R. Marsh

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CCR5 inhibitor as novel acute graft versus host disease prophylaxis in children and young adults undergoing allogeneic stem cell transplant: results of the phase II study

K. Myers, S. Davies, P. Mehta, 2020, Bone Marrow Transplantation.

A Pharmacokinetic and Pharmacodynamic Study of Maraviroc as Acute Graft-versus-Host Disease Prophylaxis in Pediatric Allogeneic Stem Cell Transplant Recipients with Nonmalignant Diagnoses.

A. Vinks, Kana Mizuno, S. Davies, 2016, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Mg2+ Regulates Cytotoxic Functions of NK and CD8 T Cells in Chronic EBV Infection Through NKG2D

S. Pittaluga, M. Biancalana, T. Kuijpers, 2013, Science.

A Reduced-Intensity Conditioning Regimen for Patients with Dyskeratosis Congenita Undergoing Hematopoietic Stem Cell Transplantation.

K. Myers, S. Davies, P. Mehta, 2016, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Current Flow Cytometric Assays for the Screening and Diagnosis of Primary HLH

S. Chiang, R. Marsh, J. Bleesing, 2019, Front. Immunol..

Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy

J. Hughes, J. McElwee, Y. Zhang, 2015, Science.

Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency.

K. Nichols, M. Lenardo, A. Snow, 2009, The Journal of clinical investigation.

Comprehensive molecular diagnosis of Epstein–Barr virus-associated lymphoproliferative diseases using next-generation sequencing

O. Ohara, H. Kanegane, C. Picard, 2018, International Journal of Hematology.

APOL1-Associated Collapsing Focal Segmental Glomerulosclerosis in a Patient With Stimulator of Interferon Genes (STING)-Associated Vasculopathy With Onset in Infancy (SAVI)

G. Schulert, C. Larsen, R. Marsh, 2019, American Journal of Kidney Diseases.

Reduced-intensity single-unit unrelated cord blood transplant with optional immune boost for nonmalignant disorders.

P. Szabolcs, Xiaohua Chen, Mark T. Vander Lugt, 2020, Blood advances.

The Value of Chromosome Analysis to Interrogate Variants in DNMT3B Causing Immunodeficiency, Centromeric Instability, and Facial Anomaly Syndrome Type I (ICF1)

T. Smolarek, Miao Sun, P. Rathbun, 2019, Journal of Clinical Immunology.

Pediatric Hemophagocytic Lymphohistiocytosis (HLH).

S. Canna, R. Marsh, 2020, Blood.

T cell activation profiles distinguish hemophagocytic lymphohistiocytosis and early sepsis.

Michael M. Henry, T. Nguyen, H. Wong, 2020, Blood.

Epstein–Barr Virus and Hemophagocytic Lymphohistiocytosis

R. Marsh, 2018, Front. Immunol..

Blood, and not urine, BK viral load predicts renal outcome in children with hemorrhagic cystitis following hematopoietic stem cell transplantation.

S. Davies, P. Mehta, J. Goebel, 2011, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Evolution of Our Understanding of XIAP Deficiency

C. Booth, R. Marsh, Anne C. Mudde, 2021, Frontiers in Pediatrics.

The X-Linked Lymphoproliferative Syndromes

K. Nichols, R. Marsh, 2014 .

Cytokine Profile of Engraftment Syndrome in Pediatric Hematopoietic Stem Cell Transplant Recipients.

S. Davies, A. Filipovich, J. Villanueva, 2016, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Thinking Beyond HLH: Clinical Features of Patients with Concurrent Presentation of Hemophagocytic Lymphohistiocytosis and Thrombotic Microangiopathy

K. Myers, S. Davies, S. Jodele, 2020, Journal of Clinical Immunology.

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease.

Kejian Zhang, A. Filipovich, B. Mcclimon, 2010, Blood.

Histiocytic disorders

M. Merad, K. Ribeiro, J. Haroche, 2021, Nature Reviews Disease Primers.

A rapid flow cytometric screening test for X‐linked lymphoproliferative disease due to XIAP deficiency

A. Snow, D. Marmer, H. Su, 2009, Cytometry. Part B, Clinical cytometry.

Patients with X-linked lymphoproliferative disease due to BIRC4 mutation have normal invariant natural killer T-cell populations.

D. Marmer, Mi‐Ok Kim, Kejian Zhang, 2009, Clinical immunology.

Screening for Wiskott-Aldrich syndrome by flow cytometry.

