Contributions
Abstract: PB1879
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is associated with non-Hodgkin lymphoma (NHL) and with chronic lymphocytic leukemia (CLL) in 2-3% and 5-10% of cases, resp. The most effective treatment for these diseases is chemotherapy with corticosteroid hormones. However, the presence of AIHA in combination with massive splenomegaly may significantly reduce the efficacy of cytostatics. In such cases, splenectomy becomes a reasonable treatment of choice.
Aims
To conduct a retrospective analysis of immediate and long-term outcomes of splenectomy in patients with CLL and NHL, complicated by AIHA.
Methods
10 patients with NHL and 14 patients with CLL, complicated by AIHA (warm-type) underwent splenectomy.
Results
Splenectomy (SE) proved effective in 12 (85.7 %) patients with CLL and AIHA: a big tumor mass was resected, hemolysis was suppressed, hemoglobin level normalized or increased, and platelet count normalized in patients with Rai IV stage. One patient with Rai III stage died of acute adrenal insufficiency three days following operation. As shown by the evaluation of long-term outcomes of SE, median event-free survival (EFS) reached 12.0 months (duration of follow-up was 1-26 months), and median overall survival (OS) reached 25.5 months (duration of follow-up was 2-256 months); there were four (33.3%) patients with a 2-year survival, one (8.3%) patient with a 5-year survival, and two (16.7%) patients with a 10-year survival. Mean post-splenectomy survival in patients with Rai III and IV stage reached 77.6 and 54.5 months, resp. Eight (57.1%) patients were initiated on chemotherapy following 1-14 months after SE, and one patient has been alive for 256 months after surgery with no supportive therapy but complaining of frequent acure respiratory infections. Eight (57.1%) patients with CLL and AIHA died following 2-32 months after SE. Seven patients died due to progression of underlying disease, whereas the relapse of hemolysis was observed just in a single patient. One patient died following 26 months after operation of OPSI-syndrome with a clinical manifestation of severe septic shock. SE was effective in 100 % patients with NHL and AIHA: hemolysis was suppressed, hemoglobin level increased; in patients with leukopenia, their leukocyte count normalized, and their abdominal discomfort due to an increased spleen weight disappeared. During the first-year postoperatively, just one patient required cytostatic treatment. As shown by the evaluation of long-term outcomes of SE in patients with NHL and AIHA, EFS reached 29.0 months [12.0-49.0 months], and OS reached 31.0 months [18.1-52.2 months] with a 3-year survival in 45% patients and a 5-year survival in 22% patients. The shortest postoperative survival (3 months) was observed in a patient with nodal marginal zone lymphoma , and the best outcome was achieved in splenic marginal zone lymphoma (SMZL): the patient has been living for 206 months after SE in the condition of a complete clinical remission without any treatment. One patient with SMZL died following 9 months after SE of OPSI-syndrome. Patients with diffuse large B-cell lymphoma (DLBCL, 6 cases) showed a low median of overall survival (26 months), despite of promising immediate outcomes of SE.
Conclusion
Splenectomy remains to be a leading treatment of choice in the management of chronic lymphoid neoplasias, complicated by AIHA and massive splenomegaly. Long-term outcomes are worse in patients with CLL and Rai IV stage of AIHA. In patients with NHL, complicated by AIHA, the efficacy of operation depends on the type of lymphoma.
Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical
Keyword(s): AIHA (see autoimmune hemolytic anemia), Chronic Lymphocytic Leukemia, Non-Hodgkin's lymphoma, Splenectomy
Abstract: PB1879
Type: Publication Only
Background
Autoimmune hemolytic anemia (AIHA) is associated with non-Hodgkin lymphoma (NHL) and with chronic lymphocytic leukemia (CLL) in 2-3% and 5-10% of cases, resp. The most effective treatment for these diseases is chemotherapy with corticosteroid hormones. However, the presence of AIHA in combination with massive splenomegaly may significantly reduce the efficacy of cytostatics. In such cases, splenectomy becomes a reasonable treatment of choice.
Aims
To conduct a retrospective analysis of immediate and long-term outcomes of splenectomy in patients with CLL and NHL, complicated by AIHA.
Methods
10 patients with NHL and 14 patients with CLL, complicated by AIHA (warm-type) underwent splenectomy.
Results
Splenectomy (SE) proved effective in 12 (85.7 %) patients with CLL and AIHA: a big tumor mass was resected, hemolysis was suppressed, hemoglobin level normalized or increased, and platelet count normalized in patients with Rai IV stage. One patient with Rai III stage died of acute adrenal insufficiency three days following operation. As shown by the evaluation of long-term outcomes of SE, median event-free survival (EFS) reached 12.0 months (duration of follow-up was 1-26 months), and median overall survival (OS) reached 25.5 months (duration of follow-up was 2-256 months); there were four (33.3%) patients with a 2-year survival, one (8.3%) patient with a 5-year survival, and two (16.7%) patients with a 10-year survival. Mean post-splenectomy survival in patients with Rai III and IV stage reached 77.6 and 54.5 months, resp. Eight (57.1%) patients were initiated on chemotherapy following 1-14 months after SE, and one patient has been alive for 256 months after surgery with no supportive therapy but complaining of frequent acure respiratory infections. Eight (57.1%) patients with CLL and AIHA died following 2-32 months after SE. Seven patients died due to progression of underlying disease, whereas the relapse of hemolysis was observed just in a single patient. One patient died following 26 months after operation of OPSI-syndrome with a clinical manifestation of severe septic shock. SE was effective in 100 % patients with NHL and AIHA: hemolysis was suppressed, hemoglobin level increased; in patients with leukopenia, their leukocyte count normalized, and their abdominal discomfort due to an increased spleen weight disappeared. During the first-year postoperatively, just one patient required cytostatic treatment. As shown by the evaluation of long-term outcomes of SE in patients with NHL and AIHA, EFS reached 29.0 months [12.0-49.0 months], and OS reached 31.0 months [18.1-52.2 months] with a 3-year survival in 45% patients and a 5-year survival in 22% patients. The shortest postoperative survival (3 months) was observed in a patient with nodal marginal zone lymphoma , and the best outcome was achieved in splenic marginal zone lymphoma (SMZL): the patient has been living for 206 months after SE in the condition of a complete clinical remission without any treatment. One patient with SMZL died following 9 months after SE of OPSI-syndrome. Patients with diffuse large B-cell lymphoma (DLBCL, 6 cases) showed a low median of overall survival (26 months), despite of promising immediate outcomes of SE.
Conclusion
Splenectomy remains to be a leading treatment of choice in the management of chronic lymphoid neoplasias, complicated by AIHA and massive splenomegaly. Long-term outcomes are worse in patients with CLL and Rai IV stage of AIHA. In patients with NHL, complicated by AIHA, the efficacy of operation depends on the type of lymphoma.
Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical
Keyword(s): AIHA (see autoimmune hemolytic anemia), Chronic Lymphocytic Leukemia, Non-Hodgkin's lymphoma, Splenectomy