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What is Which of the following disease is associated with adenosine deaminase deficiency? and explain in detail?
Adenosine deaminase deficiency is associated with the development of severe combined immunodeficiency. Adenosine deaminase enzyme conves adenosine and deoxyadenosine into inosine and deoxyinosine. In the absence of ADA, high intracellular levels of adenosine, deoxyadenosine, and their toxic phosphorylated metabolites cause apoptosis of lymphoid precursors, resulting in the viual absence of T lymphocytes, that is usually associated with marked reduction of B and NK lymphocytes. Ref: Williams Hematology, 8th Edition, Chapter 82; Harrisons Internal Medicine, 18th Edition, Chapter 316