Hematologic Manifestations of Deficiency of Adenosine Deaminase 2 (DADA2) and Response to Tumor Necrosis Factor Inhibition in DADA2-Associated Bone Marrow Failure

J Clin Immunol. 2018 Feb;38(2):166-173. doi: 10.1007/s10875-018-0480-4. Epub 2018 Feb 6.

Authors

Thomas F Michniacki¹, Mark Hannibal², Charles W Ross³, David G Frame⁴, Adam S DuVall⁵, Rami Khoriaty⁶, Mark T Vander Lugt⁷, Kelly J Walkovich⁷

Affiliations

¹ Pediatrics and Communicable Diseases, Pediatric Hematology/Oncology, University of Michigan, 1500 E. Medical Center Drive, D4202 Medical Professional Building, Ann Arbor, MI, 48109, USA. tmich@med.umich.edu.
² Pediatrics - Genetics, University of Michigan, Ann Arbor, MI, USA.
³ Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
⁴ Department of Pharmacy, University of Michigan, Ann Arbor, MI, USA.
⁵ Pediatric Hematology/Oncology, Oregon Health Sciences University, Portland, OR, USA.
⁶ Department of Internal Medicine, Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA.
⁷ Pediatrics and Communicable Diseases, Pediatric Hematology/Oncology, University of Michigan, 1500 E. Medical Center Drive, D4202 Medical Professional Building, Ann Arbor, MI, 48109, USA.

PMID: 29411230
DOI: 10.1007/s10875-018-0480-4

No abstract available

Publication types

Case Reports
Letter

MeSH terms

Adenosine Deaminase / deficiency*
Anemia, Aplastic / diagnosis
Anemia, Aplastic / drug therapy*
Anemia, Aplastic / etiology*
Biomarkers
Biopsy
Bone Marrow / pathology
Bone Marrow Diseases / diagnosis
Bone Marrow Diseases / drug therapy*
Bone Marrow Diseases / etiology*
Bone Marrow Failure Disorders
Hematologic Tests
Hemoglobinuria, Paroxysmal / diagnosis
Hemoglobinuria, Paroxysmal / drug therapy*
Hemoglobinuria, Paroxysmal / etiology*
Humans
Immunosuppressive Agents / therapeutic use
Intercellular Signaling Peptides and Proteins / deficiency*
Male
Middle Aged
Severe Combined Immunodeficiency / diagnosis
Severe Combined Immunodeficiency / etiology
Symptom Assessment
Treatment Outcome
Tumor Necrosis Factor-alpha / therapeutic use*

Substances

Biomarkers
Immunosuppressive Agents
Intercellular Signaling Peptides and Proteins
Tumor Necrosis Factor-alpha
ADA2 protein, human
Adenosine Deaminase