IMPLICATION OF THE PERCENTAGE OF MACROPHAGES WITH HAEMOPHAGOCYTOSIS IN THE DIAGNOSIS AND PROGNOSIS OF PATIENTS WITH HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
(Abstract release date: 05/19/16)
EHA Library. Knight Asorey T. 06/09/16; 134747; PB1847
Ms. Teresa Knight Asorey
Contributions
Contributions
Abstract
Abstract: PB1847
Type: Publication Only
Background
Haemophagocytic lymphohistiocytosis (HLH) is a serious condition whose diagnosis is based on clinical,analytical and histopathological criteria established by “The International Histiocyte Society”(IHS) in 2004. Haemophagocytosis is one of the diagnostic criteria. However,haemophagocytosis can also be observed in other disorders in absence of HLH
Aims
To review a series of patients with HLH and determine the percentage of macrophages and macrophages with haemophagocytosis observed in bone marrow aspirate (BMA). To compare the haemophagocytosis in this group with that observed in patients without HLH. To evaluate the possible impact of both parameters in diagnosis and survival of HLH patients
Methods
A retrospective study of 31 patients diagnosed with HLH between 2008-2015.Cytomorphological review was carried out by 2 experienced cytohaematologists. The percentage of macrophages was established from a minimum sample of 500 nucleated cells and the percentage of haemophagocytosis from number of macrophages (minimum 30 macrophages).Clinical and analytical data were gathered concerning diagnosis, treatment and follow-up. A control group consisting of patients with haemophagocytosis without HLH criteria was formed. SPSS v19.0 was used for the statistical analysis. Survival analyses were performed using Kaplan-Meier method
Results
31 patients were included:median age 41 (range 6 months-80 years),17 male and 14 female. Underlying diseases were:lymphomas 11/31 (36%); infections 10/31 (32%): 3 EBV, 3 leishmanosis, 2 CMV and 2 leishmanosis associated to EBV; autoimmune diseases 5/31 (16%); other causes (solid neoplasm and surgery) 2/31 (6%); idiopathic 3/31 (10%). At diagnosis 30/31 (97%) had fever and 27/31 (87%) had splenomegaly. One cytopenia was observed in 5/31 (16%), bicytopenia 12/31 (39%) and pancytopenia 14/31 (45%). Biochemical parameters:hyperferritinemia in 20/31 (64%),hypertriglyceridemia in 13/31 (42%),high levels of LDH 17/31 (55%), and hypofybrinogenemia in 9/31 (29%).From the total, 12 patients (39%) were treated according to IHS protocol; 10 (32%) with chemotherapy aimed at lymphoma; 3 (10%) steroides; 5 (16%) with antibiotics and 1 was not treated. At median of follow up of 8.2 months,overall survival was 50%. 15 of 31 patients died, 10 of them (32%) within the first 90 days. 40 patients with haemophagocytosis not attributed to HLH were used as control group. Underlying diseases were:lymphomas 11/40 (27.5%);cytopenias 8/40 (20%);leukaemias 4/40 (10%);plasma cell neoplasms 3/40 (7.5%);bone marrow failure 3/40 (7.5%);myelodysplastic or myeloproliferative syndromes 3/40 (7.5%);infections 2/40 (5%);other causes 6/40 (15%).The average of macrophages in patients with HLH was 1.5% (range 0.5-90%) compared to 1% (0.2- 35%) in the control group (p=0.25). The average percentage of macrophages with haemophagocytosis in patients with HLH was 25% (range 0.5-75%) compared to 3.5% (0.5- 60%) in control group (p<0.001). In the group of patients with HLH, those who had a percentage of macrophages >1.5% had a median survival of 99 days compared to 243 days for those with lower percentage (p=0.76). Patients with percentage of macrophages with haemophagocytosis >25% had a survival rate of 107 days compared to 190 days for those who had a percentage lower than 25% (p=0.45)
Conclusion
The percentage of macrophages observed in BMA in patients fulfilling criteria of HLH is similar to that of patients without HLH. However, there are significant differences in the percentage of macrophages with features of haemophagocytosis. This information could be useful in the differential diagnosis. Among patients with HLH, a trend towards a lower median survival was observed in those with the highest percentage of macrophages as well as in those with a higher percentage of haemophagocytosis.
