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

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 neutr...

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Bibliographic Details
Published inImmunological investigations Vol. 51; no. 3; pp. 558 - 566
Main Authors Süleyman, Merve, Tan, Çağman, Uner, Aysegul, İnkaya, Çağkan, Aytaç, Selin, Büyükaşık, Yahya, Boztug, Kaan, Tezcan, İlhan, Cagdas, Deniz
Format Journal Article
LanguageEnglish
Published England Taylor & Francis 03.04.2022
Subjects
Adenosine Deaminase
autoinflammatory disease
Bone Marrow
chronic neutropenia
Deficiency of adenosine deaminase 2
Humans
Intercellular Signaling Peptides and Proteins
Neutropenia - diagnosis
Severe Combined Immunodeficiency - diagnosis
Deficiency of adenosine deaminase 2
autoinflammatory disease
chronic neutropenia
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Summary: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.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:0882-0139
1532-4311
DOI:10.1080/08820139.2020.1853153