Contributions
Abstract: PB1799
Type: Publication Only
Background
Richter syndrome (RS) represents transformation of chronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL) into more aggressive B-cell lymphoproliferative disorder, most commonly, diffuse large B cell lymphoma (DLBCL). vera rarely classical Hodgkin lymphoma (HL). In some point of disease course, 2-10% of all CLL/SLL population develope RS, usually exhibiting chemoresistance and survival within a year after diagnosis.
Aims
The aim of the study is to evaluate clinical, laboratory and histopathological features of patients with RS at transformation, and their impact on the outcome.
Methods
We processed data from the medical records of 36 CLL and SLL patients with RS diagnosed and treated in four institutions in Serbia from 2003 to 2016: Clinic for Hematology, Clinical Center of Serbia; Clinic for Hematology, Medical Military Academy; Clinical Hospital Center Zemun; and Clinic for Hematology, Clinical Center Kragujevac. In all of RS patients, diagnosis was established after histopathological and immunohistochemistry analysis of biopted tissue (lymph node, bone marrow, Waldeyer’s ring, maxillary sinus, spleen or liver).
Results
In four institutions RS was diagnosed in 36/1250 CLL/SLL patients (2.8%). Median age was 57.5 years (range 41-79). In 16 (44%) patients RS was confirmed in lymph node sample, in 13 (35%) patients in bone marrow, in 4 (11%) patients in Waldeyer´s ring, in 2 (5%) patients in maxillary sinus, in 2 (5%) patients in liver or/and spleen, while in 3 patients in more than one location. Histopathological findings of all patients in transformation were consistent with DLBCL, except one, showing pattern of HL. Prior to the transformation, 26 (72%) patients received chemotherapy (Chlorambucil 6 patients, Fludarabin based regimens 11 patients, CHOP 3 patients, COP/RCOP 4 patients, other modality 3 patients), 4 (11%) of them were on the “watch and wait” strategy, while 6 (17%) patients were diagnosed with RS at presentation, and treated as RS. Median time to transformation was 36 (0-180) months, At the time of transformation median LDH and beta-2 microglobulin levels were significantly higher than on presentation (p=0.035 and p=0.010, respectively). The majority of patients received CHOP (20/36, 55%) and RCHOP (7/36, 19%) as initial therapy for RS. The reminder of patients received other treatment modalities, such as ESHAP, FC, high dose corticosteroids, COP, RCOP and radiotherapy. After excluding 6 patients with SLL from the group, Cox regression analysis showed that high LDH and low Hb levels at the time of transformation are significant predictors of shorter survival after diagnosis of RS (HR=1.001; 95% CI 1.000-1.001; p<0.001 and HR=0.978; 95% CI 0.961-0.995; p=0.011, respectively). Bone marrow as a site of transformation did not reach statistical significance as a predictor of shorter survival after transformation (p=0.087). Median survival after diagnosis of RS was 8 months (range 0-133) (Figure 1).
Conclusion
Incidence of RS in our study is partly coherent with literature data. Levels of LDH and Hb at the time of transformation are significant predictors of outcome of patients with RS. Real number of patients with RS is probably higher, but commonly bad condition of these patients on diagnosis of RS probably influences the decision of a clinician not to indicate biopsy.
Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical
Keyword(s): Transformation, Diffuse large B cell lymphoma, Chronic Lymphocytic Leukemia
Abstract: PB1799
Type: Publication Only
Background
Richter syndrome (RS) represents transformation of chronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL) into more aggressive B-cell lymphoproliferative disorder, most commonly, diffuse large B cell lymphoma (DLBCL). vera rarely classical Hodgkin lymphoma (HL). In some point of disease course, 2-10% of all CLL/SLL population develope RS, usually exhibiting chemoresistance and survival within a year after diagnosis.
Aims
The aim of the study is to evaluate clinical, laboratory and histopathological features of patients with RS at transformation, and their impact on the outcome.
Methods
We processed data from the medical records of 36 CLL and SLL patients with RS diagnosed and treated in four institutions in Serbia from 2003 to 2016: Clinic for Hematology, Clinical Center of Serbia; Clinic for Hematology, Medical Military Academy; Clinical Hospital Center Zemun; and Clinic for Hematology, Clinical Center Kragujevac. In all of RS patients, diagnosis was established after histopathological and immunohistochemistry analysis of biopted tissue (lymph node, bone marrow, Waldeyer’s ring, maxillary sinus, spleen or liver).
Results
In four institutions RS was diagnosed in 36/1250 CLL/SLL patients (2.8%). Median age was 57.5 years (range 41-79). In 16 (44%) patients RS was confirmed in lymph node sample, in 13 (35%) patients in bone marrow, in 4 (11%) patients in Waldeyer´s ring, in 2 (5%) patients in maxillary sinus, in 2 (5%) patients in liver or/and spleen, while in 3 patients in more than one location. Histopathological findings of all patients in transformation were consistent with DLBCL, except one, showing pattern of HL. Prior to the transformation, 26 (72%) patients received chemotherapy (Chlorambucil 6 patients, Fludarabin based regimens 11 patients, CHOP 3 patients, COP/RCOP 4 patients, other modality 3 patients), 4 (11%) of them were on the “watch and wait” strategy, while 6 (17%) patients were diagnosed with RS at presentation, and treated as RS. Median time to transformation was 36 (0-180) months, At the time of transformation median LDH and beta-2 microglobulin levels were significantly higher than on presentation (p=0.035 and p=0.010, respectively). The majority of patients received CHOP (20/36, 55%) and RCHOP (7/36, 19%) as initial therapy for RS. The reminder of patients received other treatment modalities, such as ESHAP, FC, high dose corticosteroids, COP, RCOP and radiotherapy. After excluding 6 patients with SLL from the group, Cox regression analysis showed that high LDH and low Hb levels at the time of transformation are significant predictors of shorter survival after diagnosis of RS (HR=1.001; 95% CI 1.000-1.001; p<0.001 and HR=0.978; 95% CI 0.961-0.995; p=0.011, respectively). Bone marrow as a site of transformation did not reach statistical significance as a predictor of shorter survival after transformation (p=0.087). Median survival after diagnosis of RS was 8 months (range 0-133) (Figure 1).
Conclusion
Incidence of RS in our study is partly coherent with literature data. Levels of LDH and Hb at the time of transformation are significant predictors of outcome of patients with RS. Real number of patients with RS is probably higher, but commonly bad condition of these patients on diagnosis of RS probably influences the decision of a clinician not to indicate biopsy.
Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical
Keyword(s): Transformation, Diffuse large B cell lymphoma, Chronic Lymphocytic Leukemia