J. Bussel, Kejian Zhang, Ammar Husami, 2018, The Journal of allergy and clinical immunology.

Allogeneic hematopoietic stem cell transplantation for severe, refractory juvenile idiopathic arthritis.

G. Lucchini, J. Glanville, A. Gennery, 2018, Blood advances.

A challenging undertaking: Stem cell transplantation for immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.

S. Davies, Kejian Zhang, A. Filipovich, 2016, The Journal of allergy and clinical immunology.

Risk Factors of Pneumonia in Primary Antibody Deficiency Patients Receiving Immunoglobulin Therapy: Data from the US Immunodeficiency Network (USIDNET)

C. Cunningham-Rundles, K. Sullivan, R. Fuleihan, 2022, Journal of Clinical Immunology.

T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome

S. Prahalad, E. Waller, C. Bennett, 2021, Blood.

Hemophagocytic Lymphohistiocytosis: Clinical Presentations and Diagnosis.

K. Risma, R. Marsh, 2019, The journal of allergy and clinical immunology. In practice.

Reduced-intensity conditioning for hematopoietic cell transplant for HLH and primary immune deficiencies.

S. Pai, C. Bollard, M. Pulsipher, 2018, Blood.

Salvage Therapy and Allogeneic Hematopoietic Cell Transplantation for the Severe Cytokine Storm Syndrome of Hemophagocytic Lymphohistiocytosis

R. Marsh, 2019, Cytokine Storm Syndrome.

How i treat primary haemophagocytic lymphohistiocytosis

E. Haddad, R. Marsh, 2018, British journal of haematology.

Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab

A. Filipovich, J. Kanter, C. Allen, 2013, Pediatric blood & cancer.

Evaluation of Natural Killer (NK) Cell Defects

K. Risma, R. Marsh, 2016 .

A Prospective Study of Alemtuzumab as a Second-Line Agent for Steroid-Refractory Acute Graft-versus-Host Disease in Pediatric and Young Adult Allogeneic Hematopoietic Stem Cell Transplantation.

A. Vinks, K. Myers, S. Davies, 2016, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

The successful use of alemtuzumab for treatment of steroid‐refractory acute graft‐versus‐host disease in pediatric patients

K. Myers, S. Davies, P. Mehta, 2014, Pediatric transplantation.

Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders.

A. Filipovich, J. Villanueva, R. Marsh, 2011, Journal of immunological methods.

Impaired immune function in children and adults with Fanconi anemia

K. Myers, S. Davies, P. Mehta, 2017, Pediatric blood & cancer.

Clinical Flow Cytometric Screening of SAP and XIAP Expression Accurately Identifies Patients with SH2D1A and XIAP/BIRC4 Mutations.

Kejian Zhang, A. Filipovich, J. Villanueva, 2014, Cytometry. Part B, Clinical cytometry.

Epstein Barr virus-mediated transformation of B cells from XIAP-deficient patients leads to increased expression of the tumor suppressor CADM1

A. Grundhoff, C. Münz, J. Pachlopnik Schmid, 2022, Cell Death & Disease.

Prospective two center study of CD38 bright CD8+ effector memory T-cells as a predictor of acute GVHD

R. Garzon, Y. Efebera, S. Devine, 2022, Transplantation Reports.

Ruxolitinib as Salvage Therapy in Steroid-Refractory Acute Graft-versus-Host Disease in Pediatric Hematopoietic Stem Cell Transplant Patients.

K. Myers, S. Davies, S. Jodele, 2017, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Familial hemophagocytic lymphohistiocytosis and X‐linked lymphoproliferative disease

A. Filipovich, R. Marsh, 2011, Annals of the New York Academy of Sciences.

Leukocyte Adhesion Deficiency Type 1 Presenting with Recurrent Pyoderma Gangrenosum and Flaccid Scarring

A. Lucky, K. Bove, M. Passo, 2010, Pediatric dermatology.

Daratumumab for the management of autoimmune cytopenias in children and young adults: a case series

S. Davies, P. Mehta, M. Jordan, 2021, British journal of haematology.

Allogeneic hematopoietic cell transplantation for XIAP deficiency: an international survey reveals poor outcomes.

A. Fischer, H. Kanegane, P. Veys, 2013, Blood.

X-linked lymphoproliferative syndromes: brothers or distant cousins?

