Adenosine Deaminase Type II Deficiency: Severe Chronic Neutropenia, Lymphoid Infiltration in Bone Marrow, and Inflammatory Features. (3rd April 2022)

• Record Type: Journal Article
• Authors: Süleyman, Merve; Tan, Çağman; Uner, Aysegul; İnkaya, Çağkan; Aytaç, Selin; Büyükaşık, Yahya; Boztug, Kaan; Tezcan, İlhan; Cagdas, Deniz
• Is Part Of: Immunological investigations. Volume 51:Number 3(2022)
• Page Start: 558
• Page End: 566
• Publication Date: 2022-04-03
• Subjects: Deficiency of adenosine deaminase 2 -- chronic neutropenia -- autoinflammatory disease; Immunology -- Periodicals; Immunochemistry -- Periodicals; Cellular immunity -- Periodicals; Communicable diseases -- Periodicals; 616.079
• DOI: 10.1080/08820139.2020.1853153
• Languages: English
• ISSNs: 0882-0139

Abstract

ABSTRACT: Deficiency of adenosine deaminase type 2 (DADA2) is an autoinflammatory disease characterized with immunologic, hematologic, and neurological features. Here, we presented two patients with severe persistent chronic neutropenia, which required differential diagnosis of congenital and autoimmune neutropenia, myelodysplastic syndrome (MDS), and primary immunodeficiency diseases, including autoimmune lymphoproliferative disease. The therapy of the disease except hematopoietic stem cell transplantation is a challenging experience.