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A rare case of Coombs negative autoimmune haemolytic anaemia in a young male
Poster Title: A rare case of Coombs negative autoimmune haemolytic anaemia in a young male
Submitted on 17 Sep 2022
Author(s): Pallaavi Goel, Naveen Gupta, Puneet Rijhwani
Affiliations: Mahatma Gandhi Hospital, Jaipur
This poster was presented at 4th haematology update at AIIMS Jodhpur
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Abstract: Autoimmune Haemolytic Anaemia (AIHA) is an uncommon disorder with an estimated incidence of 0.8-3 per 100,000/year and a prevalence of 17:100,000 in the adult population. Coombs negative AIHA is characterized by laboratory evidence of hemolysis plus a negative Coombs test. We present a case of a 31-year-old hypertensive male, who presented with fever, red coloured urine, jaundice and fatigue for 3-5 days. The laboratory findings revealed severe anaemia with signs suggestive of hemolysis. A direct Coombs test and indirect Coombs test was negative, and a diagnosis of Coombs-negative autoimmune hemolytic anemia was made based on his clinical profile. He was treated with steroid pulse therapy, but showed no response, hence was shifted to second line treatment of IVIG therapy and Rituximab. Patient required multiple blood transfusions due to persistent low haemoglobin levels and ongoing haemoglobinuria. Patient is currently improving and is still admitted under observation. Early diagnosis and prompt decision on therapy is needed for proper treatment of such difficult cases.Summary: High degree of clinical suspicion of autoimmune hemolytic anemia should be made in patients presenting with acute hemolysis and should be diagnosed after exclusion of secondary causes by appropriate investigations including Coombs test. Negative coombs does not rule out possibility of AIHA and in such cases compatible clinical course helps in diagnosis of AIHA.References: 1.Ajaya kumar Dhakal, Devendra Shrestha et al,Coomb’s Test Negative Autoimmune Hemolytic Anemia, MJSBH July-December 2013|Vol 12| Issue 2
2.Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. American Journal of Hematology. 2002;69(4):258-71.
3.Aladjidi N, Leverger G, Leblanc T, Picat MQ, Michel G, Bertrand Y, et al. New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children. Haematologica. 2011;96(5):655-63.
4.Telio D, Pi D, Zalunardo N, Tucker LB, Chen LY. Atypical autoimmune hemolytic anemia. Haematologica. 2011;96(11):e43.
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