Adenosine Deaminase Type II Deficiency: Severe Chronic Neutropenia, Lymphoid Infiltration in Bone Marrow, and Inflammatory Features


SÜLEYMAN M. , TAN Ç. , ÜNER A. , Inkaya C. , Aytac S., BÜYÜKAŞIK Y. , ...Daha Fazla

IMMUNOLOGICAL INVESTIGATIONS, 2020 (SCI İndekslerine Giren Dergi) identifier identifier

Özet

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.