Contributions
Abstract: PB2332
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent B-cell lymphoma frequently associated with monoclonal gammopathy and autoimmune disorders. Therefore, whereas it is well known that the prevalence of autoimmune hemolytic anemia (AIHA) is highest in the more advanced stages of the disease and may depend on the type of treatment administered.
Aims
The ame of this study was to investigate association between autoimmune hemolytic anemia (AIHA) and IgVH genes status.
Methods
We searched in our database of 118 SMZL patients consecutively referred from 2005 to 2017. We identified 9 SMZL patients (7,6%) who developed overt AIHA. All patients met the SMZL WHO diagnostic criteria. AIHA was defined with standard criteria: a fall in hemoglobin level of at least 2 g/dL, associated with a positive direct Coombs test and/or increased reticulocyte count, and a rise in indirect bilirubin with no other causes of anemia identified. IGHV mutational status was determined by Sanger sequencing. Cases with 98% or higher IGHV gene homology to germinal sequences were considered unmutated. We performed a case-control study comparing the 9 patients with SMZL and AIHA with 10 SMZL patients without AIHA.
Results
A higher prevalence of unmutated IgVH was found in patients with AIHA (7 of 9, 77,8%). In comparison only 1 of 10 (10%) controls showed unmutated IgVH, as expected in an unselected cohort. The Pirson criteria significant in cases with unmutated IgVH in SMZL with AIHA (p<0,05). No significant difference was observed in VH family distribution between the two groups. There was a tendency towards more common tumor progression in patients with unmutated IgVH genes with AIHA than in those with mutated ones without AIHA. In all cases SMZL with AIHA were present monoclonal secretion of paraprotein.
Conclusion
Our data show that AIHA is associated with unmutated status in SMZL patients and that patients with early onset of AIHA have a shorter survival. Larger prospective cohorts are needed in order to verify this observation, and to tell us whether the association is directly causal or whether it reflects an increased tumor bulk at the time of AIHA development.
Session topic: 19. Non-Hodgkin lymphoma Biology & Translational Research
Keyword(s): Autoimmune hemolytic anemia (AIHA), Splenic marginal zone lymphoma, VH gene
Abstract: PB2332
Type: Publication Only
Background
Splenic marginal zone lymphoma (SMZL) is an indolent B-cell lymphoma frequently associated with monoclonal gammopathy and autoimmune disorders. Therefore, whereas it is well known that the prevalence of autoimmune hemolytic anemia (AIHA) is highest in the more advanced stages of the disease and may depend on the type of treatment administered.
Aims
The ame of this study was to investigate association between autoimmune hemolytic anemia (AIHA) and IgVH genes status.
Methods
We searched in our database of 118 SMZL patients consecutively referred from 2005 to 2017. We identified 9 SMZL patients (7,6%) who developed overt AIHA. All patients met the SMZL WHO diagnostic criteria. AIHA was defined with standard criteria: a fall in hemoglobin level of at least 2 g/dL, associated with a positive direct Coombs test and/or increased reticulocyte count, and a rise in indirect bilirubin with no other causes of anemia identified. IGHV mutational status was determined by Sanger sequencing. Cases with 98% or higher IGHV gene homology to germinal sequences were considered unmutated. We performed a case-control study comparing the 9 patients with SMZL and AIHA with 10 SMZL patients without AIHA.
Results
A higher prevalence of unmutated IgVH was found in patients with AIHA (7 of 9, 77,8%). In comparison only 1 of 10 (10%) controls showed unmutated IgVH, as expected in an unselected cohort. The Pirson criteria significant in cases with unmutated IgVH in SMZL with AIHA (p<0,05). No significant difference was observed in VH family distribution between the two groups. There was a tendency towards more common tumor progression in patients with unmutated IgVH genes with AIHA than in those with mutated ones without AIHA. In all cases SMZL with AIHA were present monoclonal secretion of paraprotein.
Conclusion
Our data show that AIHA is associated with unmutated status in SMZL patients and that patients with early onset of AIHA have a shorter survival. Larger prospective cohorts are needed in order to verify this observation, and to tell us whether the association is directly causal or whether it reflects an increased tumor bulk at the time of AIHA development.
Session topic: 19. Non-Hodgkin lymphoma Biology & Translational Research
Keyword(s): Autoimmune hemolytic anemia (AIHA), Splenic marginal zone lymphoma, VH gene