Session topic: E-poster
Keyword(s): Bone Marrow, Macrophage
Type: Publication Only
Background
Haemophagocytic lymphohistiocytosis (HLH) is a serious condition whose diagnosis is based on clinical,analytical and histopathological criteria established by “The International Histiocyte Society”(IHS) in 2004. Haemophagocytosis is one of the diagnostic criteria. However,haemophagocytosis can also be observed in other disorders in absence of HLH
Aims
To review a series of patients with HLH and determine the percentage of macrophages and macrophages with haemophagocytosis observed in bone marrow aspirate (BMA). To compare the haemophagocytosis in this group with that observed in patients without HLH. To evaluate the possible impact of both parameters in diagnosis and survival of HLH patients
Methods
A retrospective study of 31 patients diagnosed with HLH between 2008-2015.Cytomorphological review was carried out by 2 experienced cytohaematologists. The percentage of macrophages was established from a minimum sample of 500 nucleated cells and the percentage of haemophagocytosis from number of macrophages (minimum 30 macrophages).Clinical and analytical data were gathered concerning diagnosis, treatment and follow-up. A control group consisting of patients with haemophagocytosis without HLH criteria was formed. SPSS v19.0 was used for the statistical analysis. Survival analyses were performed using Kaplan-Meier method
Results
31 patients were included:median age 41 (range 6 months-80 years),17 male and 14 female. Underlying diseases were:lymphomas 11/31 (36%); infections 10/31 (32%): 3 EBV, 3 leishmanosis, 2 CMV and 2 leishmanosis associated to EBV; autoimmune diseases 5/31 (16%); other causes (solid neoplasm and surgery) 2/31 (6%); idiopathic 3/31 (10%). At diagnosis 30/31 (97%) had fever and 27/31 (87%) had splenomegaly. One cytopenia was observed in 5/31 (16%), bicytopenia 12/31 (39%) and pancytopenia 14/31 (45%). Biochemical parameters:hyperferritinemia in 20/31 (64%),hypertriglyceridemia in 13/31 (42%),high levels of LDH 17/31 (55%), and hypofybrinogenemia in 9/31 (29%).From the total, 12 patients (39%) were treated according to IHS protocol; 10 (32%) with chemotherapy aimed at lymphoma; 3 (10%) steroides; 5 (16%) with antibiotics and 1 was not treated. At median of follow up of 8.2 months,overall survival was 50%. 15 of 31 patients died, 10 of them (32%) within the first 90 days. 40 patients with haemophagocytosis not attributed to HLH were used as control group. Underlying diseases were:lymphomas 11/40 (27.5%);cytopenias 8/40 (20%);leukaemias 4/40 (10%);plasma cell neoplasms 3/40 (7.5%);bone marrow failure 3/40 (7.5%);myelodysplastic or myeloproliferative syndromes 3/40 (7.5%);infections 2/40 (5%);other causes 6/40 (15%).The average of macrophages in patients with HLH was 1.5% (range 0.5-90%) compared to 1% (0.2- 35%) in the control group (p=0.25). The average percentage of macrophages with haemophagocytosis in patients with HLH was 25% (range 0.5-75%) compared to 3.5% (0.5- 60%) in control group (p<0.001). In the group of patients with HLH, those who had a percentage of macrophages >1.5% had a median survival of 99 days compared to 243 days for those with lower percentage (p=0.76). Patients with percentage of macrophages with haemophagocytosis >25% had a survival rate of 107 days compared to 190 days for those who had a percentage lower than 25% (p=0.45)
Conclusion
The percentage of macrophages observed in BMA in patients fulfilling criteria of HLH is similar to that of patients without HLH. However, there are significant differences in the percentage of macrophages with features of haemophagocytosis. This information could be useful in the differential diagnosis. Among patients with HLH, a trend towards a lower median survival was observed in those with the highest percentage of macrophages as well as in those with a higher percentage of haemophagocytosis.