A. Snow, Kejian Zhang, A. Filipovich, 2010, Blood.

BCG-osis and Hematopoietic Cell Transplant for Primary Immunodeficiencies

S. Davies, Anthony Sabulski, R. Marsh, 2020, Journal of clinical immunology.

Reduced-Intensity/Reduced-Toxicity Conditioning Approaches Are Tolerated in XIAP Deficiency but Patients Fare Poorly with Acute GVHD

S. Choo, S. Ehl, A. Worth, 2021, Journal of clinical immunology.

Using flow cytometry to screen patients for X-linked lymphoproliferative disease due to SAP deficiency and XIAP deficiency.

A. Filipovich, R. Marsh, J. Bleesing, 2010, Journal of immunological methods.

Bortezomib for refractory autoimmunity in pediatrics.

S. Davies, S. Jodele, A. Filipovich, 2014, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Peripheral Blood CD38 Bright CD8+ Effector Memory T Cells Predict Acute Graft-versus-Host Disease.

S. Davies, D. Marmer, A. Filipovich, 2015, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Allogeneic hematopoietic stem cell transplant outcomes for patients with dominant negative IKZF1/IKAROS mutations.

S. Rosenzweig, A. Stray-Pedersen, D. Boutboul, 2019, The Journal of allergy and clinical immunology.

High-dose methylprednisolone for veno-occlusive disease of the liver in pediatric hematopoietic stem cell transplantation recipients.

K. Myers, S. Davies, S. Jodele, 2013, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

The Natural History of X-Linked Lymphoproliferative Disease (XLP1): Lessons from a Long-Term Survivor

J. Hajjar, Yike Jiang, M. Firan, 2020, Case reports in immunology.

HIF1A is a critical downstream mediator for hemophagocytic lymphohistiocytosis

Y. Hayashi, Xiaomei Yan, Lingyun Wu, 2017, Haematologica.

The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis.

B. Burkhardt, A. Schulz, S. Ehl, 2016, Blood.

Experience with a Reduced Toxicity Allogeneic Transplant Regimen for Non-CGD Primary Immune Deficiencies Requiring Myeloablation

S. Davies, M. Jordan, Ashish R. Kumar, 2020, Journal of Clinical Immunology.

Experience with a Reduced Toxicity Allogeneic Transplant Regimen for Non-CGD Primary Immune Deficiencies Requiring Myeloablation

S. Davies, M. Jordan, Ashish R. Kumar, 2020, Journal of clinical immunology.

Pretransplant Absolute Lymphocyte Counts Impact the Pharmacokinetics of Alemtuzumab.

A. Vinks, P. Mehta, T. Fukuda, 2017, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation.

S. Davies, P. Mehta, Mi‐Ok Kim, 2010, Blood.

Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience

K. Nichols, M. Jordan, Ashish R. Kumar, 2017, Pediatric blood & cancer.

Flow cytometric measurement of SLAM-associated protein and X-linked inhibitor of apoptosis.

A. Filipovich, R. Marsh, J. Bleesing, 2013, Methods in molecular biology.

Reduced‐intensity conditioning haematopoietic cell transplantation for haemophagocytic lymphohistiocytosis: an important step forward

A. Filipovich, M. Jordan, R. Marsh, 2011, British journal of haematology.

Frequency and spectrum of disease-causing variants in 1892 patients with suspected genetic HLH disorders.

Jianjun Chen, Miao Sun, Kejian Zhang, 2020, Blood advances.

Treatment dilemmas in asymptomatic children with primary hemophagocytic lymphohistiocytosis.

G. Lucchini, P. Veys, A. Worth, 2018, Blood.

STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America

N. Satake, Kejian Zhang, A. Filipovich, 2010, Pediatric blood & cancer.

Treatment of Newly Diagnosed HLH and Refractory Disease

J. Henter, R. Marsh, T. Greenwood, 2018 .

CD38 bright CD8+ T-cells Associated with the Development of Acute GVHD are Activated, Proliferating, and Cytotoxic Trafficking Cells.

C. Lutzko, S. Davies, M. Jordan, 2019, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Reduced Fertility Potential in Young Patients Following Hematopoietic Stem Cell Transplantation Despite Reduced Intensity Conditioning

K. Myers, S. Davies, S. Jodele, 2018 .

Reduced Intensity Conditioning Mitigates Risk for Primary Ovarian Insufficiency but Does Not Decrease Risk for Infertility in Pediatric and Young Adult Survivors of Hematopoietic Stem Cell Transplant: Gonadal Toxicity and Fertility Following Pediatric HSCT.

