Hematology
Contributions
Type: Publication Only
Background
Anaplastic large-cell lymphoma (ALCL) accounts for approximately 3% of non-Hodgkin´s lymphomas (NHL). It is a peripheral T-cell lymphoma characterized by expression of CD30. Currently the WHO recognized two kinds of ALCL based on anaplastic lymphoma kinase (ALK) expression. In previous studies, ALK positive ALCL was associated with younger patients and a better prognosis. The International Peripheral T-Cell Lymphoma Project Study reported a 5 year overall survival of 70% and 49% in ALK positive and ALK negative ALCL respectively.
Aims
To study the clinical characteristics and outcomes of patients with systemic ALK-positive and negative ALCL in our population.
Methods
We retrospectively analyzed 39 consecutive cases of ALCL referred and treated at the Instituto Nacional de Cancerologia, México
Results
A total of 1951 charts of NHL patients from 2008 to 2013 were reviewed. ALCL was confirmed in 49 (2.5%). Ten patients had primary cutaneous anaplastic lymphoma, thus 39 patients were analyzed.
Twelve patients were ALK-positive (33%) and 27 (77%) Alk-negative. Median aged was 26 (17-48) vs 47 years (22-76) respectively (p=0.40). Clinical characteristics are shown in table 1. There were not significance differences between groups.
Seventeen patients achieved a CR (44%), 3 (8%) partial responses, 5(13%) had refractory /relapsed disease and 14 patients (36%) could not be evaluated for response, 12 died before treatment completion. The median follow up was 39 months with an overall survival (OS) of 47%, median EFS was not reached. The multivariate analysis for OS in all group identified B2-microglobulin ≥2.7 mg/dL as the only prognosis factor (p=0.01). No difference in overall response, overall survival or event-free survival was found in relation with ALK status.
| ALCL ALK negative n(%) | ALCL ALK positive n(%) | p |
Range, y |
7 (29) 17 (71) |
8 (67) 4 (33) |
0.71 |
Performance status 0-1 | 12 (52) | 7 (70) | 0.45 |
Ann Arbor stage III-IV | 19 (83) | 9 (75) | 0.67 |
B symptoms | 19 (83) | 11 (92) | 0.64 |
Bulky disease | 9 (39) | 6 (50) | 0.53 |
Extranodal disease | 11 (48) | 5 (42) | 0.72 |
Albumin ≤3.5 mg/dL | 15 (65) | 8 (89) | 0.38 |
DHL elevated ≥213 UI/L | 13 (56) | 6 (67) | 0.70 |
Hemoglobin ≤10 g/dL | 4 (17) | 3 (33) | 0.37 |
Lymphocyte ≤1200 cel/mm3 | 15 (65) | 7 (78) | 0.68 |
β2microglobulin ≥2.7 mg/dL | 11 (50) | 7 (78) | 0.23 |
IPI score |
9 (39) 14 (61) |
5 (50) 5 (50) | 0.56 |
Treatment |
13 (72) - 5 (28) |
6 (54) 4 (36) 1 (9) |
0.039 |
Radiotherapy | 6 (25) | 7 (58) | 0.050 |
Overall response (CR+PR) | 10 (71.4) | 8 (88.9) | 0.61 |
Relapsed | 7 (29) | 2 (17) | 0.68 |
3 years OS | 49.5% | 70% | 0.38 |
10 months EFS | 50% | 80% | 0.15 |
Summary
Interestingly, we found no difference in outcome between Alk positive and negative patients. There are controversial reports about the prognostic significance of ALK status in ALCL. Gascoyne and cols. reported a 5 years OS of 93% vs 37%. However a GELA study did not find such a difference, while an elevated B2-microglobulin was a poor prognostic factor (OS 84% vs 22%). We also found B2-microglobulin to be a significant prognosis factor.
