Attenuated form of Evans syndrome among pediatric ITP patients.
Long term follow up of adult patients with immune thrombocytopenic purpura (ITP) have shown evolvement of secondary autoimmune diseases such as SLE, Evans syndrome, autoimmune neutropenia, Graves disease etc. We studied 30 cases of pediatric ITP patients for evidence of hemolysis to assess the possibility of Evans like syndrome. Measurement of free serum haptoglobin, a sensitive indicator of red cell destruction was used after careful exclusion of micro angiopathic hemolysis, SLE or overt Evans Syndrome. Results showed abnormally low level of free serum haptoglobin in 11 of the 30 (36.7%) patients compared to that in 20 age matched controls (P < 0.001) as an evidence of hemolysis. Our data in pediatric patients is similar to that reported in adult ITP cases and support the observation of Evans made 50 years ago that there is a spectrum like relationship between primary thrombocytopenia and hemolytic anemia. Thus the concept of attenuated form of Evans syndrome could be considered, in group of patients with ITP in pediatric age group.
Dash
Sumitra
S
Department of Hematolog, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India. sumitradash2001@yahoo.com
Marwaha
Ram
R
Trehan
Amita
A
D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood* / Q000150:complications / Q000473:pathology; D015415:Biomarkers / Q000097:blood; D001854:Bone Marrow Cells / Q000378:metabolism; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006242:Haptoglobins / Q000378:metabolism; D006454:Hemoglobins / Q000378:metabolism; D006461:Hemolysis; D006801:Humans; D007223:Infant; D008297:Male; D008533:Megakaryocytes / Q000378:metabolism; D010976:Platelet Count; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood* / Q000150:complications / Q000473:pathology; D013577:Syndrome
D015415:Biomarkers; D006242:Haptoglobins; D006454:Hemoglobins