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A Case Report of a 14-year-old Boy with Mixed-type Autoimmune Hemolytic Anemia
Sudesh Kumar1, Avinash Priyadarshi2, Supriya Kumari1
1Department of Pediatrics, MGM Medical College and LSK Hospital, Kishanganj, Bihar, India.
Abstract:
Autoimmune hemolytic anemia (AIHA) is uncommon in the pediatric population, particularly when it manifests as severe anemia. AIHA is characterized by a positive direct antiglobulin test (DAT) and immune-mediated red blood cell (RBC) destruction. AIHA is subclassified on the basis of the thermal characteristics of autoantibody into warm, cold, and mixed. Mixed AIHA shows both the common types and characteristics of warm and cold types. A 14-year-old male, born of nonconsanguineous marriage, admitted with complaints of dizziness and hematuria. It was not associated with decreased urine output and abdominal pain. The child had a similar type of history 2 years back for that, he was treated with a 3-unit-packed RBC transfusion. On examination marked pallor, icterus and mild splenomegaly were present. Diagnosis of mixed AIHA was done on the basis of a DAT and was 4+ positive against Ig G and C3d. In cold, an agglutination test was done which was > 1:64 in titer. In peripheral blood smear, at below 37°C, it denoted the clumping of RBC with polychromasia, which is not reversed at room temperature. The child was treated with broad spectrum antibiotics, multiple-packed RBC transfusion, and injection methyl prednisolone. In follow-up, the child's clinically improved but DAT for immunoglobulin G remained positive and prednisolone was tapered to a maintenance dose of 0.5 mg/kg on alternate days. Mixed AIHA in pediatrics is an extremely rare disease, especially when presenting with severe anemia. It is very difficult to diagnose and treat. Hence, detailed clinical and extensive laboratory workup is required to diagnose the case. Clinical presentation of mixed AIHA, other than acute hemolysis, may manifest as blood group cross-match incompatibility, which is challenging for pathologists and awareness of this occurrence is essential for clinicians. A case of mixed AIHA should be treated with steroids immediately along with supportive care of packed RBC transfusion with the least incompatibility and long-term follow-up is required to improve outcome.
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