ABSTRACT
PURPOSE: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare cancer, and large international cooperative efforts are needed to evaluate the significance of clinical risk factors and immunoarchitectural patterns (IAPs) for all stages of pediatric and adult patients with NLPHL. METHODS: Thirty-eight institutions participated in the Global nLPHL One Working Group retrospective study of NLPHL cases from 1992 to 2021. We measured progression-free survival (PFS), overall survival (OS), transformation rate, and lymphoma-specific death rate. We performed uni- and multivariable (MVA) Cox regression stratified by management to select factors for the lymphocyte-predominant international prognostic score (LP-IPS) validated by five-fold cross-validation. RESULTS: We identified 2,243 patients with a median age of 37 years (IQR, 23-51). The median follow-up was 6.3 years (IQR, 3.4-10.8). Most had stage I to II (72.9%) and few B symptoms (9.9%) or splenic involvement (5.4%). IAP was scored for 916 (40.8%). Frontline management included chemotherapy alone (32.4%), combined modality therapy (30.5%), radiotherapy alone (24.0%), observation after excision (4.6%), rituximab alone (4.0%), active surveillance (3.4%), and rituximab and radiotherapy (1.1%). The PFS, OS, transformation, and lymphoma-specific death rates at 10 years were 70.8%, 91.6%, 4.8%, and 3.3%, respectively. On MVA, IAPs were not associated with PFS or OS, but IAP E had higher risk of transformation (hazard ratio [HR], 1.81; P < .05). We developed the LP-IPS with 1 point each for age ≥45 years, stage III-IV, hemoglobin <10.5 g/dL, and splenic involvement. Increasing LP-IPS was significantly associated with worse PFS (HR, 1.52) and OS (HR, 2.31) and increased risk of lymphoma-specific death (HR, 2.63) and transformation (HR, 1.41). CONCLUSION: In this comprehensive study of all ages of patients with NLPHL, we develop the LP-IPS to identify high-risk patients and inform upcoming prospective clinical trials evaluating de-escalation of therapy for patients with low LP-IPS scores (<2).
ABSTRACT
OBJECTIVE: To compare the treatment of childhood immune thrombocytopenia (ITP) with historical practice in the UK. DESIGN: Data collection through a national UK Childhood ITP registry (http://www.uk-itp.org) started in January 2007. SETTINGS: UK hospitals. PARTICIPANTS: Children admitted with a new diagnosis of acute ITP and their treating physicians. MAIN OUTCOME MEASURES: Bleeding severity, platelet count, disease management and outcome from the time of presentation to 6 months. RESULTS: Data from 225 children were analysed. 54% of children had clinically mild, 42% had moderate and 4% had severe disease as defined previously. The mean platelet counts at diagnosis for these groups were 14, 8 and 6×10(9)/l respectively. Children with mild disease had fewer bleeding sites (1.9), compared with moderate (2.5) or severe disease (3.6). There was one intracranial haemorrhage reported. At 6 months' follow-up, 32% had a persistent platelet count <150×10(9)/l, but only 4.8% had a count <20. The proportion of UK children receiving platelet-raising treatment was noted to decrease from 61% in 1995 to 38% in 2000. The current UK 2009 registry data show a continued decrease in treatment to 16% of all the children. In contrast, historical international data report 69% of children receiving interventional therapy. CONCLUSION: The current UK practice has shown a continued reduction in the number of children receiving treatment in comparison with historical data and international practice.
