Michael D. Keller
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View article: Hematopoietic Cell Transplantation for Wiskott-Aldrich syndrome: A PIDTC Report
Hematopoietic Cell Transplantation for Wiskott-Aldrich syndrome: A PIDTC Report Open
Wiskott-Aldrich syndrome (WAS), an X-linked disorder characterized by immunodeficiency, thrombocytopenia, autoimmunity, and malignancy, can be effectively treated with allogeneic hematopoietic cell transplantation (HCT). Older age at HCT a…
View article: Outcomes Following Matched Sibling Donor Transplantation for Severe Combined Immunodeficiency: A Report from the PIDTC
Outcomes Following Matched Sibling Donor Transplantation for Severe Combined Immunodeficiency: A Report from the PIDTC Open
The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 133 patients with severe combined immunodeficiency (SCID) receiving matched sibling donor (MSD) hematopoietic cell transplantation (HCT) between…
View article: Harnessing virus-specific T cells: expanding therapeutic strategies across diverse populations
Harnessing virus-specific T cells: expanding therapeutic strategies across diverse populations Open
Adoptive transfer of virus-specific T cells (VSTs) has been used for managing viral diseases in immunocompromised patients, including those undergoing hematopoietic stem cell transplantation and solid organ transplantation. Clinical trials…
View article: TCF1 and LEF1 promote B-1a cell homeostasis and regulatory function
TCF1 and LEF1 promote B-1a cell homeostasis and regulatory function Open
B-1 cells are innate-like immune cells abundant in serosal cavities with antibodies enriched in bacterial recognition, yet their existence in humans has been controversial 1–3 . The CD5 + B-1a subset expresses anti-inflammatory molecules i…
View article: Autologous HIV-specific T cell therapy targeting conserved epitopes is well-tolerated in six adults with HIV: an open-label, single-arm phase 1 study
Autologous HIV-specific T cell therapy targeting conserved epitopes is well-tolerated in six adults with HIV: an open-label, single-arm phase 1 study Open
Novel cellular therapies may enable HIV control or cure. HIV-specific T cells targeting conserved immunogenic protein regions of HIV Gag/Pol and the entirety of HIV Nef, termed HST-NEETs, eliminate HIV infected cells in vitro. Here we enro…
View article: Norovirus Infection at the NIH Clinical Center from 2010 to 2023: A Comparative Analysis of Acute vs. Chronic Norovirus Infection with a Focus on Inborn Errors of Immunity
Norovirus Infection at the NIH Clinical Center from 2010 to 2023: A Comparative Analysis of Acute vs. Chronic Norovirus Infection with a Focus on Inborn Errors of Immunity Open
Rationale Chronic norovirus infection (CNI) is a significant cause of morbidity and mortality in immunocompromised patients. We sought to determine clinical and virologic features associated with CNI with an emphasis on IEI. Methods Norovi…
View article: Elevated CD38 Expression and a Concomitant Reduction in NAD+ Levels Underlie CD8+ T Cell Dysfunction in STAT1 GOF
Elevated CD38 Expression and a Concomitant Reduction in NAD+ Levels Underlie CD8+ T Cell Dysfunction in STAT1 GOF Open
Background and Aims STAT1 gain-of-function (GOF) patients experience severe fungal and viral infections and autoimmunity. CD8+ T cells are key mediators of the adaptive immune response by combating infections. CD8+ T cell dysfunction, in t…
View article: Multi-Year Registry Study of Elapegademase Treatment in Patients With Adenosine Deaminase Severe Combined Immunodeficiency (ADA-SCID) Requiring Enzyme Replacement Therapy
Multi-Year Registry Study of Elapegademase Treatment in Patients With Adenosine Deaminase Severe Combined Immunodeficiency (ADA-SCID) Requiring Enzyme Replacement Therapy Open
Purpose The safety and tolerability of elapegademase (elapegademase-lvlr; Revcovi ® ) a PEGylated recombinant adenosine deaminase (ADA), were demonstrated in two Phase 3 clinical trials in the U.S. and Japan in patients with ADA-deficient …
View article: Altered Purinergic Signaling and CD8+ T Cell Dysregulation in STAT3 GOF Syndrome
Altered Purinergic Signaling and CD8+ T Cell Dysregulation in STAT3 GOF Syndrome Open
Signal transduction downstream of activating stimuli controls CD8+ T cell biology, however these external inputs can become uncoupled from transcriptional regulation in Primary Immune Regulatory Disorders (PIRDs). Gain-of-function (GOF) va…
View article: Reconstitution of Norovirus-Specific T-Cell Responses Following Hematopoietic Stem Cell Transplantation in Patients With Inborn Errors of Immunity and Chronic Norovirus Infection
Reconstitution of Norovirus-Specific T-Cell Responses Following Hematopoietic Stem Cell Transplantation in Patients With Inborn Errors of Immunity and Chronic Norovirus Infection Open
Background Chronic norovirus infection (CNI) causes significant morbidity in immunocompromised patients. No effective prevention or treatment currently exists. Methods Two patients with inborn errors of immunity, X-linked severe combined i…
