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Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma (HL) with an annual incidence of approximately 1-3/1 million, accounting for all new cases of HL 5-15%
of .
The course of NLPHL is often painless, and at diagnosis it is often localized disease with a risk of advanced disease progression and histological transformation to large B-cell lymphoma
.
Compared with classical Hodgkin lymphoma (cHL), patients with NLPHL have better survival and lower excess mortality, mainly including death after transformation to large B-cell lymphoma, treatment-related secondary malignancies and Cardiovascular disease, etc.
lead to death
.
Because most cancer registries do not record disease transformation status, there are currently limited data on disease transformation in Finnish NLPHL patients
.
Ilja Kalashnikov et al conducted a Finnish national population-based study to assess the risk and relative survival of transformation to large B-cell lymphoma in Finnish patients diagnosed with NLPHL from 1995 to 2018, comparing disease transformation with and without disease transformation mortality of patients
.
>>>Research Methods The data of this study came from the Finnish Cancer Registry Center (FCR).
The study retrieved a total of 485 patients diagnosed with NLPHL in Finland from 1995 to 2018, excluding 11 patients with incorrect or missing registration, Five patients were diagnosed with other lymphomas before the diagnosis of NLPHL, and 16 patients were diagnosed with NLPHL combined with large B-cell lymphoma (ie, complex lymphoma), and the remaining 453 patients were included in the study analysis
.
The study defined disease transformation as morphologically confirmed large B-cell lymphoma or high-grade B-cell lymphoma at least 3 months after the initial diagnosis of NLPHL
.
For all NLPHL patients, the study collected all patients' gender, age, NLPHL diagnosis and time of last follow-up, vital status at the end of follow-up, date of transformation in patients with disease transformation, and cause of death in patients who died
.
Data on deaths of NLPHL patients were obtained from Statistics Finland, and the causes of death were determined according to the International Classification of Diseases (ICD-10) edited by the World Health Organization (WHO) and were classified into three groups: lymphoma, secondary malignancies, or other causes
.
>>>Study Results The 453 patients were more male (76%), the median age at diagnosis was 48 years (range 5-87 years), and the age at diagnosis was significantly greater in women than in men (median age, 58 years vs 45 years, p <0.
001), with a median follow-up of 8.
1 years (range 0-24 years)
.
The risk of disease transformation After follow-up, a total of 25 patients had disease transformation (crude transformation rate 6.
2/1000), of which the cumulative incidence of transformation 5 years after diagnosis was 3.
5% (95%CI 2.
1-5.
9), and the cumulative incidence of 10 years was 3.
5%.
The incidence of conversion was 6.
3% (95% CI 4.
2-9.
6), after which a plateau was reached (Figure 1)
.
The majority of patients with disease transformation (22 of 25) occurred within 10 years of diagnosis, with one occurring between 19 and 20 years
.
Of the 25 patients with disease conversion, 18 (72%) were male, and the median age at conversion was 57 years (range 27-87 years)
.
Risk of disease transformation with age at diagnosis (95%CI 0.
94-1.
57; p=0.
13), sex (95%CI 0.
50-3.
18; p=0.
6), year of diagnosis (95%CI 0.
37-1.
43; p=0.
4) There were no significant correlations
.
At transformation, histological diagnosis revealed diffuse large B-cell lymphoma in 15 (60%) patients and T/HRLBCL in 10 (40%) patients
.
Figure 1 Overall survival After follow-up, 78 patients died (crude death rate 18.
6/1000)
.
Overall survival was 85% (95%CI 81%-89%) at 10 years after diagnosis and 65% (95%CI 58%-74%) at 20 years
.
12 patients with disease transformation died, the crude death rate was 974/1000, and the crude death rate of patients without disease transformation was 16.
3/1000
.
After adjusting for potential confounders (age at diagnosis, sex, and year of diagnosis), disease transformation was associated with a substantial increase in overall mortality (95% CI 4.
49-16.
3, p < 0.
001) (Table 1)
.
In patients with disease transformation, the time to transformation was probably the greatest relative risk factor for death (Figure 2), with a hazard ratio (HR) of 15.
0 (95%CI 7.
16-31.
4) for 1, 5, and 10 years of transformation, respectively.
