviations: B-NHL = B-cell non-Hodgkin lymphoma, Bor = bortezomib, CR = complete response, CS = corticosteroids, Dexa = dexamethasone, MGUS = monoclonal gammopathy of unknown significance, MM = multiple myeloma, NR = nonresponder, PR = partial response, RTX = rituximab, Thal = thalidomide.
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Figure 2: Efficacy of alkylating agents-based regimen in patients with type I CryoVas according to its use in first or second line. Abbreviations: B-NHL = B-cell non-Hodgkin lymphoma, Bor = bortezomib, CR = complete response, CS = corticosteroids, Dexa = dexamethasone, MGUS = monoclonal gammopathy of unknown significance, MM = multiple myeloma, NR = nonresponder, PR = partial response, RTX = rituximab, Thal = thalidomide.

Mentions: Alkylating agent-based regimen: Twenty-five patients received at least 1 course of alkylating agents (in the absence of associated multidrug chemotherapy) for the treatment of type I CryoVas, as first-line (n = 16) or second-line (n = 9) treatment. The initial response rate, which was assessable in 23 patients, was 83% (17 complete response [CR] and 2 partial response [PR]), although 13 of 19 (68%) responding patients relapsed (Figure 2).


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)

Efficacy of alkylating agents-based regimen in patients with type I CryoVas according to its use in first or second line. Abbreviations: B-NHL = B-cell non-Hodgkin lymphoma, Bor = bortezomib, CR = complete response, CS = corticosteroids, Dexa = dexamethasone, MGUS = monoclonal gammopathy of unknown significance, MM = multiple myeloma, NR = nonresponder, PR = partial response, RTX = rituximab, Thal = thalidomide.
© Copyright Policy
Related In: Results  -  Collection

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

Figure 2: Efficacy of alkylating agents-based regimen in patients with type I CryoVas according to its use in first or second line. Abbreviations: B-NHL = B-cell non-Hodgkin lymphoma, Bor = bortezomib, CR = complete response, CS = corticosteroids, Dexa = dexamethasone, MGUS = monoclonal gammopathy of unknown significance, MM = multiple myeloma, NR = nonresponder, PR = partial response, RTX = rituximab, Thal = thalidomide.
Mentions: Alkylating agent-based regimen: Twenty-five patients received at least 1 course of alkylating agents (in the absence of associated multidrug chemotherapy) for the treatment of type I CryoVas, as first-line (n = 16) or second-line (n = 9) treatment. The initial response rate, which was assessable in 23 patients, was 83% (17 complete response [CR] and 2 partial response [PR]), although 13 of 19 (68%) responding patients relapsed (Figure 2).

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