K. Myers, S. Jodele, C. Dandoy, 2022, Transplantation and cellular therapy.

Micafungin antifungal prophylaxis in children undergoing HSCT: can we give higher doses, less frequently? A pharmacokinetic study

A. Vinks, Kana Mizuno, S. Davies, 2018, The Journal of antimicrobial chemotherapy.

Alternate-day micafungin antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study.

A. Vinks, S. Davies, P. Mehta, 2010, Biology of Blood and Marrow Transplantation.

T cell dynamics and response of the microbiota after gene therapy to treat X-linked severe combined immunodeficiency

David A. Williams, Erik L. Clarke, F. Bushman, 2018, Genome Medicine.

Use of Genetic Testing for Primary Immunodeficiency Patients

J. Orange, J. Chou, T. Torgerson, 2018, Journal of Clinical Immunology.

Standardizing Definitions of Hematopoietic Recovery, Graft Rejection, Graft Failure, Poor Graft Function, and Donor Chimerism in Allogeneic Hematopoietic Cell Transplantation: A Report on Behalf of the American Society for Transplantation and Cellular Therapy.

M. Bar, J. Wagner, J. Sierra, 2021, Transplantation and cellular therapy.

Post-Transplant CD34+ Selected Stem Cell "Boost" for Mixed Chimerism after Reduced-Intensity Conditioning Hematopoietic Stem Cell Transplantation in Children and Young Adults with Primary Immune Deficiencies.

S. Davies, M. Jordan, P. Khandelwal, 2018, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Cutaneous infection with Metarhizium anisopliae in a patient with hypohidrotic ectodermal dysplasia and immune deficiency.

David A. Williams, J. Casanova, A. Puel, 2008, The Pediatric infectious disease journal.

Granulocyte Transfusions in Patients with Chronic Granulomatous Disease Undergoing Hematopoietic Cell Transplantation or Gene Therapy

L. Notarangelo, J. Puck, T. Torgerson, 2022, Journal of Clinical Immunology.

Nucleotide-binding oligomerization domain (NOD) signaling defects and cell death susceptibility cannot be uncoupled in X-linked inhibitor of apoptosis (XIAP)-driven inflammatory disease

G. Dubyak, Derek W. Abbott, Steven M. Chirieleison, 2017, The Journal of Biological Chemistry.

Perforin and CD107a testing is superior to NK cell function testing for screening patients for genetic HLH.

S. Choo, Kejian Zhang, A. Lane, 2017, Blood.

Reduced-intensity conditioning hematopoietic cell transplantation is an effective treatment for patients with SLAM-associated protein deficiency/X-linked lymphoproliferative disease type 1.

S. Davies, A. Filipovich, M. Jordan, 2014, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Clinical flow cytometric screening of SAP and XIAP expression accurately identifies patients with SH2D1A and XIAP/BIRC4 mutations

Kejian Zhang, A. Filipovich, J. Villanueva, 2014, Cytometry. Part B, Clinical cytometry.

Dominant‐negative IKZF1 mutations cause a T, B, and myeloid cell combined immunodeficiency

A. Fischer, H. Kanegane, C. Picard, 2018, The Journal of clinical investigation.

Diagnostic dilemmas in HLH: Can T‐cell phenotyping help?

R. Marsh, 2017, European journal of immunology.

Outcome of hematopoietic stem cell transplantation for adenosine deaminase-deficient severe combined immunodeficiency.

A. Fischer, W. Friedrich, L. Notarangelo, 2012, Blood.

Hemophagocytic Lymphohistiocytosis, Familial

Kejian Zhang, A. Filipovich, J. Villanueva, 2013 .

The transcription factor Bcl11b promotes both canonical and adaptive NK cell differentiation

R. Månsson, Joseph C. Sun, M. Shoukier, 2021, Science Immunology.

An intermediate alemtuzumab schedule reduces the incidence of mixed chimerism following reduced-intensity conditioning hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis.

K. Myers, S. Davies, P. Mehta, 2013, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Adenosine Deaminase (ADA)–Deficient Severe Combined Immune Deficiency (SCID) in the US Immunodeficiency Network (USIDNet) Registry

C. Cunningham-Rundles, J. Puck, K. Sullivan, 2020, Journal of Clinical Immunology.

Incidence and Outcomes of Central Nervous System Hemophagocytic Lymphohistiocytosis Relapse after Reduced-Intensity Conditioning Hematopoietic Stem Cell Transplantation.