View article: Secondary bone marrow graft loss after third-party virus-specific T cell infusion: Case report of a rare complication
Secondary bone marrow graft loss after third-party virus-specific T cell infusion: Case report of a rare complication Open
Virus-specific T cells (VST) from partially-HLA matched donors have been effective for treatment of refractory viral infections in immunocompromised patients in prior studies with a good safety profile, but rare adverse events have been de…
View article: Antiviral Cellular Therapy for Enhancing T-cell Reconstitution Before or After Hematopoietic Stem Cell Transplantation (ACES): a two-arm, open label phase II interventional trial of pediatric patients with risk factor assessment
Antiviral Cellular Therapy for Enhancing T-cell Reconstitution Before or After Hematopoietic Stem Cell Transplantation (ACES): a two-arm, open label phase II interventional trial of pediatric patients with risk factor assessment Open
Flow cytometry and TCR sequencing dataset from the ACES study (NCT03475212), a phase II multicenter consortium study for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell tran…
View article: Risk factors impacting outcomes of third party virus-specific T cell therapy for refractory viral infections in children
Risk factors impacting outcomes of third party virus-specific T cell therapy for refractory viral infections in children Open
Flow cytometry dataset from the ACES study (NCT#03475212), a phase II multicenter consortium study for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell transplant with refrac…
View article: Antiviral Cellular Therapy for Enhancing T-cell Reconstitution Before or After Hematopoietic Stem Cell Transplantation (ACES): a two-arm, open label phase II interventional trial of pediatric patients with risk factor assessment
Antiviral Cellular Therapy for Enhancing T-cell Reconstitution Before or After Hematopoietic Stem Cell Transplantation (ACES): a two-arm, open label phase II interventional trial of pediatric patients with risk factor assessment Open
Flow cytometry and TCR sequencing dataset from the ACES study (NCT03475212), a phase II multicenter consortium study for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell tran…
View article: Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion
Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion Open
Here, we describe a severe adverse event of virus-specific T cell (VST therapy) in an infant with severe combined immunodeficiency who received VSTs for treatment of CMV viremia post-transplant and developed secondary graft rejection at 1-…
View article: Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion
Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion Open
Here, we describe a severe adverse event of virus-specific T cell (VST therapy) in an infant with severe combined immunodeficiency who received VSTs for treatment of CMV viremia post-transplant and developed secondary graft rejection at 1-…
View article: Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion
Secondary Graft Loss After Third-party "Off-The-Shelf" Antigen-Specific T cell Infusion Open
Here, we describe a severe adverse event of virus-specific T cell (VST therapy) in an infant with severe combined immunodeficiency who received VSTs for treatment of CMV viremia post-transplant and developed secondary graft rejection at 1-…
View article: Chromosomal microarray analysis supplements exome sequencing to diagnose children with suspected inborn errors of immunity
Chromosomal microarray analysis supplements exome sequencing to diagnose children with suspected inborn errors of immunity Open
Purpose Though copy number variants (CNVs) have been suggested to play a significant role in inborn errors of immunity (IEI), the precise nature of this role remains largely unexplored. We sought to determine the diagnostic contribution of…
View article: Third-Party and Patient-Specific Donor-Derived Virus-Specific T Cells Demonstrate Similar Efficacy and Safety for Management of Viral Infections after Hematopoietic Stem Cell Transplantation in Children and Young Adults
Third-Party and Patient-Specific Donor-Derived Virus-Specific T Cells Demonstrate Similar Efficacy and Safety for Management of Viral Infections after Hematopoietic Stem Cell Transplantation in Children and Young Adults Open
Infections with double-stranded DNA viruses are a common complication after hematopoietic stem cell transplantation (HSCT) and cause significant morbidity and mortality in the post-transplantation period. Both donor-derived (DD) and third-…
View article: Impaired thymic AIRE expression underlies autoantibodies against type I IFNs in humans with inborn errors of the alternative NF-kB pathway
Impaired thymic AIRE expression underlies autoantibodies against type I IFNs in humans with inborn errors of the alternative NF-kB pathway Open
Patients with inborn errors of the alternative NF-κB pathway have low thymic AIRE expression, leading to the development of auto-Abs neutralizing type I IFNs, and severe viral diseases.