Contributions
Abstract: PB1578
Type: Publication Only
Session title: Indolent and mantle-cell non-Hodgkin lymphoma - Clinical
Background
Hairy cell leukemia (HCL) is an uncommon chronic B cell lymphoproliferative disorder that represents approximately 2% of all leukemias and less than 1% of lymphoid neoplasms. The lack of large national and international registries on HCL has limited the ability to identify clear clinico-epidemiological characteristics in this rare disease.
Aims
To evaluate the hairy cell leukemia patients in Egyptian population as regard epidemiological, pathological, immunophenotypic, clinical data as well as treatment modalities and outcome.
Methods
This is a retrospective observational study in which 92 patients diagnosed with HCL were reviewed through the electronic health records between January 1, 2008 and December 31, 2019.
Results
Median age of patients was 50 years (23-81), patients have < 60 years represented 78.3%. Male to female ratio was 5.5:1. The prevalence of hepatitis c virus (HCV) infection was triple that of the general population and appeared to have no effect on overall survival (OS). Most patients (88%) had no comorbidities. The most common presentations were weakness and fatigue then B symptoms, 80% of patients had splenomegaly with a median of 21 cm (massive) and 19% had splenectomies. Hepatomegaly is reported in 40% of cases and uncommonly less than 2% of the cases had abdominal lymphadenopathy. Most of the patients had varying degree of cytopenia varying from pancytopenia (50%), bicytopenia (43.5%), thrombocytopenia (69.3%), leukopenia (53%) and anemia only (6.5%). Hairy cells in peripheral blood samples were observed in 18.5% of cases, dry bone marrow aspirates were in 40% and lymphoid aggregates in 79% of cases. Diffuse infiltrative pattern in bone marrow biopsies was observed in 75% of the patients and the pattern of infiltration posed no effect on overall survival. Regarding the therapeutic options; 61 patients received cladribine, five patients received interferon (INFa), six patients had palliative splenectomies, five patients were subjected to watchful waiting and 15 patients received supportive therapy. Irrespective of the received treatment; complete remission (CR) was achieved in 43.5% of the patients, 8.7% had partial remission (PR), 26.1% had stationary disease, 3.3% had progressive disease, 5.4% had treatment related mortality and 13% had disease related mortality. The presence of comorbidities, Age >60 years, fever, pancytopenia, hemoglobin level < 8 gm/dl and WBCs < 4 k/ml all inversely affected OS (p-value = 0.028, 0.002, 0.008, 0.03, 0.02, 0.02 respectively). After median follow up of 57 months, 68 cases (73.9%) were alive. The OS% of all the cases regardless of the treatment modality was 78% at 1 year, 65% at 5 years and 53% at 10 years. For the patients who received cladribine; 64% had CR and 11.5% had PR with a relapse rate of 19.5%. The OS with cladribine as first line was 91% at 5 years, 83% at 10 years. Treatment with cladribine improved OS in comparison to other treatment modalities (p < 0.0001).
Conclusion
HCL in Egypt is a rare NHL that have different clinicopathological features especially in association with hepatitis C infection that necessitate participation in international registries for HCL e.g. Hairy Cell Leukemia Foundation, to investigate the most optimal treatment strategies for these neoplasms in the real world population.
Keyword(s): Cladribine, Hairy cell leukemia, Hepatitis C virus
Abstract: PB1578
Type: Publication Only
Session title: Indolent and mantle-cell non-Hodgkin lymphoma - Clinical
Background
Hairy cell leukemia (HCL) is an uncommon chronic B cell lymphoproliferative disorder that represents approximately 2% of all leukemias and less than 1% of lymphoid neoplasms. The lack of large national and international registries on HCL has limited the ability to identify clear clinico-epidemiological characteristics in this rare disease.
Aims
To evaluate the hairy cell leukemia patients in Egyptian population as regard epidemiological, pathological, immunophenotypic, clinical data as well as treatment modalities and outcome.
Methods
This is a retrospective observational study in which 92 patients diagnosed with HCL were reviewed through the electronic health records between January 1, 2008 and December 31, 2019.
Results
Median age of patients was 50 years (23-81), patients have < 60 years represented 78.3%. Male to female ratio was 5.5:1. The prevalence of hepatitis c virus (HCV) infection was triple that of the general population and appeared to have no effect on overall survival (OS). Most patients (88%) had no comorbidities. The most common presentations were weakness and fatigue then B symptoms, 80% of patients had splenomegaly with a median of 21 cm (massive) and 19% had splenectomies. Hepatomegaly is reported in 40% of cases and uncommonly less than 2% of the cases had abdominal lymphadenopathy. Most of the patients had varying degree of cytopenia varying from pancytopenia (50%), bicytopenia (43.5%), thrombocytopenia (69.3%), leukopenia (53%) and anemia only (6.5%). Hairy cells in peripheral blood samples were observed in 18.5% of cases, dry bone marrow aspirates were in 40% and lymphoid aggregates in 79% of cases. Diffuse infiltrative pattern in bone marrow biopsies was observed in 75% of the patients and the pattern of infiltration posed no effect on overall survival. Regarding the therapeutic options; 61 patients received cladribine, five patients received interferon (INFa), six patients had palliative splenectomies, five patients were subjected to watchful waiting and 15 patients received supportive therapy. Irrespective of the received treatment; complete remission (CR) was achieved in 43.5% of the patients, 8.7% had partial remission (PR), 26.1% had stationary disease, 3.3% had progressive disease, 5.4% had treatment related mortality and 13% had disease related mortality. The presence of comorbidities, Age >60 years, fever, pancytopenia, hemoglobin level < 8 gm/dl and WBCs < 4 k/ml all inversely affected OS (p-value = 0.028, 0.002, 0.008, 0.03, 0.02, 0.02 respectively). After median follow up of 57 months, 68 cases (73.9%) were alive. The OS% of all the cases regardless of the treatment modality was 78% at 1 year, 65% at 5 years and 53% at 10 years. For the patients who received cladribine; 64% had CR and 11.5% had PR with a relapse rate of 19.5%. The OS with cladribine as first line was 91% at 5 years, 83% at 10 years. Treatment with cladribine improved OS in comparison to other treatment modalities (p < 0.0001).
Conclusion
HCL in Egypt is a rare NHL that have different clinicopathological features especially in association with hepatitis C infection that necessitate participation in international registries for HCL e.g. Hairy Cell Leukemia Foundation, to investigate the most optimal treatment strategies for these neoplasms in the real world population.
Keyword(s): Cladribine, Hairy cell leukemia, Hepatitis C virus