S. Davies, M. Jordan, Ashish R. Kumar, 2017, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Elevated Granzyme B in Cytotoxic Lymphocytes is a Signature of Immune Activation in Hemophagocytic Lymphohistiocytosis

Kejian Zhang, A. Filipovich, J. Villanueva, 2013, Front. Immunol..

A Single-Center Experience Comparing Alemtuzumab, Fludarabine, and Melphalan Reduced-Intensity Conditioning with Myeloablative Busulfan, Cyclophosphamide, and Antithymocyte Globulin for Chronic Granulomatous Disease.

S. Davies, P. Khandelwal, R. Marsh, 2016, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey

L. Notarangelo, J. Walter, J. Puck, 2020, Frontiers in Immunology.

Peri-Transplant Alemtuzumab Levels IMPACT ACUTE Gvhd, MIXED Chimerism, and Lymphocyte Recovery Following Reduced Intensity Conditioning with Alemtuzumab, Fludarabine, and Melphalan

S. Davies, P. Mehta, S. Jodele, 2016 .

Alemtuzumab levels impact acute GVHD, mixed chimerism, and lymphocyte recovery following alemtuzumab, fludarabine, and melphalan RIC HCT.

S. Davies, P. Mehta, S. Jodele, 2016, Blood.

Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis

Michael M. Henry, S. Holland, E. Haddad, 2020, Journal of Clinical Immunology.

Chronic Granulomatous Disease-Associated IBD Resolves and Does Not Adversely Impact Survival Following Allogeneic HCT

L. Notarangelo, J. Puck, T. Torgerson, 2019, Journal of Clinical Immunology.

A Comparison of Hematopoietic Cell Transplant Conditioning Regimens for Hemophagocytic Lymphohistiocytosis Disorders.

Michael J. Eckrich, A. Gennery, T. Olson, 2021, The Journal of allergy and clinical immunology.

Reduced-Intensity/Reduced-Toxicity Conditioning Approaches Are Tolerated in XIAP Deficiency but Patients Fare Poorly with Acute GVHD

S. Choo, S. Ehl, A. Worth, 2021, Journal of Clinical Immunology.

Outcome of patients with NEMO deficiency following allogeneic hematopoietic cell transplant.

S. Holland, G. Uzel, K. Shah, 2017, The Journal of allergy and clinical immunology.

X-Linked Agammaglobulinemia: Infection Frequency and Infection-Related Mortality in the USIDNET Registry

C. Cunningham-Rundles, H. Ochs, K. Sullivan, 2022, Journal of Clinical Immunology.

Next-Generation Sequencing Based Clinical Molecular Diagnosis of Primary Immunodeficiency Diseases

Kejian Zhang, C. A. Valencia, Lijun Wang, 2017 .

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH.

J. Meller, P. S. Klein, Kejian Zhang, 2011, Blood.

A Toolkit and Framework for Optimal Laboratory Evaluation of Individuals with Suspected Primary Immunodeficiency.

Karin Chen, D. Vinh, M. Lawrence, 2021, The journal of allergy and clinical immunology. In practice.

with adult-onset familial hemophagocytic lymphohistiocytosis Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated

J. Meller, P. S. Klein, Kejian Zhang, 2012 .

Experience with Alemtuzumab, Fludarabine, and Melphalan Reduced-Intensity Conditioning Hematopoietic Cell Transplantation in Patients with Nonmalignant Diseases Reveals Good Outcomes and That the Risk of Mixed Chimerism Depends on Underlying Disease, Stem Cell Source, and Alemtuzumab Regimen.

K. Myers, S. Davies, P. Mehta, 2015, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Outcomes of donor lymphocyte infusion for treatment of mixed donor chimerism after a reduced-intensity preparative regimen for pediatric patients with nonmalignant diseases.

S. Davies, A. Filipovich, M. Jordan, 2015, Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation.

Accuracy of flow cytometric perforin screening for detecting patients with FHL due to PRF1 mutations.

S. Choo, Kejian Zhang, A. Filipovich, 2015, Blood.

Genetic Syndromes with Associated Immunodeficiencies

A. Lindsley, R. Marsh, 2021 .

Hypogammaglobulinemia with decreased class-switched B-cells and dysregulated T-follicular-helper cells in IPEX syndrome.

