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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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Related in: MedlinePlus

Kaplan-Meier survival curve in 64 patients with type I CryoVas included in the survey (A), and according to the type of B-cell lymphoproliferative disorder (B). Survival rates were compared using the log-rank test in univariate analysis.
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Figure 6: Kaplan-Meier survival curve in 64 patients with type I CryoVas included in the survey (A), and according to the type of B-cell lymphoproliferative disorder (B). Survival rates were compared using the log-rank test in univariate analysis.

Mentions: After a mean follow-up of 46.2 ± 42.0 months, 8 patients (13%) were found to have severe infections, including bacterial septicemia in 3 patients, bacterial pneumonia in 3 patients, herpes virus infection in 2 patients, and cutaneous infection in 1 patient. Three of the 23 patients (13%) receiving rituximab experienced worsening vasculitis within 48 hours following the rituximab infusion. All 3 patients had lymphoplasmacytic lymphoma associated with high cryoglobulin levels (5.20, 2.75, and 2.14 g/L, respectively). In 1 of these patients, retreatment with rituximab was associated with a new episode of vasculitis flare. Four deaths (7%) were noted and were attributed to sepsis (n = 2), hemopathy (n = 1), and an unknown cause (n = 1). All patients who died had hematologic malignancy, including Waldenström macroglobulinemia in 3 patients and multiple myeloma in 1 patient. The 1-year, 3-year, 5-year, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively (Figure 6A). Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis (p = 0.06) (Figure 6B).


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)

Kaplan-Meier survival curve in 64 patients with type I CryoVas included in the survey (A), and according to the type of B-cell lymphoproliferative disorder (B). Survival rates were compared using the log-rank test in univariate analysis.
© Copyright Policy
Related In: Results  -  Collection

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

Figure 6: Kaplan-Meier survival curve in 64 patients with type I CryoVas included in the survey (A), and according to the type of B-cell lymphoproliferative disorder (B). Survival rates were compared using the log-rank test in univariate analysis.
Mentions: After a mean follow-up of 46.2 ± 42.0 months, 8 patients (13%) were found to have severe infections, including bacterial septicemia in 3 patients, bacterial pneumonia in 3 patients, herpes virus infection in 2 patients, and cutaneous infection in 1 patient. Three of the 23 patients (13%) receiving rituximab experienced worsening vasculitis within 48 hours following the rituximab infusion. All 3 patients had lymphoplasmacytic lymphoma associated with high cryoglobulin levels (5.20, 2.75, and 2.14 g/L, respectively). In 1 of these patients, retreatment with rituximab was associated with a new episode of vasculitis flare. Four deaths (7%) were noted and were attributed to sepsis (n = 2), hemopathy (n = 1), and an unknown cause (n = 1). All patients who died had hematologic malignancy, including Waldenström macroglobulinemia in 3 patients and multiple myeloma in 1 patient. The 1-year, 3-year, 5-year, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively (Figure 6A). Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis (p = 0.06) (Figure 6B).

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