Coombs‐positive Autoimmune Haemolytic Anemia in Ulcerative Colitis: Spectrum of Same Disease?

Abstract

Hae Ulcerative Colitis (UC) is known to be associated with autoimmune haemolytic anaemia (AIHA). This rare complication has been described in 0.2 to 0.7% of adult patients affected by UC. 1.82% of patients with UC have a positive direct Coombs test without evidence of haemolysis. The aetiology is not completely understood; colon seems to play a role in the production of anti-erythrocytes antibodies and sometimes colectomy is necessary to resolve refractory haemolisys in UC. We report a case of Coombs-positive haemolytic anemia (AIHA) refractory to steroids and immunoglobulin infusions (IVIG) in a child with severe UC. Case report: a 7-year-old patient with UC followed at GI Unit of Meyer Children Hospital of Florence, was admitted to our Clinic because of jaundice. He had a history of severe steroid-refractory UC which had been controlled with cyclosporine and azathioprine. At time of admission UC was in remission on azathioprine and mesalazine although mild intestinal bleeding was occasionally present. Examination revealed pallor, skin and scleral icterus, tachycardia, palpable splenic tip; laboratory tests revealed Coombs-positive haemolytic anemia. IVIG (1 gr/kg/day for 3 days) and oral prednisone (2 mg/kg/day) were prescribed with clinical improvement. The child was discharged with oral steroids and azathioprine. Four weeks later, while on oral steroids (1 mg/kg/day), the child had again haemolytic crisis refractory to IVIG (1 gr/kg/day for 3 days) and metylpredisone pulses. Colonscopy revealed active mucosal lesions. Cyclosporine was then introduced orally. On this treatment both AIHA and UC went in remission. To our knowledge this is the first reported case of the association of AIHA and UC in childhood. In our case the clinical course of AIHA and treatment response requiring immunosuppressive therapy seemed to be dependent on UC activity. Presumably the colon displays a role in the production of anti-erythrocyte antibodies.