Patience and commitment needed to fight very rare tumours
Mené au Japon sur 37 patients atteints d'un lymphome intravasculaire à grandes cellules B (âge : de 20 à 79 ans ; durée médiane de suivi : 3,9 ans), cet essai multicentrique de phase II évalue l'efficacité, du point de vue du taux de survie sans progression à 2 ans, et la toxicité d'un traitement combinant une chimiothérapie de type R-CHOP, une forte dose de méthotrexate et une chimiothérapie intrathécale par méthotrexate-cytarabine-prednisolone
Intravascular large B-cell lymphoma (IVLBCL) is a very rare and aggressive neoplasm characterised by selective growth of tumour cells within blood vessel lumina. This peculiar growth pattern, the virtual absence of tumour masses, and the multifaceted and severe presentation make IVLBCL an attractive study model, but published work on this disease is scarce and fragmentary. More than a decade ago, international researchers established diagnostic and therapeutic recommendations for IVLBCL and distinguished two clinical forms: classic IVLBCL, which affects almost all patients diagnosed in European and North American countries; and a haemophagocytic syndrome-related form, which is a more aggressive type and is frequently diagnosed in Japan. After two studies from the Japanese Society of Hematology and the International Extranodal Lymphoma Study Group 3 showed a positive survival effect of rituximab, most patients with IVLBCL have been treated with R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone). Subsequent studies have shown that the CNS is the main site of recurrence after treatment with R-CHOP providing the rationale for addition of a CNS-oriented strategy to treatment regimens.
The Lancet Oncology , commentaire, 2019