A. Joshi, L. Kobrynski, K. Patel, 2018, Clinical immunology.

Sustained elevation of serum interleukin-18 and its association with hemophagocytic lymphohistiocytosis in XIAP deficiency.

H. Kanegane, Y. Nakazawa, T. Imamura, 2014, Cytokine.

TNFR2 induced priming of the inflammasome leads to a RIPK1-dependent cell death in the absence of XIAP

M. Robinson, H. Wajant, T. Kaufmann, 2019, Cell Death & Disease.

Allogeneic Hematopoietic Cell Transplantation for Chronic Granulomatous Disease: Controversies and State of the Art

J. Talano, R. Marsh, J. Connelly, 2018, Journal of the Pediatric Infectious Diseases Society.

Outcomes following hematopoietic cell transplantation for Wiskott–Aldrich syndrome

S. Davies, P. Mehta, S. Jodele, 2012, Bone Marrow Transplantation.

Inborn Errors of Immunity Associated With Type 2 Inflammation in the USIDNET Registry

J. Puck, K. Sullivan, D. Dai, 2022, Frontiers in Immunology.

Tocilizumab for Treatment of Children and Young Adults With Severe Acute COVID-19: Experience at a Quaternary-care Children’s Hospital

Joshua D. Courter, Ranjit S. Chima, R. Frenck, 2022, The Pediatric infectious disease journal.

Ibrutinib for the treatment of chronic graft‐vs‐host disease in pediatric hematopoietic stem cell transplant patients: A single‐center experience

S. Davies, C. Dandoy, P. Khandelwal, 2020, Pediatric transplantation.

A First Report of Secondary Hemophagocytic Lymphohistiocytosis Associated With Papillary Thyroid Carcinoma.

M. Gelfand, P. Mehta, C. Wakefield, 2017, Journal of pediatric hematology/oncology.

Immune Effector Cell associated Hemophagocytic Lymphohistiocytosis-like Syndrome (IEC-HS).

Matthew J. Frigault, D. Teachey, M. Perales, 2023, Transplantation and cellular therapy.

α4β7 Integrin expression and blockade in pediatric and young adult gastrointestinal graft‐versus‐host disease

K. Myers, S. Davies, P. Mehta, 2021, Pediatric blood & cancer.

Thrombotic Microangiopathy Can Occur Before Transplant in Children with HLH

S. Davies, S. Jodele, M. Jordan, 2017 .

Test‐dose pharmacokinetics guided melphalan dose adjustment in reduced intensity conditioning allogeneic transplant for non‐malignant disorders

K. Setchell, A. Vinks, Kana Mizuno, 2021, British journal of clinical pharmacology.

Resolution of CGD Related Colitis after Allogeneic Hematopoietic Stem Cell Transplantation in Patients with Chronic Granulomatous Disease—Early Results From the 6903 Study of the Primary Immune Deficiency Treatment Consortium (PIDTC)

L. Notarangelo, J. Puck, K. Sullivan, 2018 .

Hemophagocytic lymphohistiocytosis in a female patient due to a heterozygous XIAP mutation and skewed X chromosome inactivation

S. Davies, Kejian Zhang, Jennifer R. Holle, 2015, Pediatric blood & cancer.

BCG-osis and Hematopoietic Cell Transplant for Primary Immunodeficiencies

Anthony Sabulski, R. Marsh, S. Chandra, 2020, Journal of Clinical Immunology.

Quercetin Ameliorates XIAP Deficiency Associated Hyperinflammation.

S. Davies, P. Mehta, C. Booth, 2022, Blood.

Outcomes Following Hematopoietic Cell Transplantation for Wiskott Aldrich Syndrome

P. Mehta, Mi‐Ok Kim, S. Jodele, 2011 .

Model‐informed precision dosing for alemtuzumab in paediatric and young adult patients undergoing allogeneic haematopoietic cell transplantation

A. Vinks, P. Mehta, T. Fukuda, 2021, British journal of clinical pharmacology.

Antibody deficiency testing for primary immunodeficiency

J. Orange, R. Marsh, 2019, Annals of Allergy, Asthma & Immunology.

Antibody Deficiency Testing for Primary Immune Deficiency - A Practical Review for the Clinician.

