The British Journal of Haematology publishes original research papers in clinical, laboratory and experimental haematology. The Journal also features annotations, reviews, short reports, images in haematology and Letters to the Editor.
Since its initial description, classical Hodgkin lymphoma (cHL) portends a greatly improved prognosis and the goal of treatment in most patients is cure with minimisation of toxicity from treatment. Outcomes in older patients (>60 years old) lag behind those of their younger counterparts however, and cure remains achievable mostly for those who can tolerate full doses of conventional chemotherapy. This review addresses the difference in biology between younger and older patients with cHL and examines the impact of frailty and comorbidities on outcomes. The toxicities of conventional chemotherapy in anthracycline-fit and -unfit patients are examined, with a particular focus on pulmonary toxicity associated with bleomycin in older patients. New advances are discussed, including the possibility of using more targeted therapies such as the anti-CD30 antibody brentuximab vedotin (BV) and checkpoint inhibitors as a method of reducing dependency on conventional chemotherapy for those less well able to tolerate it. Treatment of older patients with cHL remains an area of unmet need in hematological research, and efforts to rectify this knowledge gap should continue.
The disease shift of chronic lymphocytic leukemia (CLL) to diffuse large B-cell lymphoma (DLBCL), so-called Richter transformation (RT), is a catastrophic clinical event. Rarely, survival can outperform expectations and accurate prognostication for patients may affect therapeutic choices. To date, prognosis has relied on readily available factors such as TP53 disruption, previous CLL treatment status, and performance score. Recently, a shared clonality assessment by immunoglobulin heavy-chain variable region gene (IgHV) sequencing of the CLL and RT has been considered therapeutically relevant, but this is infrequently performed. We performed a systematic review of peer-reviewed manuscripts where outcomes in relation to clonal relatedness and lack thereof (clonally related and clonally unrelated RT-DLBCL) were examined. Fifteen manuscripts that included 336 patients were found, of which 6 compared survival outcomes between the 2 groups in a statistically meaningful way. Two analyses showed no difference in survival outcomes with 4 studies reporting a significantly poorer prognosis with clonally related RT-DLBCL. In 2 of these studies, the baseline characteristics of clonally related and clonally unrelated groups were compared and the clonally related cases were enriched for underlying CLL, which was TP53 disrupted, IgHV unmutated, more heavily pretreated, and exhibiting stereotyped B-cell receptor VH CDR3 and RT-DLBCL, which was MYD88 wild type. We demonstrate that although clonal relatedness of the underlying CLL confers a poorer survival, this is not demonstrated in any study to be independent of other well-described clinical and genomic variables known to influence outcomes in RT-DLBCL. Further independent validation of this prognostic factor is required to help guide universal adoption into clinical practice.
21 December 2017 in Irish Journal of Medical Science (1971 -)
Central nervous system (CNS) involvement by mantle cell lymphoma (MCL) is rare and portends a poor prognosis. We describe the first patient to have a complete response with front‐line treatment with single‐agent acalabrutinib for MCL CNS.