), 3.
38 (95%CI 0.
86-13.
4) and 3.
00 (95%CI 0.
79-11.
5)
.
Table 1Figure 2 Relative survival and excessive risk of death Over the entire follow-up period from 1995 to 2018, the 10-year age-standardized relative survival rate was 94% (95% CI 89%-100%) (Figure 3A)
.
At different times, the 5-year age-standardized relative survival rate was 96% (95%CI 92%-100%) in 2007-2018 and 89% (95%CI 83%-95%) in 1995-2006 (Figure 3B )
.
Adjusted mortality rates are shown in Table 1
.
Older age at diagnosis was associated with higher mortality (95%CI 1.
15-2.
66; p=0.
009), while patients diagnosed in later years had a lower risk of excess mortality (95%CI 0.
15-0.
98; p=0.
046)
.
Disease transformation was associated with a substantial increase in excess mortality (95% CI 9.
41-75.
9; p<0.
001), but the relative risk of excess mortality likely decreased over time, at 1, 5, and 10 years after transformation Dependent excess HRs were 30.
7 (95%CI 12.
2-77.
1), 6.
79 (95%CI 1.
16-39.
6) and 7.
39 (95%CI 1.
18-46.
1), respectively
.
Figure 3 Causes of death During the study period, a total of 78 patients died, including 32 patients (41%) from lymphoma, 14 (18%) from secondary malignancies, and 32 (41%) from other Causes, the most common of which are cardiovascular disease and infectious diseases
.
Of the 12 patients with disease transformation, 11 (92%) died of lymphoma
.
>>>Study Conclusions This population-based long-term follow-up cohort study showed that Finnish patients diagnosed with NLPHL from 1995 to 2018 had good long-term relative survival
.
Excess mortality was lower among NLPHL patients diagnosed in recent years
.
The risk of transformation to aggressive B-cell lymphoma 10 years after diagnosis was 6.
3%, and disease transformation was associated with a significantly increased risk of death
.
Reference: Kalashnikov I, Tanskanen T, Pitkäniemi J, et al.
Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study.
Blood Cancer J.
2021;11(12):1–6.
Poke" Read the original", we make progress together
of .
The course of NLPHL is often painless, and at diagnosis it is often localized disease with a risk of advanced disease progression and histological transformation to large B-cell lymphoma
.
Compared with classical Hodgkin lymphoma (cHL), patients with NLPHL have better survival and lower excess mortality, mainly including death after transformation to large B-cell lymphoma, treatment-related secondary malignancies and Cardiovascular disease, etc.
lead to death
.
Because most cancer registries do not record disease transformation status, there are currently limited data on disease transformation in Finnish NLPHL patients
.
Ilja Kalashnikov et al conducted a Finnish national population-based study to assess the risk and relative survival of transformation to large B-cell lymphoma in Finnish patients diagnosed with NLPHL from 1995 to 2018, comparing disease transformation with and without disease transformation mortality of patients
.
>>>Research Methods The data of this study came from the Finnish Cancer Registry Center (FCR).
The study retrieved a total of 485 patients diagnosed with NLPHL in Finland from 1995 to 2018, excluding 11 patients with incorrect or missing registration, Five patients were diagnosed with other lymphomas before the diagnosis of NLPHL, and 16 patients were diagnosed with NLPHL combined with large B-cell lymphoma (ie, complex lymphoma), and the remaining 453 patients were included in the study analysis
.
The study defined disease transformation as morphologically confirmed large B-cell lymphoma or high-grade B-cell lymphoma at least 3 months after the initial diagnosis of NLPHL
.
For all NLPHL patients, the study collected all patients' gender, age, NLPHL diagnosis and time of last follow-up, vital status at the end of follow-up, date of transformation in patients with disease transformation, and cause of death in patients who died
.
Data on deaths of NLPHL patients were obtained from Statistics Finland, and the causes of death were determined according to the International Classification of Diseases (ICD-10) edited by the World Health Organization (WHO) and were classified into three groups: lymphoma, secondary malignancies, or other causes
.
>>>Study Results The 453 patients were more male (76%), the median age at diagnosis was 48 years (range 5-87 years), and the age at diagnosis was significantly greater in women than in men (median age, 58 years vs 45 years, p <0.