Session topic: E-poster
Keyword(s): Bone Marrow, Macrophage
Abstract: PB1847
Type: Publication Only
Background
Haemophagocytic lymphohistiocytosis (HLH) is a serious condition whose diagnosis is based on clinical,analytical and histopathological criteria established by “The International Histiocyte Society”(IHS) in 2004. Haemophagocytosis is one of the diagnostic criteria. However,haemophagocytosis can also be observed in other disorders in absence of HLH
Aims
To review a series of patients with HLH and determine the percentage of macrophages and macrophages with haemophagocytosis observed in bone marrow aspirate (BMA). To compare the haemophagocytosis in this group with that observed in patients without HLH. To evaluate the possible impact of both parameters in diagnosis and survival of HLH patients
Methods
A retrospective study of 31 patients diagnosed with HLH between 2008-2015.Cytomorphological review was carried out by 2 experienced cytohaematologists. The percentage of macrophages was established from a minimum sample of 500 nucleated cells and the percentage of haemophagocytosis from number of macrophages (minimum 30 macrophages).Clinical and analytical data were gathered concerning diagnosis, treatment and follow-up. A control group consisting of patients with haemophagocytosis without HLH criteria was formed. SPSS v19.0 was used for the statistical analysis. Survival analyses were performed using Kaplan-Meier method
Results
31 patients were included:median age 41 (range 6 months-80 years),17 male and 14 female. Underlying diseases were:lymphomas 11/31 (36%); infections 10/31 (32%): 3 EBV, 3 leishmanosis, 2 CMV and 2 leishmanosis associated to EBV; autoimmune diseases 5/31 (16%); other causes (solid neoplasm and surgery) 2/31 (6%); idiopathic 3/31 (10%). At diagnosis 30/31 (97%) had fever and 27/31 (87%) had splenomegaly. One cytopenia was observed in 5/31 (16%), bicytopenia 12/31 (39%) and pancytopenia 14/31 (45%). Biochemical parameters:hyperferritinemia in 20/31 (64%),hypertriglyceridemia in 13/31 (42%),high levels of LDH 17/31 (55%), and hypofybrinogenemia in 9/31 (29%).From the total, 12 patients (39%) were treated according to IHS protocol; 10 (32%) with chemotherapy aimed at lymphoma; 3 (10%) steroides; 5 (16%) with antibiotics and 1 was not treated. At median of follow up of 8.2 months,overall survival was 50%. 15 of 31 patients died, 10 of them (32%) within the first 90 days. 40 patients with haemophagocytosis not attributed to HLH were used as control group. Underlying diseases were:lymphomas 11/40 (27.5%);cytopenias 8/40 (20%);leukaemias 4/40 (10%);plasma cell neoplasms 3/40 (7.5%);bone marrow failure 3/40 (7.5%);myelodysplastic or myeloproliferative syndromes 3/40 (7.5%);infections 2/40 (5%);other causes 6/40 (15%).The average of macrophages in patients with HLH was 1.5% (range 0.5-90%) compared to 1% (0.2- 35%) in the control group (p=0.25). The average percentage of macrophages with haemophagocytosis in patients with HLH was 25% (range 0.5-75%) compared to 3.5% (0.5- 60%) in control group (p<0.001). In the group of patients with HLH, those who had a percentage of macrophages >1.5% had a median survival of 99 days compared to 243 days for those with lower percentage (p=0.76). Patients with percentage of macrophages with haemophagocytosis >25% had a survival rate of 107 days compared to 190 days for those who had a percentage lower than 25% (p=0.45)
Conclusion
The percentage of macrophages observed in BMA in patients fulfilling criteria of HLH is similar to that of patients without HLH. However, there are significant differences in the percentage of macrophages with features of haemophagocytosis. This information could be useful in the differential diagnosis. Among patients with HLH, a trend towards a lower median survival was observed in those with the highest percentage of macrophages as well as in those with a higher percentage of haemophagocytosis.
