Outcome of children requiring intensive care following haematopoietic SCT for primary immunodeficiency and other non-malignant disorders

Cole, TS; Johnstone, IC; Pearce, Mark S.; Fulton, Bret; Cant, AJ; Gennery, AR; Slatter, M

doi:10.1038/bmt.2011.26

Cited by 27 publications

(41 citation statements)

References 25 publications

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“…The association between infections and posttransplant mortality has been reported in PID patients in the medical literature. 18,[21][22] In our study, death related to GVHD was associated with infectious complications in 3 patients. In addition, 2 patients died from intracranial hemorrhage in this study (Table 3).…”

Section: Discussionmentioning

confidence: 86%

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Untitled

Patıroğlu

Hh²,

Ünal³

et al. 2017

Exp Clin Transplant

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Objectives: The only curative treatment for many patients with primary immunodeficiency disease is hematopoietic stem cell transplant. In this study, we report the transplant outcomes of patients with primary immunodeficiency diseases. Materials and Methods: Herein, we present the transplant outcomes of 20 patients with primary immunodeficiency disease seen at our center in Kayseri, Turkey, from 2010 to 2015. Results: The disease distribution of th 3 e 20 patients were as follows: 6 patients with severe combined immunodeficiency, 4 patients with hemophagocytic lymphohistiocytosis, 2 patients with chronic granulomatous disease, 2 patients with type 2 Griscelli syndrome, 2 patients with B-cell deficiency plus bone marrow failure, 1 patient with severe congenital neutropenia, 1 patient with X-linked lymphoproliferative disease, 1 patient with T-cell deficiency plus relapsed non-Hodgkin lymphoma, and 1 patient with type 1 leukocyte adhesion deficiency. Of the 20 patients, 11 received related HLA-matched, 6 received haploidentical, 2 received unrelated HLA-matched, and 1 received HLA-mismatched transplant. The median age at transplant was 21 months, and median follow-up was 5 months. Overall survival rate was 65%. Mean engraftment times for neutrophils and platelets were 14.25 ± 3.08 and 24.7 ± 11.4 days. Graft-versushost disease was observed in 30% of patients. Conclusions: Patients with primary immunodeficiency disease treated at our center had acceptable transplant outcomes. This study supports the use of hematopoietic stem cell transplant in patients with primary immunodeficiency disease.

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Section: Discussionmentioning

confidence: 86%

“…This percentage is lower than in previously reported studies. 3,21 Cole and associates 21 reported that 35% of children had at least 1 intensive care unit admission. Two of 6 patients who needed admission (33%) were discharged (Table 3).…”

Section: Discussionmentioning

confidence: 99%

Untitled

Patıroğlu

Hh²,

Ünal³

et al. 2017

Exp Clin Transplant

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“…Data from a previous study demonstrated that HSCT patients with underlying non-malignant conditions had an increased risk for PICU admission. [21] In a large study of 111 critically ill children with primary immunodeficiency undergoing HSCT, 39 (35%) children required a total of 58 PICU admissions. [21] This is in contrast with only 12 (20%) of our 59 infants requiring emergent PICU admissions.…”

Section: Discussionmentioning

confidence: 99%

“…[21] In a large study of 111 critically ill children with primary immunodeficiency undergoing HSCT, 39 (35%) children required a total of 58 PICU admissions. [21] This is in contrast with only 12 (20%) of our 59 infants requiring emergent PICU admissions. One possible reason for this difference is that in the prior study, 104 (94%) children received chemotherapy prior to their HSCT, whereas less than half of our patients received immunosuppression (generally consisting of calcineurin inhibitor and/or rabbit anti thymocyte globulin therapy).…”

Section: Discussionmentioning

confidence: 99%

Clinical Course and Outcome Predictors of Critically Ill Infants With Complete DiGeorge Anomaly Following Thymus Transplantation

Lee

Markert²,

Hornik³

et al. 2014

Pediatric Critical Care Medicine

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Objective To identify risk factors for pediatric intensive care unit (PICU) admission and mortality of infants with complete DiGeorge anomaly treated with thymus transplantation. We hypothesized that age at transplantation and presence of congenital heart disease (CHD) would be risk factors for emergent PICU admission, and these factors plus development of septicemia would increase morbidity and mortality. Design Retrospective review Setting Academic medical-surgical PICU Patients All infants with complete DiGeorge anomaly treated with thymus transplantation between January 1, 1993 and July 1, 2010 Interventions None Measurements and Main Results Seventy one infants with complete DiGeorge anomaly were consented for thymus transplantation, and 59 were transplanted. Median age at transplantation was 5.0 (range: 1.1 – 22.1) months. After transplantation, 12/59 infants (20%) required 25 emergent PICU admissions. Seven of 12 (58%) infants survived to PICU discharge with six surviving six months post-transplantation. Forty two of 59 (71%) infants transplanted had CHD, and 9/12 (75%) who were admitted to the PICU had CHD. In 15/25 admissions (60%), intubation and mechanical ventilation were necessary. There was no difference between median ventilation-free days between infants with and without CHD (33 vs. 23 days, p = 0.544). There was also no correlation between ventilation-free days and age of transplantation (R0.17, p=0.423). Age at transplantation and presence of CHD were not associated with risk for PICU admission (OR: 0.95, 95%CI: 0.78 – 1.15 and OR: 1.27, 95%CI: 0.30 – 5.49, respectively) or PICU mortality (OR: 0.98, 95%CI: 0.73 – 1.31 and OR: 0.40, 95%CI: 0.15 – 1.07, respectively). Conclusions Most transplanted infants did not require emergent PICU admission. Age at transplantation and presence of CHD were not associated with PICU admission or mortality.

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“…For those patients requiring additional support through the transplant period, improvement in paediatric intensive care, close liaison between BMT and PICU teams, and earlier interventions are leading to improved outcomes of patients receiving HSCT but needing to go to PICU. Survival rates are now approaching up to 60%, better survival for those who receive only invasive ventilation and worse survival for those with multi organ failure [56].…”

Section: Severe Combined Immunodeficiencymentioning

confidence: 99%