001), with a median follow-up of 8.
1 years (range 0-24 years)
.
The risk of disease transformation After follow-up, a total of 25 patients had disease transformation (crude transformation rate 6.
2/1000), of which the cumulative incidence of transformation 5 years after diagnosis was 3.
5% (95%CI 2.
1-5.
9), and the cumulative incidence of 10 years was 3.
5%.
The incidence of conversion was 6.
3% (95% CI 4.
2-9.
6), after which a plateau was reached (Figure 1)
.
The majority of patients with disease transformation (22 of 25) occurred within 10 years of diagnosis, with one occurring between 19 and 20 years
.
Of the 25 patients with disease conversion, 18 (72%) were male, and the median age at conversion was 57 years (range 27-87 years)
.
Risk of disease transformation with age at diagnosis (95%CI 0.
94-1.
57; p=0.
13), sex (95%CI 0.
50-3.
18; p=0.
6), year of diagnosis (95%CI 0.
37-1.
43; p=0.
4) There were no significant correlations
.
At transformation, histological diagnosis revealed diffuse large B-cell lymphoma in 15 (60%) patients and T/HRLBCL in 10 (40%) patients
.
Figure 1 Overall survival After follow-up, 78 patients died (crude death rate 18.
6/1000)
.
Overall survival was 85% (95%CI 81%-89%) at 10 years after diagnosis and 65% (95%CI 58%-74%) at 20 years
.
12 patients with disease transformation died, the crude death rate was 974/1000, and the crude death rate of patients without disease transformation was 16.
3/1000
.
After adjusting for potential confounders (age at diagnosis, sex, and year of diagnosis), disease transformation was associated with a substantial increase in overall mortality (95% CI 4.
49-16.
3, p < 0.
001) (Table 1)
.
In patients with disease transformation, the time to transformation was probably the greatest relative risk factor for death (Figure 2), with a hazard ratio (HR) of 15.
0 (95%CI 7.
16-31.
4) for 1, 5, and 10 years of transformation, respectively.
), 3.
38 (95%CI 0.
86-13.
4) and 3.
00 (95%CI 0.
79-11.
5)
.
Table 1Figure 2 Relative survival and excessive risk of death Over the entire follow-up period from 1995 to 2018, the 10-year age-standardized relative survival rate was 94% (95% CI 89%-100%) (Figure 3A)
.
At different times, the 5-year age-standardized relative survival rate was 96% (95%CI 92%-100%) in 2007-2018 and 89% (95%CI 83%-95%) in 1995-2006 (Figure 3B )
.
Adjusted mortality rates are shown in Table 1
.
Older age at diagnosis was associated with higher mortality (95%CI 1.
15-2.
66; p=0.
009), while patients diagnosed in later years had a lower risk of excess mortality (95%CI 0.
15-0.
98; p=0.
046)
.
Disease transformation was associated with a substantial increase in excess mortality (95% CI 9.
41-75.
9; p<0.
001), but the relative risk of excess mortality likely decreased over time, at 1, 5, and 10 years after transformation Dependent excess HRs were 30.
7 (95%CI 12.
2-77.
1), 6.
79 (95%CI 1.
16-39.
6) and 7.
39 (95%CI 1.
18-46.
1), respectively
.
Figure 3 Causes of death During the study period, a total of 78 patients died, including 32 patients (41%) from lymphoma, 14 (18%) from secondary malignancies, and 32 (41%) from other Causes, the most common of which are cardiovascular disease and infectious diseases
.
Of the 12 patients with disease transformation, 11 (92%) died of lymphoma
.
>>>Study Conclusions This population-based long-term follow-up cohort study showed that Finnish patients diagnosed with NLPHL from 1995 to 2018 had good long-term relative survival
.
Excess mortality was lower among NLPHL patients diagnosed in recent years
.
The risk of transformation to aggressive B-cell lymphoma 10 years after diagnosis was 6.
3%, and disease transformation was associated with a significantly increased risk of death
.
Reference: Kalashnikov I, Tanskanen T, Pitkäniemi J, et al.
Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study.
Blood Cancer J.
2021;11(12):1–6.
Poke" Read the original", we make progress together