Keyword(s): Anaplastic large cell lymphoma, Anaplastic lymphoma kinase, Clinical outcome, Prognosis
Type: Publication Only
Background
Anaplastic large-cell lymphoma (ALCL) accounts for approximately 3% of non-Hodgkin´s lymphomas (NHL). It is a peripheral T-cell lymphoma characterized by expression of CD30. Currently the WHO recognized two kinds of ALCL based on anaplastic lymphoma kinase (ALK) expression. In previous studies, ALK positive ALCL was associated with younger patients and a better prognosis. The International Peripheral T-Cell Lymphoma Project Study reported a 5 year overall survival of 70% and 49% in ALK positive and ALK negative ALCL respectively.
Aims
To study the clinical characteristics and outcomes of patients with systemic ALK-positive and negative ALCL in our population.
Methods
We retrospectively analyzed 39 consecutive cases of ALCL referred and treated at the Instituto Nacional de Cancerologia, México
Results
A total of 1951 charts of NHL patients from 2008 to 2013 were reviewed. ALCL was confirmed in 49 (2.5%). Ten patients had primary cutaneous anaplastic lymphoma, thus 39 patients were analyzed.
Twelve patients were ALK-positive (33%) and 27 (77%) Alk-negative. Median aged was 26 (17-48) vs 47 years (22-76) respectively (p=0.40). Clinical characteristics are shown in table 1. There were not significance differences between groups.
Seventeen patients achieved a CR (44%), 3 (8%) partial responses, 5(13%) had refractory /relapsed disease and 14 patients (36%) could not be evaluated for response, 12 died before treatment completion. The median follow up was 39 months with an overall survival (OS) of 47%, median EFS was not reached. The multivariate analysis for OS in all group identified B2-microglobulin ≥2.7 mg/dL as the only prognosis factor (p=0.01). No difference in overall response, overall survival or event-free survival was found in relation with ALK status.
| ALCL ALK negative n(%) | ALCL ALK positive n(%) | p |
Range, y |
7 (29) 17 (71) |
8 (67) 4 (33) |
0.71 |
Performance status 0-1 | 12 (52) | 7 (70) | 0.45 |
Ann Arbor stage III-IV | 19 (83) | 9 (75) | 0.67 |
B symptoms | 19 (83) | 11 (92) | 0.64 |
Bulky disease | 9 (39) | 6 (50) | 0.53 |
Extranodal disease | 11 (48) | 5 (42) | 0.72 |
Albumin ≤3.5 mg/dL | 15 (65) | 8 (89) | 0.38 |
DHL elevated ≥213 UI/L | 13 (56) | 6 (67) | 0.70 |
Hemoglobin ≤10 g/dL | 4 (17) | 3 (33) | 0.37 |
Lymphocyte ≤1200 cel/mm3 | 15 (65) | 7 (78) | 0.68 |
β2microglobulin ≥2.7 mg/dL | 11 (50) | 7 (78) | 0.23 |
IPI score |
9 (39) 14 (61) |
5 (50) 5 (50) | 0.56 |
Treatment |
13 (72) - 5 (28) |
6 (54) 4 (36) 1 (9) |
0.039 |
Radiotherapy | 6 (25) | 7 (58) | 0.050 |
Overall response (CR+PR) | 10 (71.4) | 8 (88.9) | 0.61 |
Relapsed | 7 (29) | 2 (17) | 0.68 |
3 years OS | 49.5% | 70% | 0.38 |
10 months EFS | 50% | 80% | 0.15 |
Summary
Interestingly, we found no difference in outcome between Alk positive and negative patients. There are controversial reports about the prognostic significance of ALK status in ALCL. Gascoyne and cols. reported a 5 years OS of 93% vs 37%. However a GELA study did not find such a difference, while an elevated B2-microglobulin was a poor prognostic factor (OS 84% vs 22%). We also found B2-microglobulin to be a significant prognosis factor.
Keyword(s): Anaplastic large cell lymphoma, Anaplastic lymphoma kinase, Clinical outcome, Prognosis