Outcomes of Hodgkin variant Richter transformation in chronic lymphocytic leukaemia and small lymphocytic lymphoma in British Columbia

Summary Hodgkin variant Richter transformation (HvRT) is a rare and challenging complication of chronic lymphocytic leukaemia (CLL) for which information on prognostic factors and treatment approaches remain limited. We analysed characteristics and survival outcomes of a population‐based cohort of 3...

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Published in:British journal of haematology 2022-08, Vol.198 (4), p.684-692
Main Authors: Zhu, Kai, Jamroz, Andrew, Huang, Steven, Villa, Diego, Freeman, Ciara L., Scott, David W., Slack, Graham, Sehn, Laurie H., Connors, Joseph M., Toze, Cynthia L., Savage, Kerry J., Gerrie, Alina S.
Format: Article
Language:English
Subjects:
Chronic lymphocytic leukemia
CLL
Genetic transformation
Hematology
hodgkins lymphoma
L-Lactate dehydrogenase
Lactic acid
Leukemia
Lymphoma
lymphomas
Medical prognosis
Patients
Richter‐S syndrome
Survival
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Summary:Summary Hodgkin variant Richter transformation (HvRT) is a rare and challenging complication of chronic lymphocytic leukaemia (CLL) for which information on prognostic factors and treatment approaches remain limited. We analysed characteristics and survival outcomes of a population‐based cohort of 32 patients with HvRT identified in British Columbia over a 40‐year period. Median interval from CLL diagnosis to HvRT was 5.6 years (range, 0–33.6), with five cases diagnosed concurrently. Most patients (80%) had treatment for CLL prior to HvRT. Median age at HvRT was 71 years (range, 51–86) and the majority of patients had high‐risk disease, including stage 3–4 in 87% and International Prognostic Score (IPS) ≥ 4 in 65%. Two‐year progression‐free (PFS) and overall survival (OS) from HvRT were 47% (95% CI: 29%–64%) and 57% (95% CI: 38%–72%), respectively. OS from HvRT was significantly worse in those with anaemia (p = 0.02), elevated lactate dehydrogenase (p = 0.04), high IPS (p = 0.04), and worse performance status (p = 0.001). For those treated with curative‐intent ABVD/ABVD‐like therapy, 2‐year PFS and OS were 70% (95% CI: 45%–85%) and 74% (95% CI: 49%–89%), respectively. In this real‐world population‐based cohort, HvRT was associated with poor clinical outcomes overall; however, those able to tolerate curative‐intent therapy had similar survival to older patients with de novo HL.
ISSN:0007-1048
1365-2141
DOI:10.1111/bjh.18241