Contributions
Abstract: PB2038
Type: Publication Only
Background
Hairy cell leukemia (HCL) is a rare indolent disease which is curable in most cases. However, a small number of patients is still expected to relapse, or die, mainly due to infective complications.
Aims
The aim of the study is to analyze the course of the disease in a population of uniformly treated patients and identify early and late adverse events during a 20-year follow-up.
Methods
We retrospectively reviewed the charts and collected clinical and laboratory data of 37 patients diagnosed with HCL from October 1997 through May 2017. They were all treated with one course of Cladribine as first line therapy at our Hematology Department. Particularly, we focused on infective events, allergy, secondary neoplasms and relapse during the follow-up.
Results
Most patients sought medical evaluation because of constitutional symptoms and emerging abdominal discomfort. At disease onset, two-third of patients presented with white blood cells count lower than 4x10^9/L and platelets count lower than 100 x10^9/L; massive splenomegaly was detected in 50% of patients. The median time to treatment was 1.4 months (range 0.2-20.6 months): cladribine were administered as a 7-day continue infusion in 89% of patients. The overall response rate was 97%, with 92% complete and 5% partial remissions. During neutropenia, 16 out of 23 patients (69.5%) experienced at least one infectious episode: one patients died, probably due to invasive aspergillosis, 2.7 months after treatment with no sign of hematologic recovery. Median time to recovery of neutropenia was 23 days (range: 5-720 days). Eight patients (22%) reported a diffuse cutaneous allergic reaction: all recovered after few days of antihistamine and steroid administration. Seven patients (19%) experienced a relapse, after a median time of 51.2 months (range: 17.3-146.1 months): 4 were retreated with Cladribine, 1 with Cladribine and Rituximab, 1 with Interferon α and 1 refused treatment. The overall response rate was 57.1% (4 patients, 2 complete and 2 partial responses); 1 patient died two weeks after treatment due to sepsis and 1 was treated with Pentostatin after failure of Interferon α. This patient experienced another relapse 2 years later, and died of septic shock 14 days after starting a BRAF inhibitor. After a median follow-up of 50.6 months (range: 3-219.5 months), overall mortality was 13%: 3 patients died from infection during neutropenia related to chemotherapy, and 2 patients died of causes unrelated to treatment (1 following a myocardial infarction and 1 from senescence). Overall survival and disease-free survival at 20 years were 78% and 81% respectively. Incidence of secondary neoplasms was 2.7%: one patient developed breast cancer 5 years after HCL onset. Age, white blood cells count, bone marrow fibrosis, splenomegaly, infections, allergy, duration of neutropenia and time to best response did not show any statistical significance for mortality at univariate analysis.
Conclusion
Due to the rarity of disease, multicentric survey is needed to identify factors predictive of response to treatment and ultimately survival. The patients who experienced a fatal infection during prolonged grade 4 neutropenia are of concern: reducing the duration of neutropenia using lower grade immunosuppressive and myelotoxic drugs, should be tested in order to improve the life expectancy of these patients.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Cladribine, Hairy cell leukemia, Infection
Abstract: PB2038
Type: Publication Only
Background
Hairy cell leukemia (HCL) is a rare indolent disease which is curable in most cases. However, a small number of patients is still expected to relapse, or die, mainly due to infective complications.
Aims
The aim of the study is to analyze the course of the disease in a population of uniformly treated patients and identify early and late adverse events during a 20-year follow-up.
Methods
We retrospectively reviewed the charts and collected clinical and laboratory data of 37 patients diagnosed with HCL from October 1997 through May 2017. They were all treated with one course of Cladribine as first line therapy at our Hematology Department. Particularly, we focused on infective events, allergy, secondary neoplasms and relapse during the follow-up.
Results
Most patients sought medical evaluation because of constitutional symptoms and emerging abdominal discomfort. At disease onset, two-third of patients presented with white blood cells count lower than 4x10^9/L and platelets count lower than 100 x10^9/L; massive splenomegaly was detected in 50% of patients. The median time to treatment was 1.4 months (range 0.2-20.6 months): cladribine were administered as a 7-day continue infusion in 89% of patients. The overall response rate was 97%, with 92% complete and 5% partial remissions. During neutropenia, 16 out of 23 patients (69.5%) experienced at least one infectious episode: one patients died, probably due to invasive aspergillosis, 2.7 months after treatment with no sign of hematologic recovery. Median time to recovery of neutropenia was 23 days (range: 5-720 days). Eight patients (22%) reported a diffuse cutaneous allergic reaction: all recovered after few days of antihistamine and steroid administration. Seven patients (19%) experienced a relapse, after a median time of 51.2 months (range: 17.3-146.1 months): 4 were retreated with Cladribine, 1 with Cladribine and Rituximab, 1 with Interferon α and 1 refused treatment. The overall response rate was 57.1% (4 patients, 2 complete and 2 partial responses); 1 patient died two weeks after treatment due to sepsis and 1 was treated with Pentostatin after failure of Interferon α. This patient experienced another relapse 2 years later, and died of septic shock 14 days after starting a BRAF inhibitor. After a median follow-up of 50.6 months (range: 3-219.5 months), overall mortality was 13%: 3 patients died from infection during neutropenia related to chemotherapy, and 2 patients died of causes unrelated to treatment (1 following a myocardial infarction and 1 from senescence). Overall survival and disease-free survival at 20 years were 78% and 81% respectively. Incidence of secondary neoplasms was 2.7%: one patient developed breast cancer 5 years after HCL onset. Age, white blood cells count, bone marrow fibrosis, splenomegaly, infections, allergy, duration of neutropenia and time to best response did not show any statistical significance for mortality at univariate analysis.
Conclusion
Due to the rarity of disease, multicentric survey is needed to identify factors predictive of response to treatment and ultimately survival. The patients who experienced a fatal infection during prolonged grade 4 neutropenia are of concern: reducing the duration of neutropenia using lower grade immunosuppressive and myelotoxic drugs, should be tested in order to improve the life expectancy of these patients.
Session topic: 20. Indolent Non-Hodgkin lymphoma – Clinical
Keyword(s): Cladribine, Hairy cell leukemia, Infection