J. Orange, R. Marsh, 2019, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

CD38 Bright CD8+ TEM Cells Detected Prior to Acute Gvhd are Activated, Cytotoxic, Proliferating, Trafficking Cells Which are Not Viral Specific

S. Davies, P. Khandelwal, R. Marsh, 2017 .

Abstracts From the 32nd Annual Meeting of the Histiocyte Society Dublin, Ireland October 17-19, 2016.

Pooja, Wright, Tracey, 2016, Pediatric Blood & Cancer.

Excessive IL-10 and IL-18 Trigger HLH-Like Hyperinflammation and Enhanced Myelopoiesis.

D. Witte, Xiaomei Yan, G. Huang, 2022, Journal of Allergy and Clinical Immunology.

Cutaneous T-cell lymphoma as a unique presenting malignancy in X-linked magnesium defect with EBV infection and neoplasia (XMEN) disease.

S. Chiang, J. Jacobsen, B. Wright, 2021, Clinical immunology.

Does Shining a Spotlight on XIAP Deficiency Bring the Role of Allogeneic HCT into Better Focus?

R. Marsh, 2022, Journal of Allergy and Clinical Immunology.

The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS)

J. Carcillo, L. Henderson, K. Nichols, 2023, Annals of the Rheumatic Diseases.

Pediatric Phase II Study of Maraviroc for Acute Graft Versus Host Disease Prophylaxis

A. Vinks, K. Myers, S. Davies, 2019, Biology of Blood and Marrow Transplantation.

Histiocytic disorders

M. Merad, K. Ribeiro, J. Haroche, 2021, Nature Reviews Disease Primers.

A prospective pilot study of a novel alemtuzumab target concentration intervention strategy

A. Vinks, K. Myers, S. Davies, 2021, Bone Marrow Transplantation.

Practice pattern changes and improvements in hematopoietic cell transplantation for primary immunodeficiencies.

Michael J. Eckrich, N. Kapoor, C. Dvorak, 2018, The Journal of allergy and clinical immunology.

Lymphoproliferative Disease, X-Linked

Kejian Zhang, R. Marsh, E. Wakefield, 2016 .

Relationship Between Severity of T Cell Lymphopenia and Immune Dysregulation in Patients with DiGeorge Syndrome (22q11.2 Deletions and/or Related TBX1 Mutations): a USIDNET Study

J. Orange, K. Sullivan, R. Marsh, 2020, Journal of clinical immunology.

Relationship Between Severity of T Cell Lymphopenia and Immune Dysregulation in Patients with DiGeorge Syndrome (22q11.2 Deletions and/or Related TBX1 Mutations): a USIDNET Study

J. Orange, K. Sullivan, R. Marsh, 2020, Journal of Clinical Immunology.

Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency.

K. Nichols, M. Lenardo, A. Snow, 2009, The Journal of clinical investigation.

Adolescents and Young Adults With Hemophagocytic Lymphohistiocytosis Who Undergo Allogeneic Hematopoietic Cell Transplantation Are At Increased Risk Of Mortality Compared To Younger Patients

A. Filipovich, M. Jordan, S. Chandrakasan, 2013 .

Outcome of Hematopoietic Cell Transplant for Hemophagocytic Lymphohistiocytosis in Adults: A Single Center Experience

S. Davies, A. Filipovich, M. Jordan, 2013 .

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH.

J. Meller, P. S. Klein, Kejian Zhang, 2011, Blood.

Preemptive hematopoietic cell transplantation for asymptomatic patients with X-linked lymphoproliferative syndrome type 1.

H. Kanegane, T. Morio, A. Gennery, 2022, Clinical immunology.

The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS)

R. Cron, D. Aletaha, S. Vastert, 2023, Arthritis & rheumatology.

Use of Genetic Testing for Primary Immunodeficiency Patients

J. Orange, J. Chou, T. Torgerson, 2018, Journal of Clinical Immunology.

COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

G. Giardino, C. Pignata, J. Puck, 2024, Journal of clinical immunology.

Neutropenia Is an Underrecognized Finding in Pediatric Primary Immunodeficiency Diseases: An Analysis of the United States Immunodeficiency Network Registry

D. Dale, E. Garabedian, K. Walkovich, 2020, Journal of pediatric hematology/oncology.

Single Site Experience of the use of Monoclonal Antibodies for the Treatment of COVID-19 in High-risk Pediatric and Young Adult Patients

Joshua D. Courter, R. Frenck, P. Spearman, 2022, The Pediatric infectious disease journal.