: INVASIVE PULMONARY ASPERGILLOSIS IN ALLOGENEIC BONE MARROW RECIPIENTS WITH Β- THALASSAEMIA MAJOR OR SICKLE CELL DISEASE: INCIDENCE AND RISK FACTORS.
(Abstract release date: 05/19/16)
EHA Library. Paciaroni K. 06/09/16; 132725; E1176
Dr. Katia Paciaroni
Contributions
Contributions
Abstract
Abstract: E1176
Type: Eposter Presentation
Background
Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in allogeneic Haematopoietic Stem Cell Transplantation (HSCT) recipients. IPA has been well characterized in adults and in the setting of oncological transplant. No data are available regarding IPA in patients with β- globin gene disorders undergoing bone marrow transplant (BMT).
Aims
To evaluate the incidence and the outcome of IPA among BMT recipients with β-Thalassaemia Major or Sicke cell Anaemia (SCA).
Methods
We evaluated the occurrence, the clinical setting and the clinical outcome of IPA in pediatric patients affected by Thalassaemia major or SCA transplanted at our institution.
Results
A total of 292 consecutive patients (median age 11,6, range 1,9-28 years) with β- globin gene disorders who underwent BMT (232 HLA-identical, related donor; 54 haplotype-identical donor and 4 matched, unrelated donor ) were studied. Overall, the incidence of proven or probable IPA was 2.73% (8 out of 292 cases). The median time to onset IPA infection after transplantation was 68 days (range, 13-183 days). In particular, in 5 cases (50%) IPA were diagnosed in the late phase after transplant (day >60) and in 3 cases (50%) were diagnosed during the post-BMT neutropenic period before engraftment.
All grade of Graft-versus-host-disease (GVHD) was present in 4 (50.0%) of 8 patients with IPA, compared with 97 (37,7%) of 284 patients without fungal infection ( P= n.s.). Among 8 cases with IPA an alternative donor (matched unrelated or haplotype-identical) was used in 5 patients (62.5%) compared with 55 cases (19.3%) of 284 recipients without IPA (P= 0.003).
The infection remained confined in the lung in 7 (87.5%) of 8 IPA cases, in 2 cases surgical intervention was adopted in addition to the adequate systemic anti-fungal medical therapy; only in 1 case the infection was multifocal with CSN involvement. The overall mortality rate for IPA was 0.7% (2 of 292 patients) whereas the IPA attributable mortality rate observed in our population was 25% (2 of 8 cases).
Conclusion
Our data show that in a population affected by β- globin gene disorders who undergoing allogeneic BMT, the IPA rarely develop (2,7%) and the overall mortality (0.7%) and the IPA attributable mortality rate (25%) is markedly lower then the one observed in the setting of haematological malignancies. In our cohort, a significant risk factor for IPA was the alternative donor.
Session topic: E-poster
Keyword(s): Haploidentical stem cell transplantation, Hemoglobinopathy, Invasive pulmonary aspergillosis
Type: Eposter Presentation
Background
Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in allogeneic Haematopoietic Stem Cell Transplantation (HSCT) recipients. IPA has been well characterized in adults and in the setting of oncological transplant. No data are available regarding IPA in patients with β- globin gene disorders undergoing bone marrow transplant (BMT).
Aims
To evaluate the incidence and the outcome of IPA among BMT recipients with β-Thalassaemia Major or Sicke cell Anaemia (SCA).
Methods
We evaluated the occurrence, the clinical setting and the clinical outcome of IPA in pediatric patients affected by Thalassaemia major or SCA transplanted at our institution.
Results
A total of 292 consecutive patients (median age 11,6, range 1,9-28 years) with β- globin gene disorders who underwent BMT (232 HLA-identical, related donor; 54 haplotype-identical donor and 4 matched, unrelated donor ) were studied. Overall, the incidence of proven or probable IPA was 2.73% (8 out of 292 cases). The median time to onset IPA infection after transplantation was 68 days (range, 13-183 days). In particular, in 5 cases (50%) IPA were diagnosed in the late phase after transplant (day >60) and in 3 cases (50%) were diagnosed during the post-BMT neutropenic period before engraftment.
All grade of Graft-versus-host-disease (GVHD) was present in 4 (50.0%) of 8 patients with IPA, compared with 97 (37,7%) of 284 patients without fungal infection ( P= n.s.). Among 8 cases with IPA an alternative donor (matched unrelated or haplotype-identical) was used in 5 patients (62.5%) compared with 55 cases (19.3%) of 284 recipients without IPA (P= 0.003).
The infection remained confined in the lung in 7 (87.5%) of 8 IPA cases, in 2 cases surgical intervention was adopted in addition to the adequate systemic anti-fungal medical therapy; only in 1 case the infection was multifocal with CSN involvement. The overall mortality rate for IPA was 0.7% (2 of 292 patients) whereas the IPA attributable mortality rate observed in our population was 25% (2 of 8 cases).
Conclusion
Our data show that in a population affected by β- globin gene disorders who undergoing allogeneic BMT, the IPA rarely develop (2,7%) and the overall mortality (0.7%) and the IPA attributable mortality rate (25%) is markedly lower then the one observed in the setting of haematological malignancies. In our cohort, a significant risk factor for IPA was the alternative donor.