Session topic: E-poster
Keyword(s): Bone Marrow, Macrophage
Type: Publication Only
Background
Haemophagocytic lymphohistiocytosis (HLH) is a serious condition whose diagnosis is based on clinical,analytical and histopathological criteria established by “The International Histiocyte Society”(IHS) in 2004. Haemophagocytosis is one of the diagnostic criteria. However,haemophagocytosis can also be observed in other disorders in absence of HLH
Aims
To review a series of patients with HLH and determine the percentage of macrophages and macrophages with haemophagocytosis observed in bone marrow aspirate (BMA). To compare the haemophagocytosis in this group with that observed in patients without HLH. To evaluate the possible impact of both parameters in diagnosis and survival of HLH patients
Methods
A retrospective study of 31 patients diagnosed with HLH between 2008-2015.Cytomorphological review was carried out by 2 experienced cytohaematologists. The percentage of macrophages was established from a minimum sample of 500 nucleated cells and the percentage of haemophagocytosis from number of macrophages (minimum 30 macrophages).Clinical and analytical data were gathered concerning diagnosis, treatment and follow-up. A control group consisting of patients with haemophagocytosis without HLH criteria was formed. SPSS v19.0 was used for the statistical analysis. Survival analyses were performed using Kaplan-Meier method
Results
31 patients were included:median age 41 (range 6 months-80 years),17 male and 14 female. Underlying diseases were:lymphomas 11/31 (36%); infections 10/31 (32%): 3 EBV, 3 leishmanosis, 2 CMV and 2 leishmanosis associated to EBV; autoimmune diseases 5/31 (16%); other causes (solid neoplasm and surgery) 2/31 (6%); idiopathic 3/31 (10%). At diagnosis 30/31 (97%) had fever and 27/31 (87%) had splenomegaly. One cytopenia was observed in 5/31 (16%), bicytopenia 12/31 (39%) and pancytopenia 14/31 (45%). Biochemical parameters:hyperferritinemia in 20/31 (64%),hypertriglyceridemia in 13/31 (42%),high levels of LDH 17/31 (55%), and hypofybrinogenemia in 9/31 (29%).From the total, 12 patients (39%) were treated according to IHS protocol; 10 (32%) with chemotherapy aimed at lymphoma; 3 (10%) steroides; 5 (16%) with antibiotics and 1 was not treated. At median of follow up of 8.2 months,overall survival was 50%. 15 of 31 patients died, 10 of them (32%) within the first 90 days. 40 patients with haemophagocytosis not attributed to HLH were used as control group. Underlying diseases were:lymphomas 11/40 (27.5%);cytopenias 8/40 (20%);leukaemias 4/40 (10%);plasma cell neoplasms 3/40 (7.5%);bone marrow failure 3/40 (7.5%);myelodysplastic or myeloproliferative syndromes 3/40 (7.5%);infections 2/40 (5%);other causes 6/40 (15%).The average of macrophages in patients with HLH was 1.5% (range 0.5-90%) compared to 1% (0.2- 35%) in the control group (p=0.25). The average percentage of macrophages with haemophagocytosis in patients with HLH was 25% (range 0.5-75%) compared to 3.5% (0.5- 60%) in control group (p<0.001). In the group of patients with HLH, those who had a percentage of macrophages >1.5% had a median survival of 99 days compared to 243 days for those with lower percentage (p=0.76). Patients with percentage of macrophages with haemophagocytosis >25% had a survival rate of 107 days compared to 190 days for those who had a percentage lower than 25% (p=0.45)
Conclusion
The percentage of macrophages observed in BMA in patients fulfilling criteria of HLH is similar to that of patients without HLH. However, there are significant differences in the percentage of macrophages with features of haemophagocytosis. This information could be useful in the differential diagnosis. Among patients with HLH, a trend towards a lower median survival was observed in those with the highest percentage of macrophages as well as in those with a higher percentage of haemophagocytosis.
Session topic: E-poster
Keyword(s): Bone Marrow, Macrophage
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