Search

This article discusses the use of brentuximab vedotin (BV) for the treatment of relapsed or refractory anaplastic large-cell lymphoma (ALCL) in pediatric patients. The study focuses on the role of minimal residual disease (MRD) monitoring using droplet digital PCR (dPCR) to assess treatment response and predict relapse. The results suggest that multiple cycles of BV are necessary to achieve complete remission, and MRD positivity may serve as an early indicator of relapse. The study acknowledges its limitations due to its retrospective nature and small sample size. The research was supported by various funding sources and the authors declare no conflicts of interest. [Extracted from the article]

Summary: Relapses involving the central nervous system (CNS) are rare in children and adolescents with ALK+ anaplastic large cell lymphoma (ALCL) treated with regimens including CNS prophylaxis. Early identification of patients at high‐risk for CNS relapse would enable stratification and better adaptation of initial treatment especially in the light of the upcoming targeted therapies with limited CNS penetration. We analyzed clinical and histological data of all ALK+ALCL patients with CNS relapse registered in ALCL99‐database with the aim to describe risk factors and outcome. Characteristics of patients with no relapse, relapse without CNS involvement and CNS relapse were compared. At a median follow‐up of 8 years (0.05–18 years), a CNS involvement was reported at first or subsequent relapse in 26/618 patients. Median interval between initial diagnosis and first CNS relapse was 8 months (IQR 5.55–10.61/range 1.31–130.69). The 5‐year cumulative risk of CNS relapse was 4% (95% CI 2.9–5.5). Bone marrow involvement, peripheral blasts and CNS involvement at diagnosis were more frequent in patients with CNS relapse than in patients with no relapse or with relapse with no CNS involvement. The treatment of CNS relapse was heterogeneous. The median survival after CNS relapse was 23.7 months. Eleven patients were alive at last follow‐up. Three‐year overall survival after CNS relapse was 48.70% (95% CI 30.52–67.23). [ABSTRACT FROM AUTHOR]

Interferon-gamma (IFN-gamma) mediates the final damage of the stem cell compartment in Aplastic Anaemia (AA). Normal subjects homozygous for 12 (CA) repeats of polymorphism variable number of dinucleotide (CA) repeat (VNDR) in position 1349 of the IFN-gamma gene (IFNG) were shown to overproduce IFN-gammain vitro. We studied the distribution of polymorphism VNDR 1349 of IFNG in 67 Caucasian AA patients and in normal controls. Genotype (CA)12-12, (homozygosis for allele 2) and the single allele 12 were significantly more frequent (P = 0.005 and 0.004 respectively) in patients versus controls. The polymorphism was equally distributed in AA patients regardless of their response to immunosuppression. Homozygosity for 12 (CA) repeats of polymorphism VNDR 1349 of IFNG is strongly associated with the risk of AA in Caucasian subjects.