Session topic: E-poster
Keyword(s): Haploidentical stem cell transplantation, Hemoglobinopathy, Invasive pulmonary aspergillosis
Abstract: E1176
Type: Eposter Presentation
Background
Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in allogeneic Haematopoietic Stem Cell Transplantation (HSCT) recipients. IPA has been well characterized in adults and in the setting of oncological transplant. No data are available regarding IPA in patients with β- globin gene disorders undergoing bone marrow transplant (BMT).
Aims
To evaluate the incidence and the outcome of IPA among BMT recipients with β-Thalassaemia Major or Sicke cell Anaemia (SCA).
Methods
We evaluated the occurrence, the clinical setting and the clinical outcome of IPA in pediatric patients affected by Thalassaemia major or SCA transplanted at our institution.
Results
A total of 292 consecutive patients (median age 11,6, range 1,9-28 years) with β- globin gene disorders who underwent BMT (232 HLA-identical, related donor; 54 haplotype-identical donor and 4 matched, unrelated donor ) were studied. Overall, the incidence of proven or probable IPA was 2.73% (8 out of 292 cases). The median time to onset IPA infection after transplantation was 68 days (range, 13-183 days). In particular, in 5 cases (50%) IPA were diagnosed in the late phase after transplant (day >60) and in 3 cases (50%) were diagnosed during the post-BMT neutropenic period before engraftment.
All grade of Graft-versus-host-disease (GVHD) was present in 4 (50.0%) of 8 patients with IPA, compared with 97 (37,7%) of 284 patients without fungal infection ( P= n.s.). Among 8 cases with IPA an alternative donor (matched unrelated or haplotype-identical) was used in 5 patients (62.5%) compared with 55 cases (19.3%) of 284 recipients without IPA (P= 0.003).
The infection remained confined in the lung in 7 (87.5%) of 8 IPA cases, in 2 cases surgical intervention was adopted in addition to the adequate systemic anti-fungal medical therapy; only in 1 case the infection was multifocal with CSN involvement. The overall mortality rate for IPA was 0.7% (2 of 292 patients) whereas the IPA attributable mortality rate observed in our population was 25% (2 of 8 cases).
Conclusion
Our data show that in a population affected by β- globin gene disorders who undergoing allogeneic BMT, the IPA rarely develop (2,7%) and the overall mortality (0.7%) and the IPA attributable mortality rate (25%) is markedly lower then the one observed in the setting of haematological malignancies. In our cohort, a significant risk factor for IPA was the alternative donor.
Session topic: E-poster
Keyword(s): Haploidentical stem cell transplantation, Hemoglobinopathy, Invasive pulmonary aspergillosis
Type: Eposter Presentation
Background
Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in allogeneic Haematopoietic Stem Cell Transplantation (HSCT) recipients. IPA has been well characterized in adults and in the setting of oncological transplant. No data are available regarding IPA in patients with β- globin gene disorders undergoing bone marrow transplant (BMT).
Aims
To evaluate the incidence and the outcome of IPA among BMT recipients with β-Thalassaemia Major or Sicke cell Anaemia (SCA).
Methods
We evaluated the occurrence, the clinical setting and the clinical outcome of IPA in pediatric patients affected by Thalassaemia major or SCA transplanted at our institution.
Results
A total of 292 consecutive patients (median age 11,6, range 1,9-28 years) with β- globin gene disorders who underwent BMT (232 HLA-identical, related donor; 54 haplotype-identical donor and 4 matched, unrelated donor ) were studied. Overall, the incidence of proven or probable IPA was 2.73% (8 out of 292 cases). The median time to onset IPA infection after transplantation was 68 days (range, 13-183 days). In particular, in 5 cases (50%) IPA were diagnosed in the late phase after transplant (day >60) and in 3 cases (50%) were diagnosed during the post-BMT neutropenic period before engraftment.
All grade of Graft-versus-host-disease (GVHD) was present in 4 (50.0%) of 8 patients with IPA, compared with 97 (37,7%) of 284 patients without fungal infection ( P= n.s.). Among 8 cases with IPA an alternative donor (matched unrelated or haplotype-identical) was used in 5 patients (62.5%) compared with 55 cases (19.3%) of 284 recipients without IPA (P= 0.003).
The infection remained confined in the lung in 7 (87.5%) of 8 IPA cases, in 2 cases surgical intervention was adopted in addition to the adequate systemic anti-fungal medical therapy; only in 1 case the infection was multifocal with CSN involvement. The overall mortality rate for IPA was 0.7% (2 of 292 patients) whereas the IPA attributable mortality rate observed in our population was 25% (2 of 8 cases).
Conclusion
Our data show that in a population affected by β- globin gene disorders who undergoing allogeneic BMT, the IPA rarely develop (2,7%) and the overall mortality (0.7%) and the IPA attributable mortality rate (25%) is markedly lower then the one observed in the setting of haematological malignancies. In our cohort, a significant risk factor for IPA was the alternative donor.
Session topic: E-poster
Keyword(s): Haploidentical stem cell transplantation, Hemoglobinopathy, Invasive pulmonary aspergillosis
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