THE OUTCOME OF HIGH RISK LANGERHANS CELL HISTIOCYTOSIS (LCH) IN EGYPTIAN CHILDREN. A SINGLE CENTER EXPERIENCE
(Abstract release date: 05/19/16)
EHA Library. Sedky M. 06/09/16; 134961; PB2061
Prof. Dr. Mohamed Sedky Sedky
Contributions
Contributions
Abstract
Abstract: PB2061
Type: Publication Only
Background
High risk multi-system organs (RO+) Langerhans cell histiocytosis (LCH) including hemopoietic cytopenias, liver and spleen disease, have the highest mortality if failing first line treatment. Methotrexate (MTX) association with Vinblastine (VBL) and Prednisone (PRED) has been tried upfront and a second line cladribine (2cda) has been proposed in RO+LCH.
Aims
To present the outcome of RO+ LCH in a pediatric single centre population.
Methods
A total of 50 RO+ LCH patients, M/F 26/24, median age 1.7 years (0.19-10.1) treated between August 2007 and June 2015 were retrospectively analyzed. A first line VBL, PRED with MTX without a different second line was adopted till 2012 where methotrexate was omitted and 2cda was introduced as a salvage line.
Results
The 5 year overall survival (OS) is 69% and the event free survival (EFS) is 17%. At week 6 and 12, “better’ response was obtained in 37(74%) and 33 (66%) patients respectively. Seventeen (34%) and 15 (30%) patients progressed and reactivated their disease respectively. At last follow up, 34 and 2 patients were in ‘better’ and ‘worse’ status respectively and 14 patients died. In univariate analysis, the factors that affected OS were hemopoietic cytopenias with 33%, 76 % and 62% in tri, bi and monocytopenia respectively p0.017, hepatic affection with 25%, 93% and 61% in hepatic dysfunction, hepatomegaly and both respectively p 0.027, combined RO+ with 47%, 68% and 100%, in tri, bi and mono organ(s) respectively p 0.045, number of induction: 47% and 76% in one cycle and 2cycles respectively p0.026, failure of 1st line treatment: 27%, 76% and 50% in progression, reactivation and both respectively p <0.001,Salvage with 2cda 56%, and 45% without p0.001, progression salvage with 2cda 57% and 10% without p0.008. The factors that affected EFS were cytopenias with 0%, 19 %, and 34% in tri, bi and monocytopenia respectively p0.015, bony CNS risk sites with 9% versus 27% if none p 0.06, MTX 26% with and 13% without p0.005. The factors that affected reactivation were: male gender p0.014, season autumn/winter p<0.01, lung cysts p0.043. The factors that affected disease progression were tricytopenia p <0.01, hepatic dysfunction p<0.01, hepatic dysfunction and hepatomegaly combination p0.04.
Conclusion
The outcome of RO+LCH in pediatric egyptian population remains satisfactory despite a high proportion of reactivation. Disease progression post induction carries a dismal prognosis without a salvage line. Hemopoietic tricytopenias and hepatic dysfunction carry the worst prognosis. Methotrexate as a 1st line and 2cda as salvage line are efficient in improving the outcome.
Session topic: E-poster
Keyword(s): Cladribine, Langerhans Cell Histiocytosis, Methotrexate, Progression, Survival
Type: Publication Only
Background
High risk multi-system organs (RO+) Langerhans cell histiocytosis (LCH) including hemopoietic cytopenias, liver and spleen disease, have the highest mortality if failing first line treatment. Methotrexate (MTX) association with Vinblastine (VBL) and Prednisone (PRED) has been tried upfront and a second line cladribine (2cda) has been proposed in RO+LCH.
Aims
To present the outcome of RO+ LCH in a pediatric single centre population.
Methods
A total of 50 RO+ LCH patients, M/F 26/24, median age 1.7 years (0.19-10.1) treated between August 2007 and June 2015 were retrospectively analyzed. A first line VBL, PRED with MTX without a different second line was adopted till 2012 where methotrexate was omitted and 2cda was introduced as a salvage line.
