Background. Intravascular large B-cell lymphoma (IVL) is a rare form of non-Hodgkin lymphoma, characterized by the growth of large neoplastic lymphocytes within blood vessels. Diagnosis is often difficult and outcome generally poor. The standard therapy remains to be defined; anthracycline-based chemotherapy is the most commonly used treatment. A few recent case reports seem to suggest a positive impact on outcome of the addition of rituximab to chemotherapy. Aim. to explore activity and efficacy of rituximab (375 mg/m2 on day 1) plus CHOP or CHOP-like chemotherapy (R-CT) in comparison to the same chemotherapy alone (CT) in CD20-positive IVL patients selected from the largest series collected in Western Countries [Ferreri AJM, et al. Haematologica, 2007]. Methods. 27 IVL patients eligible for anthracycline-containing regimen were evaluated. Clinical features and outcome of 7 patients treated with R-CT strategy were compared to a group of 20 patients treated with CT. Results. Median age of patients was 67 yrs (range 39-86 yrs). Fourteen patients were males. The most commonly involved organs were: skin (12 patients), CNS (7), bone marrow (8), spleen (5), and liver (4). B symptoms were complained by 20 (74%) patients and an elevation of serum LDH was present in 21/24 (88%). Anemia, leukopenia and thrombocytopenia were observed in 20/26 (77%), 8/26 (30%), and 9/26 (35%) cases, respectively. Nineteen (73%) patients presented with stage IV disease. No significant differences in patients’ characteristics between R-CT and CT subgroups were observed. After a median follow-up of 26 months, 15 patients achieved complete remission (CR), and 5 partial response (PR) (R-CT is still ongoing in 2 PR patients); 6 patients experimented progressive disease, and one patient (CT subgroup) died of toxicity All the R-CT patients (5) who completed the treatment (100%) and 10 (50%) CT patients achieved CR (p=0.06). The only variable related to CR rate was the addition of rituximab. All lymphoma-related events in CT patients were observed within the first year of follow-up; 6 CT patients are alive and disease-free at a median follow-up of 71 months. Conversely, all the 7 R-CT patients are alive and relapse-free at a median follow-up of 16 months. The 2-year EFS was 35% in CT group and 100% in R-CT group (p<0.0001). The 2-year OS was 45% and 100%, respectively for CT and R-CT group (p<0.0001). Conclusions. this international study suggests that, like for other CD20-positive lymphomas, the addition of rituximab to anthracycline-based chemotherapy could significantly improve outcome in IVL, a rare malignancy where prospective trials are lacking so far. Confirmatory analysis after a longer follow-up is warranted

Therapeutic role of rituximab in the treatment of intravascular large B-cell lymphoma / G. P., Dognini; A., Ponzoni; C., Doglioni; Martelli, Maurizio; A., Ambrosetti; G., Rossi; M., Federico; A., Candoni; A., De Renzo; O., Bairey; A., Szomor; B., Horvath; L., Uziel; J., Seymour; R., Willemze; A. J. M., Ferreri. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 92 (SUPPL. 3):(2007), pp. 186-186. (Intervento presentato al convegno 41st Congress of the Italian-Society-of-Hematology tenutosi a Bologna, ITALY nel OCT 14-17, 2007).

Therapeutic role of rituximab in the treatment of intravascular large B-cell lymphoma

MARTELLI, Maurizio;
2007

Abstract

Background. Intravascular large B-cell lymphoma (IVL) is a rare form of non-Hodgkin lymphoma, characterized by the growth of large neoplastic lymphocytes within blood vessels. Diagnosis is often difficult and outcome generally poor. The standard therapy remains to be defined; anthracycline-based chemotherapy is the most commonly used treatment. A few recent case reports seem to suggest a positive impact on outcome of the addition of rituximab to chemotherapy. Aim. to explore activity and efficacy of rituximab (375 mg/m2 on day 1) plus CHOP or CHOP-like chemotherapy (R-CT) in comparison to the same chemotherapy alone (CT) in CD20-positive IVL patients selected from the largest series collected in Western Countries [Ferreri AJM, et al. Haematologica, 2007]. Methods. 27 IVL patients eligible for anthracycline-containing regimen were evaluated. Clinical features and outcome of 7 patients treated with R-CT strategy were compared to a group of 20 patients treated with CT. Results. Median age of patients was 67 yrs (range 39-86 yrs). Fourteen patients were males. The most commonly involved organs were: skin (12 patients), CNS (7), bone marrow (8), spleen (5), and liver (4). B symptoms were complained by 20 (74%) patients and an elevation of serum LDH was present in 21/24 (88%). Anemia, leukopenia and thrombocytopenia were observed in 20/26 (77%), 8/26 (30%), and 9/26 (35%) cases, respectively. Nineteen (73%) patients presented with stage IV disease. No significant differences in patients’ characteristics between R-CT and CT subgroups were observed. After a median follow-up of 26 months, 15 patients achieved complete remission (CR), and 5 partial response (PR) (R-CT is still ongoing in 2 PR patients); 6 patients experimented progressive disease, and one patient (CT subgroup) died of toxicity All the R-CT patients (5) who completed the treatment (100%) and 10 (50%) CT patients achieved CR (p=0.06). The only variable related to CR rate was the addition of rituximab. All lymphoma-related events in CT patients were observed within the first year of follow-up; 6 CT patients are alive and disease-free at a median follow-up of 71 months. Conversely, all the 7 R-CT patients are alive and relapse-free at a median follow-up of 16 months. The 2-year EFS was 35% in CT group and 100% in R-CT group (p<0.0001). The 2-year OS was 45% and 100%, respectively for CT and R-CT group (p<0.0001). Conclusions. this international study suggests that, like for other CD20-positive lymphomas, the addition of rituximab to anthracycline-based chemotherapy could significantly improve outcome in IVL, a rare malignancy where prospective trials are lacking so far. Confirmatory analysis after a longer follow-up is warranted
2007
41st Congress of the Italian-Society-of-Hematology
04 Pubblicazione in atti di convegno::04d Abstract in atti di convegno
Therapeutic role of rituximab in the treatment of intravascular large B-cell lymphoma / G. P., Dognini; A., Ponzoni; C., Doglioni; Martelli, Maurizio; A., Ambrosetti; G., Rossi; M., Federico; A., Candoni; A., De Renzo; O., Bairey; A., Szomor; B., Horvath; L., Uziel; J., Seymour; R., Willemze; A. J. M., Ferreri. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 92 (SUPPL. 3):(2007), pp. 186-186. (Intervento presentato al convegno 41st Congress of the Italian-Society-of-Hematology tenutosi a Bologna, ITALY nel OCT 14-17, 2007).
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/485124
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