Publications by authors named "J A Kanakry"

The therapeutic efficacy of donor lymphocyte infusions (DLIs) given after allogeneic hematopoietic cell transplantation (HCT) is limited by risk of graft-versus-host disease (GVHD). Post-transplantation cyclophosphamide (PTCy) effectively prevents severe GVHD, but there are limited data on outcomes of DLIs given to PTCy-treated patients. We reviewed 162 consecutive PTCy-treated patients transplanted between 2015-2022 within the Center for Immuno-Oncology at the National Cancer Institute.

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IKAROS, encoded by IKZF1, is a tumor suppressor and a key hematopoietic transcription factor responsible for lymphoid and myeloid differentiation. IKZF1 mutations result in inborn errors of immunity presenting with increased susceptibility to infections, immune dysregulation, and malignancies. In particular, patients carrying IKZF1 gain-of-function (GOF) mutations mostly exhibit symptoms of immune dysregulation and polyclonal plasma cell proliferation.

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Article Synopsis
  • The study investigates how RAG deficiency, a genetic immune disorder, impacts the microbiome in patients, analyzing samples from skin, nares, and gut.
  • It reveals that RAG-deficient individuals show a diverse range of unique bacteria, including the growth of harmful pathogens, and a reduced specificity of microbiota across different body sites compared to healthy people.
  • Additionally, the research highlights that these patients may serve as reservoirs for long-lasting viral infections, documenting the presence of eukaryotic RNA viruses like Coronavirus 229E and Norovirus GII in their microbiomes.
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Article Synopsis
  • DADA2 is a recessive disease leading to issues like systemic vasculitis and early-onset stroke, affecting both kids and adults, with over 35,000 cases globally and no current management guidelines.
  • The DADA2 Consensus Committee, including patient representatives and experts from 18 countries, created 32 consensus statements to improve diagnostic testing, screening, and treatment based on patient symptoms.
  • Early diagnosis and treatment of DADA2 are crucial, and these consensus statements aim to provide a structured approach for doctors in evaluating and managing the disease.
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