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Ataxia-Telangiectasia and Refractory Peripheral T-cell Lymphoma: Considerations for Therapy and Role of Bone Marrow Transplantation
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  • Saara Kaviany,
  • Carrie Kitko,
  • Cesar Rueda,
  • Roshini Abraham,
  • Debra Friedman,
  • Sarah Neumann,
  • Becky Manes,
  • Sara Zarnegar-Lumley,
  • James Connelly
Saara Kaviany
Monroe Carell Junior Children's Hospital at Vanderbilt
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Carrie Kitko
Monroe Carell Junior Children's Hospital at Vanderbilt
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Cesar Rueda
Nationwide Children's Hospital
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Roshini Abraham
Nationwide Children's Hospital
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Debra Friedman
Monroe Carell Junior Children's Hospital at Vanderbilt
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Sarah Neumann
Monroe Carell Junior Children's Hospital at Vanderbilt
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Becky Manes
Monroe Carell Junior Children's Hospital at Vanderbilt
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Sara Zarnegar-Lumley
Monroe Carell Junior Children's Hospital at Vanderbilt
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James Connelly
Monroe Carell Junior Children's Hospital at Vanderbilt
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Abstract

Certain patients with inborn errors of immunity have defects in DNA damage response, predisposing them to malignancy. Subsequent cancer therapy may require substantial attenuation given defective DNA repair; however, this carries risk of incomplete disease control. We describe a 5-year-old boy with peripheral T-Cell lymphoma with ataxia-telangiectasia (A-T). After incomplete chemotherapeutic response, he underwent allogenic hematopoietic cell transplantation (allo-HCT) with an attenuated preparative regimen, but developed graft rejection and relapse. Following remission with salvage chemotherapy, second allo-HCT with reduced intensity conditioning (RIC) resulted in minimal toxicity and short-term disease control. HCT with RIC can be considered in patients with A-T.