Contributions
Abstract: EP705
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
The incidence of autoimmune hemolytic anemia (AIHA) is estimated between 1 to 3/100,000 person-years and is associated to another disease in half of the cases, mostly hematological malignancies. The accurate incidence of lymphoid malignancy (LM)-related AIHA remains elusive as previous data have been obtained from cohorts from tertiary centers, with possible selection bias.
Aims
Determination of the frequency, clinical characteristics, and evolution of LM-related AIHA based on prospective data collected in the register of hematological malignancies of Côte d’Or (RHEMCO), a population-based register recording all incident hematological malignancies occurring in a restricted geographical area of 532,886 inhabitants.
Methods
Patients included in RHEMCO between 01/01/1995 and 31/12/2015 were considered to calculate incidences. The medical records of AIHA patients referred to our tertiary hospital during the same periods were also considered for the clinical description and the evolution of LM-related AIHA. AIHA was defined as regenerative (reticulocytes >120 G/L) anemia (hemoglobin <11 g/dL) with hemolysis (haptoglobin <0.3 g/L, elevated LDH, free bilirubin >14 µmol/L) and a positive direct antiglobulin test. Response was defined as hemoglobin >10 g/dL.
Results
Based on RHEMCO, the frequency of AIHA during LM was 0.77% (n=27/3499): 1.10% for chronic lymphoid leukemia (CLL), 0.67% for B cell lymphoma, 0.87% for T cell lymphoma and 0.36% for Hodgkin lymphoma (HL). The frequency of Evans’ syndrome (ES) was 0.29% (n=10/3499): 0.44% for CLL, 0.24% for B cell lymphoma and 0.43% for T cell lymphoma.
From RHEMCO and medical records, 56 patients with secondary AIHA were identified among which 17 had ES. The median age at diagnosis was 74 [interquartile range (IQR): 54-92] years and 54% were male. For ES, the median age was 62 [IQR: 29-85] years and 76% were male. AHAI was associated with B cell lymphoma in 54%, CLL in 30.5%, T cell lymphoma in 13% and HL in 2.5%. ES was also mostly associated with low grade B cell lymphoma (47%) and CLL (41%). Anemia occurred after the diagnosis of LM in 38% of the cases with a median delay of 3 [IQR:0.5-18.4] years and 65% for ES, with a median delay of 5.1 [IQR:0.3-10.1] years. LM and AIHA were diagnosed simultaneously (≤ 3 months) in 49% of the cases for isolated AIHA and 29% for ES. Splenomegaly was observed in 63% of the cases. At AIHA diagnosis, the median hemoglobin level was 7.5 [IQR:3.5-12.3] g/dL. Warm antibodies represented 87% of the cases. Monoclonal gammopathy was detected in 47% of the cases and a circulating lymphocyte clone in 89%.
Patients with LM-related AIHA were treated in 97% of the cases, with medications dedicated to AIHA in 90% of the cases and specifically for LM in 67%. AIHA responded to steroids in 53% (n=17/32), to rituximab in 82% (n=14/17) and to splenectomy in 43% (n=3/7). After a median follow-up of 5.6 years, AIHA was considered in remission in 56%.
Conclusion
LM-related AIHA is rare, with an incidence of 0.063/100,000 person-years for CLL, 0.081/100,000 for B cell lymphoma and 0.01/100,000 for T cell lymphoma and HL. These incidences are close to the ones observed for LM-related ITP although ITP is considered 3 times more frequent than AIHA, highlighting the frequent secondary nature of AIHA. The diagnosis of AHAI was made simultaneously or after LM in almost 90% of the cases. Rituximab seems to be a treatment of choice in LM-related AIHA as shown by a response rate close to the one of primary AIHA.
Keyword(s): Autoimmune hemolytic anemia (AIHA), Lymphoid malignancy
Abstract: EP705
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
The incidence of autoimmune hemolytic anemia (AIHA) is estimated between 1 to 3/100,000 person-years and is associated to another disease in half of the cases, mostly hematological malignancies. The accurate incidence of lymphoid malignancy (LM)-related AIHA remains elusive as previous data have been obtained from cohorts from tertiary centers, with possible selection bias.
Aims
Determination of the frequency, clinical characteristics, and evolution of LM-related AIHA based on prospective data collected in the register of hematological malignancies of Côte d’Or (RHEMCO), a population-based register recording all incident hematological malignancies occurring in a restricted geographical area of 532,886 inhabitants.
Methods
Patients included in RHEMCO between 01/01/1995 and 31/12/2015 were considered to calculate incidences. The medical records of AIHA patients referred to our tertiary hospital during the same periods were also considered for the clinical description and the evolution of LM-related AIHA. AIHA was defined as regenerative (reticulocytes >120 G/L) anemia (hemoglobin <11 g/dL) with hemolysis (haptoglobin <0.3 g/L, elevated LDH, free bilirubin >14 µmol/L) and a positive direct antiglobulin test. Response was defined as hemoglobin >10 g/dL.
Results
Based on RHEMCO, the frequency of AIHA during LM was 0.77% (n=27/3499): 1.10% for chronic lymphoid leukemia (CLL), 0.67% for B cell lymphoma, 0.87% for T cell lymphoma and 0.36% for Hodgkin lymphoma (HL). The frequency of Evans’ syndrome (ES) was 0.29% (n=10/3499): 0.44% for CLL, 0.24% for B cell lymphoma and 0.43% for T cell lymphoma.
From RHEMCO and medical records, 56 patients with secondary AIHA were identified among which 17 had ES. The median age at diagnosis was 74 [interquartile range (IQR): 54-92] years and 54% were male. For ES, the median age was 62 [IQR: 29-85] years and 76% were male. AHAI was associated with B cell lymphoma in 54%, CLL in 30.5%, T cell lymphoma in 13% and HL in 2.5%. ES was also mostly associated with low grade B cell lymphoma (47%) and CLL (41%). Anemia occurred after the diagnosis of LM in 38% of the cases with a median delay of 3 [IQR:0.5-18.4] years and 65% for ES, with a median delay of 5.1 [IQR:0.3-10.1] years. LM and AIHA were diagnosed simultaneously (≤ 3 months) in 49% of the cases for isolated AIHA and 29% for ES. Splenomegaly was observed in 63% of the cases. At AIHA diagnosis, the median hemoglobin level was 7.5 [IQR:3.5-12.3] g/dL. Warm antibodies represented 87% of the cases. Monoclonal gammopathy was detected in 47% of the cases and a circulating lymphocyte clone in 89%.
Patients with LM-related AIHA were treated in 97% of the cases, with medications dedicated to AIHA in 90% of the cases and specifically for LM in 67%. AIHA responded to steroids in 53% (n=17/32), to rituximab in 82% (n=14/17) and to splenectomy in 43% (n=3/7). After a median follow-up of 5.6 years, AIHA was considered in remission in 56%.
Conclusion
LM-related AIHA is rare, with an incidence of 0.063/100,000 person-years for CLL, 0.081/100,000 for B cell lymphoma and 0.01/100,000 for T cell lymphoma and HL. These incidences are close to the ones observed for LM-related ITP although ITP is considered 3 times more frequent than AIHA, highlighting the frequent secondary nature of AIHA. The diagnosis of AHAI was made simultaneously or after LM in almost 90% of the cases. Rituximab seems to be a treatment of choice in LM-related AIHA as shown by a response rate close to the one of primary AIHA.
Keyword(s): Autoimmune hemolytic anemia (AIHA), Lymphoid malignancy