Contributions
Abstract: PB1839
Type: Publication Only
Background
Chronic primary neutropenia (CPN) is a rare benign entity with increased susceptibility to bacterial infections. So far there is no epidemiological data from Swiss CPN patients available.
Aims
We aimed to evaluate patients characteristics, rate of infections and long-term outcomes of adult CPN patients at our policlinic.
Methods
This is a retrospective study, in which we reviewed all patients recorded as chronic neutropenia in our policlinic. Patients with neutropenia secondary to infection, drugs, tumor, malnutrition, vitamin B12 deficiency, benign ethnic neutropenia, T-cell large granular leukemia or associated to pancytopenia were excluded. Repetitive infections were defined as ≥ 3 episodes per year. Infections requiring hospitalization were defined as severe.
Results
From 1998 to February 2017, we identified 20 adults with CPN. CPN patients were more often female 16/20 (80%). The median age at diagnosis was 27 (range 1-66) years. Five patients were < 18 years at diagnosis. The median absolute neutrophil count (ANC) at diagnosis was 0.41 G/L (range 0.0-0.90), and the median ANC at last follow-up was 0.8 G/L (range 0.03-7.36). All patients underwent bone marrow evaluation and there was no clear bone marrow pathology pattern. Two of twelve (16%) patients evaluated for anti-granulocyte autoantibodies were positive. Regarding management, therapy was aimed to provide a normal lifestyle. Thus 12/20 patients (60%) received G-CSF either sporadically (6/12) or continuously (6/12) and all responded with increase in the number of neutrophils. Repetitive infections were recorded in 7/20 (35%) patients. 5 patients (25%) presented a total of 23 severe bacterial infections; of these, 3 were diagnosed during childhood. 13 patients (65%) neither had repetitive nor severe infections, 6 of them were treated with different G-CSF modalities. Three infection episodes occurred in 3 patients who were under treatment with G-CSF and had normal neutrophil count. 19 pregnancies were documented (12 before initial diagnosis), 1 patient received G-CSF during 2 of her 6 pregnancies with good outcome and one patient had an uneventful pregnancy without G-CSF. The median follow-up of this cohort was 122 months. 3 patients were lost to follow-up. No death due to bacterial infections or hematologic malignancies occurred. One patient died due to glioblastoma multiforme 10 years after diagnosis of CPN.
Conclusion
Our adult CPN patient cohort is characterized by a female predominance and a benign outcome. Patients diagnosed during childhood seem to have a higher risk of infections. No secondary hematological malignancies were observed. G-CSF therapy was based on individual schedules and was well tolerated. The reasons why some patients are susceptible to repetitive infections and others are not, remain to be elucidated.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): G-CSF, Infection, neutropenia
Abstract: PB1839
Type: Publication Only
Background
Chronic primary neutropenia (CPN) is a rare benign entity with increased susceptibility to bacterial infections. So far there is no epidemiological data from Swiss CPN patients available.
Aims
We aimed to evaluate patients characteristics, rate of infections and long-term outcomes of adult CPN patients at our policlinic.
Methods
This is a retrospective study, in which we reviewed all patients recorded as chronic neutropenia in our policlinic. Patients with neutropenia secondary to infection, drugs, tumor, malnutrition, vitamin B12 deficiency, benign ethnic neutropenia, T-cell large granular leukemia or associated to pancytopenia were excluded. Repetitive infections were defined as ≥ 3 episodes per year. Infections requiring hospitalization were defined as severe.
Results
From 1998 to February 2017, we identified 20 adults with CPN. CPN patients were more often female 16/20 (80%). The median age at diagnosis was 27 (range 1-66) years. Five patients were < 18 years at diagnosis. The median absolute neutrophil count (ANC) at diagnosis was 0.41 G/L (range 0.0-0.90), and the median ANC at last follow-up was 0.8 G/L (range 0.03-7.36). All patients underwent bone marrow evaluation and there was no clear bone marrow pathology pattern. Two of twelve (16%) patients evaluated for anti-granulocyte autoantibodies were positive. Regarding management, therapy was aimed to provide a normal lifestyle. Thus 12/20 patients (60%) received G-CSF either sporadically (6/12) or continuously (6/12) and all responded with increase in the number of neutrophils. Repetitive infections were recorded in 7/20 (35%) patients. 5 patients (25%) presented a total of 23 severe bacterial infections; of these, 3 were diagnosed during childhood. 13 patients (65%) neither had repetitive nor severe infections, 6 of them were treated with different G-CSF modalities. Three infection episodes occurred in 3 patients who were under treatment with G-CSF and had normal neutrophil count. 19 pregnancies were documented (12 before initial diagnosis), 1 patient received G-CSF during 2 of her 6 pregnancies with good outcome and one patient had an uneventful pregnancy without G-CSF. The median follow-up of this cohort was 122 months. 3 patients were lost to follow-up. No death due to bacterial infections or hematologic malignancies occurred. One patient died due to glioblastoma multiforme 10 years after diagnosis of CPN.
Conclusion
Our adult CPN patient cohort is characterized by a female predominance and a benign outcome. Patients diagnosed during childhood seem to have a higher risk of infections. No secondary hematological malignancies were observed. G-CSF therapy was based on individual schedules and was well tolerated. The reasons why some patients are susceptible to repetitive infections and others are not, remain to be elucidated.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): G-CSF, Infection, neutropenia