Contributions
Abstract: EP714
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
Warm autoimmune hemolytic anemia (WAIHA) is a relatively rare hematologic disorder resulting from autoantibody directed against self-erythrocytes. There has been few studies about warm AIHA because of its low incidence.
Aims
We aimed to analyze 20-years’ experience of WAIHA patients at a single referral center in Sousse. Clinical data, survival outcome of patients who were diagnosed with WAIHA were retrospectively analyzed.
Methods
This is a descriptive retrospective study. Data belonging to WAIHA in adult patients admitted in our department of hematology between January 2001 and December 2020 were analyzed.
Results
One hundred and fifteen patients (58.3% women) were included, the mean age at the time of wAIHA onset was 54 years (range 16-98), the mean hemoglobin level was 6.2 g/dL (range 2.1-10.9 g/dL). All the patients were direct antiglobulin test (DAT) positive (80 (69.6%) patients were IgG, 34 (29.6%) were IgG plus C3d, and only one was IgA DAT positive). wAIHA was considered “primary” for 53 patients (46%) and was associated with an underlying disorder in 62 (54%), including mainly lymphoproliferative diseases (31 patients) and connective tissue disease (25 patients). All patients received steroids at therapeutic dosages (corticosteroid 1 mg/Kg) as first line treatment (+/-chemotherapy for hematologic malignancy)). Hundred and one of them showed : complete response (68/59.1%) and partial response (33/28.7%). Thirty seven patients (32.2%) relapsed at least once and was retreated with corticosteroid in 15 patients because of anemic symptoms, or received cyclophosphamide in 12 patients, rituximab in only 4 patients and splenectomy in 6 patients. Twenty nine patients presented complication (11 cases of several infections, 3 cases of osteoporosis, 3 cases of thrombotic events, 9 cases of corticosteroid-induced diabetes, 3 cases of renal failure). After a median of follow-up of 44 months (1-188), 86 patients were in remission (eight in complete response, 60 in complete remission, 18 in partial remission), 11 patients were in relapse or refractory to a treatment and eighteen patients died (3 during poorly tolerated anemia, 4 due to infections, 1 related to metabolic acidosis for multiple myeloma, 1 ischemic stroke, and nine following a progression of hematologic malignancies or cancer).
Conclusion
This study indicates that secondary WAIHA are predominant. Oral corticosteroid is effective as first line therapy for warm AIHA, but several complication may arise. Despite a proven efficacy of rituximab as corticosteroid-sparing strategy, it is still little used as a second line therapy in our department for this immune disorders.
Keyword(s): Hemolytic anemia
Abstract: EP714
Type: E-Poster Presentation
Session title: Enzymopathies, membranopathies and other anemias
Background
Warm autoimmune hemolytic anemia (WAIHA) is a relatively rare hematologic disorder resulting from autoantibody directed against self-erythrocytes. There has been few studies about warm AIHA because of its low incidence.
Aims
We aimed to analyze 20-years’ experience of WAIHA patients at a single referral center in Sousse. Clinical data, survival outcome of patients who were diagnosed with WAIHA were retrospectively analyzed.
Methods
This is a descriptive retrospective study. Data belonging to WAIHA in adult patients admitted in our department of hematology between January 2001 and December 2020 were analyzed.
Results
One hundred and fifteen patients (58.3% women) were included, the mean age at the time of wAIHA onset was 54 years (range 16-98), the mean hemoglobin level was 6.2 g/dL (range 2.1-10.9 g/dL). All the patients were direct antiglobulin test (DAT) positive (80 (69.6%) patients were IgG, 34 (29.6%) were IgG plus C3d, and only one was IgA DAT positive). wAIHA was considered “primary” for 53 patients (46%) and was associated with an underlying disorder in 62 (54%), including mainly lymphoproliferative diseases (31 patients) and connective tissue disease (25 patients). All patients received steroids at therapeutic dosages (corticosteroid 1 mg/Kg) as first line treatment (+/-chemotherapy for hematologic malignancy)). Hundred and one of them showed : complete response (68/59.1%) and partial response (33/28.7%). Thirty seven patients (32.2%) relapsed at least once and was retreated with corticosteroid in 15 patients because of anemic symptoms, or received cyclophosphamide in 12 patients, rituximab in only 4 patients and splenectomy in 6 patients. Twenty nine patients presented complication (11 cases of several infections, 3 cases of osteoporosis, 3 cases of thrombotic events, 9 cases of corticosteroid-induced diabetes, 3 cases of renal failure). After a median of follow-up of 44 months (1-188), 86 patients were in remission (eight in complete response, 60 in complete remission, 18 in partial remission), 11 patients were in relapse or refractory to a treatment and eighteen patients died (3 during poorly tolerated anemia, 4 due to infections, 1 related to metabolic acidosis for multiple myeloma, 1 ischemic stroke, and nine following a progression of hematologic malignancies or cancer).
Conclusion
This study indicates that secondary WAIHA are predominant. Oral corticosteroid is effective as first line therapy for warm AIHA, but several complication may arise. Despite a proven efficacy of rituximab as corticosteroid-sparing strategy, it is still little used as a second line therapy in our department for this immune disorders.
Keyword(s): Hemolytic anemia