Background: Clinical studies on relatively large series of intravascular lymphoma (IVL) cases are lacking. Aim: To better delineate clinical forms, histopathological features, natural history, and appropriate therapy of IVL in western countries. Methods: Clinico-pathological data for the largest reported series of HIV-negative patients (pts) with in vivo (n = 30) or post-mortem (n = 10) diagnosis of IVL were reviewed. Results: Median age of the 40 pts was 70 years (34–90; M/F ratio: 1; ECOG-PS > 1 in 25 cases). B-symptoms (52% of cases; fever in 40%), cutaneous lesions (37%) and neurological symptoms (35%) were the most common presenting features. All cases but one were constituted by large B-cells; hemophagocytic syndrome was never observed. Ann Arbor stage was IE in 12 (30%) cases and IVE in 28 (70%). Ten pts (25%) showed a disease limited to the skin after conventional staging (defined as ‘cutaneous form’ of IVL). Hepatosplenic involvement was observed in 16 cases (40%) and marrow infiltration in 12 (30%), and 4 pts (10%) had nodal disease. Anemia was observed in 25 (62%) cases, thrombocytopenia in 12 (30%), elevated ESR in 16 (40%), and elevated serum LDH in 25/29 (86%) cases. Twenty-two of the 30 pts diagnosed ante-mortem were treated with chemotherapy (anthracycline-based in 19): response was CR in 10 cases, PR in 3 (ORR = 59%); 7 pts experienced PD and two died of toxicity. Seven responders experienced relapse. Three pts treated without anthracycline showed early PD. High-dose chemotherapy supported by ASCT was used in four cases: as consolidation after conventional chemotherapy in 2 pts, who are relapse-free at 19 and 47 months, and at relapse in the other two, who died early. Five pts treated with chemotherapy had CNS involvement: four of them (CHOP in 3, CVP in one) did not achieve remission and died early; the fifth patient was treated with MACOP-B regimen followed by high-dose chemotherapy and ASCT, being relapse-free at 19 months from diagnosis. Eight pts treated with chemotherapy are alive and with no evidence of disease (NED) (median follow-up 35 months). Eight pts with in vivo diagnosis did not receive chemotherapy: four died early due to IVL or complications, one died without disease progression at 24 months; three young women with ‘cutaneous form’ are alive and NED at 7–14 years. Overall, 11 pts are alive (9 NED) with a median follow-up of 41 months, and a 3-year OS of 24 ± 7%. Patients with ‘cutaneous form’ were all females with normal platelet and leukocyte counts and ECOG-PS < 2, exhibiting a better outcome than the others (3-year OS: 56 ± 16% versus 15 ± 7%; P = 0.0004). In multivariate analysis, ECOG-PS < 2, ‘cutaneous form’ and stage-I disease were independent favorable prognosticators. Conclusions: Hepatosplenic and bone marrow involvement is an unexpectedly common feature in IVL. The frequent involvement of the brain and skin and the absence of hemophagocytic syndrome are the main differences with respect to Japanese series. Therapeutic outcome is disappointing; early consolidation with high-dose chemotherapy and ASCT may improve survival in young pts with unfavorable features. Drugs with a higher CNS bioavailability should be used in cases with brain involvement. IVL limited to the skin (‘cutaneous form’) exhibits distinctive clinical properties that deserve to be better characterized on biological grounds.
CLINICAL PRESENTATION, BEHAVIOR, MANAGEMENT, AND OUTCOME OF AN INTERNATIONAL SERIES OF 40 CASES OF INTRAVASCULAR LYMPHOMA / M., Ponzoni; A., Ambrosetti; M., Lestani; F., Ilariucci; S., Asioli; E., Zucca; G., Rossi; M., Ungari; F., Facchetti; E., Berti; P., Iuzzolino; Martelli, Maurizio; M., Federico; T., Artusi; A., Candoni; S., Cortelazzo; T., Motta; A., De Renzo; M., Milani; R., Rossi; S., Ascani; S., Pileri; S., Dell'Oro; N., Frungillo; C., Doglioni; A. J. M., Ferreri. - In: ANNALS OF ONCOLOGY. - ISSN 0923-7534. - 15 (SUPPL. 2):(2004), pp. 81-81. (Intervento presentato al convegno 6th National Congress of Medical Oncology tenutosi a Bologna, ITALY nel 21-24 September, 2004) [10.1093/annonc/mdh707].
