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Elevated red cell adenosine deaminase activity: a marker of disordered erythropoiesis in Diamond‐Blackfan anaemia and other haematologic diseases
Author(s) -
Glader Bertil E.,
Backer Karen
Publication year - 1988
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1988.tb06184.x
Subject(s) - erythropoiesis , adenosine deaminase , red cell , diamond–blackfan anemia , bone marrow , population , medicine , immunology , endocrinology , anemia , biology , adenosine , biochemistry , ribosome , rna , environmental health , gene
Summary Red‐cell adenosine deaminase (ADA) activity in children with Diamond‐Blackfan anaemia is significantly increased (1·91±0·90 U/g Hb) compared to that seen in transient erythroblastopenia of childhood (0·80±0·16 U/g Hb) or normal individuals (0·61±0·13 U/g). These data thus further support that measurement of this purine metabolic enzyme is useful in diagnosing the cause of pure RBC aplasia in children. Of interest, however, elevated RBC‐ADA activity also is seen in some children with acute leukaemia and other haematologic disorders. In children with acute lymphoblastic leukaemia (ALL), the increase in RBC‐ADA activity is proportional to the degree of anaemia. However, the elevated RBC‐ADA activity in this leukaemic population is not related to the fetal haemoglobin concentration. These data suggest increased RBC‐ADA activity may be a non‐specific manifestation of abnormal erythroid stem cell function, an alteration distinct from that seen with reactivation of fetal erythropoiesis. However, since almost all patients with Diamond‐Blackfan anaemia manifest elevated RBC‐ADA activity, this chemical alteration yet may reflect the specific erythroid differentiation lesion in this disorder.