Contributions
Abstract: PB2036
Type: Publication Only
Background
Hairy cell leukaemia (HCL) is a rare condition characterized by pancytopenia and markedly susceptibility to infections. This lymphoproliferative disease presents high rates of response and survival. Nevertheless, treatment may not be curative, and there may be a need for re-treatment at the time of relapse. Thus, the identification of patients at risk of treatment failure remains an important question.
Aims
Characterization of the population with HCL diagnosed in a tertiary hospital. To identify in this group of patients factors predictors of survival.
Methods
Retrospective analysis of patients diagnosed with HCL at our institution between January, 1988 and December, 2017.
Results
Were included 51 patients; male gender 80.4% (n=41); median age at diagnosis 57 years (35-83). Had comorbidities 56.9% (n=29). Splenomegaly was present in 68% (n=34) and B symptoms in 33.3% (n=17). Had haemoglobin <11 g/dL 39.2% (n=20), platelets <100 G/L 58.8% (n=30), neutrophils <1G/L 58% (n=29). Median follow-up was 78.8 months (0.6-290.8). First line treatment was cladribine in 77.6% (n=38), pentostatin in 4% (n=2) and IFNα in 18.4% (n=9), remission (CR+PR) was obtained in 87.8% (n=43). Use of cladribine was associated with greater achievement of complete response (79.0 vs 22.2%, OR 0.28; p=0.004). Were re-treated 22 patients – 72.7% of them with purine analogues (PA) –, 81.8% obtaining remission (CR+PR). Median overall survival (OS) was 232.8 months (232.7 with cladribine, 287.3 with IFNα). Median progression free survival (PFS) was 71.2 months (102.7 with cladribine, 67 with IFNα). Although there were no differences in OS between cladribine and IFNα (p=0.6892), in PFS there were significative differences (p=0.045). Seven patients presented second neoplasia, all of them treated with PA. Of the 51 patients, 27.5% died, 28.6% of them of infection. OS was inferior in age>65 years (p<0.001), ECOG >0 (p=0.0053) and serious infection at diagnosis (p=0.012). After multivariate analysis, age>65 years (HR 41.95; p=0.002) and ECOG>0 (HR 6.19; p=0.008) were independent predictors of lower survival at diagnosis.
Conclusion
Although the PA contributed to increase of complete response rate and PFS, there is still a need to optimize therapeutic strategies in order to increase OS, as decreasing adverse events and relapse / refractoriness in patients with HCL. Factors such as age at diagnosis >65 years and ECOG >0 are predictors of lower survival, however, more robust studies are needed to validate and identify other potential prognostic factors.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Hairy cell leukemia
Abstract: PB2036
Type: Publication Only
Background
Hairy cell leukaemia (HCL) is a rare condition characterized by pancytopenia and markedly susceptibility to infections. This lymphoproliferative disease presents high rates of response and survival. Nevertheless, treatment may not be curative, and there may be a need for re-treatment at the time of relapse. Thus, the identification of patients at risk of treatment failure remains an important question.
Aims
Characterization of the population with HCL diagnosed in a tertiary hospital. To identify in this group of patients factors predictors of survival.
Methods
Retrospective analysis of patients diagnosed with HCL at our institution between January, 1988 and December, 2017.
Results
Were included 51 patients; male gender 80.4% (n=41); median age at diagnosis 57 years (35-83). Had comorbidities 56.9% (n=29). Splenomegaly was present in 68% (n=34) and B symptoms in 33.3% (n=17). Had haemoglobin <11 g/dL 39.2% (n=20), platelets <100 G/L 58.8% (n=30), neutrophils <1G/L 58% (n=29). Median follow-up was 78.8 months (0.6-290.8). First line treatment was cladribine in 77.6% (n=38), pentostatin in 4% (n=2) and IFNα in 18.4% (n=9), remission (CR+PR) was obtained in 87.8% (n=43). Use of cladribine was associated with greater achievement of complete response (79.0 vs 22.2%, OR 0.28; p=0.004). Were re-treated 22 patients – 72.7% of them with purine analogues (PA) –, 81.8% obtaining remission (CR+PR). Median overall survival (OS) was 232.8 months (232.7 with cladribine, 287.3 with IFNα). Median progression free survival (PFS) was 71.2 months (102.7 with cladribine, 67 with IFNα). Although there were no differences in OS between cladribine and IFNα (p=0.6892), in PFS there were significative differences (p=0.045). Seven patients presented second neoplasia, all of them treated with PA. Of the 51 patients, 27.5% died, 28.6% of them of infection. OS was inferior in age>65 years (p<0.001), ECOG >0 (p=0.0053) and serious infection at diagnosis (p=0.012). After multivariate analysis, age>65 years (HR 41.95; p=0.002) and ECOG>0 (HR 6.19; p=0.008) were independent predictors of lower survival at diagnosis.
Conclusion
Although the PA contributed to increase of complete response rate and PFS, there is still a need to optimize therapeutic strategies in order to increase OS, as decreasing adverse events and relapse / refractoriness in patients with HCL. Factors such as age at diagnosis >65 years and ECOG >0 are predictors of lower survival, however, more robust studies are needed to validate and identify other potential prognostic factors.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Hairy cell leukemia