Results
The 5 year overall survival (OS) is 69% and the event free survival (EFS) is 17%. At week 6 and 12, “better’ response was obtained in 37(74%) and 33 (66%) patients respectively. Seventeen (34%) and 15 (30%) patients progressed and reactivated their disease respectively. At last follow up, 34 and 2 patients were in ‘better’ and ‘worse’ status respectively and 14 patients died. In univariate analysis, the factors that affected OS were hemopoietic cytopenias with 33%, 76 % and 62% in tri, bi and monocytopenia respectively p0.017, hepatic affection with 25%, 93% and 61% in hepatic dysfunction, hepatomegaly and both respectively p 0.027, combined RO+ with 47%, 68% and 100%, in tri, bi and mono organ(s) respectively p 0.045, number of induction: 47% and 76% in one cycle and 2cycles respectively p0.026, failure of 1st line treatment: 27%, 76% and 50% in progression, reactivation and both respectively p <0.001,Salvage with 2cda 56%, and 45% without p0.001, progression salvage with 2cda 57% and 10% without p0.008. The factors that affected EFS were cytopenias with 0%, 19 %, and 34% in tri, bi and monocytopenia respectively p0.015, bony CNS risk sites with 9% versus 27% if none p 0.06, MTX 26% with and 13% without p0.005. The factors that affected reactivation were: male gender p0.014, season autumn/winter p<0.01, lung cysts p0.043. The factors that affected disease progression were tricytopenia p <0.01, hepatic dysfunction p<0.01, hepatic dysfunction and hepatomegaly combination p0.04.
Conclusion
The outcome of RO+LCH in pediatric egyptian population remains satisfactory despite a high proportion of reactivation. Disease progression post induction carries a dismal prognosis without a salvage line. Hemopoietic tricytopenias and hepatic dysfunction carry the worst prognosis. Methotrexate as a 1st line and 2cda as salvage line are efficient in improving the outcome.
Session topic: E-poster
Keyword(s): Cladribine, Langerhans Cell Histiocytosis, Methotrexate, Progression, Survival
Abstract: PB2061
Type: Publication Only
Background
High risk multi-system organs (RO+) Langerhans cell histiocytosis (LCH) including hemopoietic cytopenias, liver and spleen disease, have the highest mortality if failing first line treatment. Methotrexate (MTX) association with Vinblastine (VBL) and Prednisone (PRED) has been tried upfront and a second line cladribine (2cda) has been proposed in RO+LCH.
Aims
To present the outcome of RO+ LCH in a pediatric single centre population.
Methods
A total of 50 RO+ LCH patients, M/F 26/24, median age 1.7 years (0.19-10.1) treated between August 2007 and June 2015 were retrospectively analyzed. A first line VBL, PRED with MTX without a different second line was adopted till 2012 where methotrexate was omitted and 2cda was introduced as a salvage line.
Results
The 5 year overall survival (OS) is 69% and the event free survival (EFS) is 17%. At week 6 and 12, “better’ response was obtained in 37(74%) and 33 (66%) patients respectively. Seventeen (34%) and 15 (30%) patients progressed and reactivated their disease respectively. At last follow up, 34 and 2 patients were in ‘better’ and ‘worse’ status respectively and 14 patients died. In univariate analysis, the factors that affected OS were hemopoietic cytopenias with 33%, 76 % and 62% in tri, bi and monocytopenia respectively p0.017, hepatic affection with 25%, 93% and 61% in hepatic dysfunction, hepatomegaly and both respectively p 0.027, combined RO+ with 47%, 68% and 100%, in tri, bi and mono organ(s) respectively p 0.045, number of induction: 47% and 76% in one cycle and 2cycles respectively p0.026, failure of 1st line treatment: 27%, 76% and 50% in progression, reactivation and both respectively p <0.001,Salvage with 2cda 56%, and 45% without p0.001, progression salvage with 2cda 57% and 10% without p0.008. The factors that affected EFS were cytopenias with 0%, 19 %, and 34% in tri, bi and monocytopenia respectively p0.015, bony CNS risk sites with 9% versus 27% if none p 0.06, MTX 26% with and 13% without p0.005. The factors that affected reactivation were: male gender p0.014, season autumn/winter p<0.01, lung cysts p0.043. The factors that affected disease progression were tricytopenia p <0.01, hepatic dysfunction p<0.01, hepatic dysfunction and hepatomegaly combination p0.04.