CLINICAL PRESENTATION, BEHAVIOR, MANAGEMENT, AND OUTCOME OF AN INTERNATIONAL SERIES OF 40 CASES OF INTRAVASCULAR LYMPHOMA
MARTELLI, Maurizio;
2004
Abstract
Background: Clinical studies on relatively large series of intravascular lymphoma (IVL) cases are lacking. Aim: To better delineate clinical forms, histopathological features, natural history, and appropriate therapy of IVL in western countries. Methods: Clinico-pathological data for the largest reported series of HIV-negative patients (pts) with in vivo (n = 30) or post-mortem (n = 10) diagnosis of IVL were reviewed. Results: Median age of the 40 pts was 70 years (34–90; M/F ratio: 1; ECOG-PS > 1 in 25 cases). B-symptoms (52% of cases; fever in 40%), cutaneous lesions (37%) and neurological symptoms (35%) were the most common presenting features. All cases but one were constituted by large B-cells; hemophagocytic syndrome was never observed. Ann Arbor stage was IE in 12 (30%) cases and IVE in 28 (70%). Ten pts (25%) showed a disease limited to the skin after conventional staging (defined as ‘cutaneous form’ of IVL). Hepatosplenic involvement was observed in 16 cases (40%) and marrow infiltration in 12 (30%), and 4 pts (10%) had nodal disease. Anemia was observed in 25 (62%) cases, thrombocytopenia in 12 (30%), elevated ESR in 16 (40%), and elevated serum LDH in 25/29 (86%) cases. Twenty-two of the 30 pts diagnosed ante-mortem were treated with chemotherapy (anthracycline-based in 19): response was CR in 10 cases, PR in 3 (ORR = 59%); 7 pts experienced PD and two died of toxicity. Seven responders experienced relapse. Three pts treated without anthracycline showed early PD. High-dose chemotherapy supported by ASCT was used in four cases: as consolidation after conventional chemotherapy in 2 pts, who are relapse-free at 19 and 47 months, and at relapse in the other two, who died early. Five pts treated with chemotherapy had CNS involvement: four of them (CHOP in 3, CVP in one) did not achieve remission and died early; the fifth patient was treated with MACOP-B regimen followed by high-dose chemotherapy and ASCT, being relapse-free at 19 months from diagnosis. Eight pts treated with chemotherapy are alive and with no evidence of disease (NED) (median follow-up 35 months). Eight pts with in vivo diagnosis did not receive chemotherapy: four died early due to IVL or complications, one died without disease progression at 24 months; three young women with ‘cutaneous form’ are alive and NED at 7–14 years. Overall, 11 pts are alive (9 NED) with a median follow-up of 41 months, and a 3-year OS of 24 ± 7%. Patients with ‘cutaneous form’ were all females with normal platelet and leukocyte counts and ECOG-PS < 2, exhibiting a better outcome than the others (3-year OS: 56 ± 16% versus 15 ± 7%; P = 0.0004). In multivariate analysis, ECOG-PS < 2, ‘cutaneous form’ and stage-I disease were independent favorable prognosticators. Conclusions: Hepatosplenic and bone marrow involvement is an unexpectedly common feature in IVL. The frequent involvement of the brain and skin and the absence of hemophagocytic syndrome are the main differences with respect to Japanese series. Therapeutic outcome is disappointing; early consolidation with high-dose chemotherapy and ASCT may improve survival in young pts with unfavorable features. Drugs with a higher CNS bioavailability should be used in cases with brain involvement. IVL limited to the skin (‘cutaneous form’) exhibits distinctive clinical properties that deserve to be better characterized on biological grounds.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.