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Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma. BL is curable for most patients with dose-intense multiple drug combination chemotherapy and improvements in supportive care. However, past definitions of low-risk BL have been inconsistent and >85–90% patients fall into the undifferentiated high-risk category. The lack of standardization for risk stratification in BL hinders the evaluation of targeted, individualized treatments based upon patient prognosis.
At the 62nd American Society of Hematology (ASH) Annual Meeting and Exposition held in December 2020, Adam Olszewski, Lifespan Cancer Institute, Providence, US, presented results from a retrospective cohort study that derived and validated a novel prognostic index for BL in geographically diverse patient cohorts.1 The data have subsequently been published in the Journal of Clinical Oncology.2 Adam Olszewski has shared his opinion on the development and validation of the BL International Prognostic Index (BL-IPI) with the Lymphoma Hub. Here we are pleased to summarize the study results.
Table 1. Baseline characteristics1,2
CNS, central nervous system; CODOX-M/IVAC ± R, cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, and high-dose cytarabine with or without rituximab; ECOG, Eastern Cooperative Oncology Group; DA-EPOCH-R, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab; HIV, human immunodeficiency virus; hyperCVAD/MA ± R, cyclophosphamide, vincristine, doxorubicin, and dexamethasone/high-dose methotrexate and cytarabine with or without rituximab; IQR, interquartile range; LDH, lactate dehydrogenase; OS, overall survival; PFS, progression-free survival; PS, performance status; ULN, upper limit of normal. |
||
Characteristic |
Derivation cohort (US) |
Validation cohort (international) |
---|---|---|
Age, median (IQR) |
47 (33–59) |
46 (34–59) |
≥ 40, n (%) |
401 (63) |
290 (64) |
≥ 60, n (%) |
146 (23) |
109 (24) |
Male, n (%) |
479 (76) |
351 (77) |
HIV-positive, n (%) |
140 (22) |
106 (23) |
PS ECOG ≥ 2, n (%) |
141 (22) |
131 (35) |
Stage 3 or 4, n (%) |
494 (78) |
363 (79) |
> 1 extra nodal site, n (%) |
270 (43) |
247 (54) |
CNS involvement, n (%) |
118 (19) |
47 (10) |
LDH ratio, median (IQR) |
2.5 (1.1–6.1) |
2.6 (1.1–7.1) |
LDH > ULN, n (%) |
465 (74) |
352 (77) |
LDH > 3 × ULN, n (%) |
268 (42) |
212 (46) |
LDH > 5 × ULN, n (%) |
178 (28) |
152 (33) |
Stage 1 or 2 with LDH ≤ ULN, n (%) |
53 (8) |
58 (13) |
First-line regimen, n (%) |
||
CODOX-M/IVAC ± R |
194 (31) |
298 (65) |
HyperCVAD/MA ± R |
195 (31) |
39 (9) |
DA-EPOCH-R |
181 (29) |
46 (10) |
Other |
63 (10) |
74 (16) |
Rituximab use, n (%) |
578 (91) |
432 (95) |
Median follow-up, months |
45 |
52 |
PFS at 3 years, 95% CI |
65 (61–69) |
75 (70–78) |
OS at 3 years, 95% CI |
70 (66–74) |
76 (72–80) |
Table 2. Univariable and multivariable analyses for association between candidate prognostic variables and PFS1,2
CI, confidence interval; CNS, central nervous system; ECOG, Eastern Cooperative Oncology Group; HR, hazard ratio; LDH, lactate dehydrogenase; PS, performance status; ULN, upper limit of normal. |
||||||
Variable |
Univariable |
Multivariable |
||||
---|---|---|---|---|---|---|
HR |
95% CI |
P |
HR |
95% CI |
P |
|
Age ≥ 40 years |
1.79 |
1.34–2.38 |
< 0.001 |
1.79 |
1.34–2.38 |
< 0.001 |
Female |
1.05 |
0.78–1.41 |
0.74 |
|
|
|
HIV-positive |
1.15 |
0.85–1.56 |
0.36 |
|
|
|
PS ECOG ≥ 2 |
2.22 |
1.69–2.92 |
< 0.001 |
2.22 |
1.69–2.92 |
< 0.001 |
No MYC rearrangement |
0.82 |
0.53–1.29 |
0.39 |
|
|
|
Stage 3 or 4 |
2.35 |
1.57–3.53 |
< 0.001 |
|
|
|
B symptoms |
1.23 |
0.95–1.59 |
0.12 |
|
|
|
> 1 extra nodal site |
1.24 |
0.95–1.60 |
0.11 |
|
|
|
Marrow involvement |
1.64 |
1.27–2.13 |
< 0.001 |
|
|
|
CNS involvement |
2.02 |
1.52–2.69 |
< 0.001 |
2.02 |
1.52–2.69 |
< 0.001 |
LDH > 3 × ULN |
2.12 |
1.62–2.77 |
< 0.001 |
2.12 |
1.62–2.77 |
< 0.001 |
Hemoglobin < 11.5 g/dL |
1.63 |
1.25–2.12 |
< 0.001 |
|
|
|
Albumin < 3.5 g/dL |
1.55 |
1.19–2.03 |
0.001 |
|
|
|
Figure 1. Percentage of patients according to number of risk factors1,2
*Risk factors: Age ≥ 40; performance status ≥ 2; LDH > 3 × ULN; and CNS involvement.
Table 3. PFS and OS in derivation and validation cohorts1,2
CI, confidence interval; OS, overall survival; PFS, progression-free survival; yr, year. |
||||||
|
Derivation cohort |
Validation cohort |
||||
---|---|---|---|---|---|---|
Outcome/ BL-IPI group |
n (%) |
3-yr estimate, % (95% CI) |
HR (95% CI) |
n (%) |
3-yr estimate, % (95% CI) |
HR (95% CI) |
PFS |
|
|
|
|
|
|
Low |
104 (18) |
92 (84.2–95.8) |
1.0 |
68 (15) |
96 (86.7–98.5) |
1.0 |
Intermediate |
206 (36) |
72 (64.8–77.5) |
4.15 (1.99–8.68) |
159 (35) |
82 (75.2–87.5) |
2.63 (1.02–6.81) |
High |
260 (45) |
53 (46.7–59.2) |
8.83 (4.32–18.03) |
230 (50) |
63 (56.5–69.2) |
6.17 (2.51–15.22) |
OS |
|
|
|
|
|
|
Low |
104 (18) |
96 (89.7–98.5) |
1.0 |
68 (15) |
99 (89.9–99.8) |
1.0 |
Intermediate |
206 (36) |
76 (69.1–81.4) |
7.06 (2.55–19.53) |
159 (35) |
85 (77.9–89.6) |
2.83 (0.99–8.13) |
High |
260 (45) |
59 (52.1–64.7) |
15.12 (5.58–40.99) |
230 (50) |
64 (57.6–70.2) |
7.59 (2.78–20.72) |
The BL-IPI is a novel, simple prognostic index for BL, developed using a real-world US cohort and validated using an international patient population, demonstrating applicability to diverse clinical settings across the world. However, as the study uses retrospective data, the BL-IPI should be interpreted with caution and further validation using clinical trial data would be of value, as well as validation for the endemic BL variant.
References
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