Conclusion
The outcome of RO+LCH in pediatric egyptian population remains satisfactory despite a high proportion of reactivation. Disease progression post induction carries a dismal prognosis without a salvage line. Hemopoietic tricytopenias and hepatic dysfunction carry the worst prognosis. Methotrexate as a 1st line and 2cda as salvage line are efficient in improving the outcome.
Session topic: E-poster
Keyword(s): Cladribine, Langerhans Cell Histiocytosis, Methotrexate, Progression, Survival
Type: Publication Only
Background
High risk multi-system organs (RO+) Langerhans cell histiocytosis (LCH) including hemopoietic cytopenias, liver and spleen disease, have the highest mortality if failing first line treatment. Methotrexate (MTX) association with Vinblastine (VBL) and Prednisone (PRED) has been tried upfront and a second line cladribine (2cda) has been proposed in RO+LCH.
Aims
To present the outcome of RO+ LCH in a pediatric single centre population.
Methods
A total of 50 RO+ LCH patients, M/F 26/24, median age 1.7 years (0.19-10.1) treated between August 2007 and June 2015 were retrospectively analyzed. A first line VBL, PRED with MTX without a different second line was adopted till 2012 where methotrexate was omitted and 2cda was introduced as a salvage line.
Results
The 5 year overall survival (OS) is 69% and the event free survival (EFS) is 17%. At week 6 and 12, “better’ response was obtained in 37(74%) and 33 (66%) patients respectively. Seventeen (34%) and 15 (30%) patients progressed and reactivated their disease respectively. At last follow up, 34 and 2 patients were in ‘better’ and ‘worse’ status respectively and 14 patients died. In univariate analysis, the factors that affected OS were hemopoietic cytopenias with 33%, 76 % and 62% in tri, bi and monocytopenia respectively p0.017, hepatic affection with 25%, 93% and 61% in hepatic dysfunction, hepatomegaly and both respectively p 0.027, combined RO+ with 47%, 68% and 100%, in tri, bi and mono organ(s) respectively p 0.045, number of induction: 47% and 76% in one cycle and 2cycles respectively p0.026, failure of 1st line treatment: 27%, 76% and 50% in progression, reactivation and both respectively p <0.001,Salvage with 2cda 56%, and 45% without p0.001, progression salvage with 2cda 57% and 10% without p0.008. The factors that affected EFS were cytopenias with 0%, 19 %, and 34% in tri, bi and monocytopenia respectively p0.015, bony CNS risk sites with 9% versus 27% if none p 0.06, MTX 26% with and 13% without p0.005. The factors that affected reactivation were: male gender p0.014, season autumn/winter p<0.01, lung cysts p0.043. The factors that affected disease progression were tricytopenia p <0.01, hepatic dysfunction p<0.01, hepatic dysfunction and hepatomegaly combination p0.04.
Conclusion
The outcome of RO+LCH in pediatric egyptian population remains satisfactory despite a high proportion of reactivation. Disease progression post induction carries a dismal prognosis without a salvage line. Hemopoietic tricytopenias and hepatic dysfunction carry the worst prognosis. Methotrexate as a 1st line and 2cda as salvage line are efficient in improving the outcome.
Session topic: E-poster
Keyword(s): Cladribine, Langerhans Cell Histiocytosis, Methotrexate, Progression, Survival
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