Dr.Sruthi Suresh, Dr.Sathyajith G Nair, Dr.C.Jayakumar
Department of Paediatrics
AIMS, KOCHI
Two year old male child developmentally normal and immunised for age, who was apparently asymptomatic until 1 week back presented with complaints of high grade intermittent fever of 1 week associated with vomiting, cola coloured urine and yellowish discolouration of skin of 1 day duration.
Outside hospital blood investigations showed low haemoglobin level (6.5g/dl) with indirect hyperbilirubinemia and abnormal RFT. In view of the same he was referred to AIMS for further evaluation and management.
On examination he was febrile and irritable. Vitals: Temp – 100 ‘F; PR-120/min; RR – 26/min; BP – 98/60 mmHg, SpO2: 98% in RA, CRT < 2 sec. He had pallor and icterus. No lymphadenopathy. Systemic examination was unremarkable and did not reveal hepatosplenomegaly.
Blood investigations showed normal counts, haemoglobin ofof 5.4g/dl with elevated inflammatory markers (CRP-111mg/L), altered renal function test (S. Urea-120mg/dl, S.Creatinine: 1.78mg/dl) and indirect hyperbilirubinemia with elevated LDH (3424 U/L). S. Potassium was also elevated(5meq/L). Peripheral smear showed spherocytic blood picture.
DCT done was positive (4+).
USG abdomen showed bilateral kidneys with raised cortical echogenicity with maintained corticomedullary differentiation. A diagnosis of autoimmune hemolytic anemia was thus made. He was admitted to PICU for further management. He was started on Inj Ceftriaxone and Azithromycin. He was also started on Inj.Dexamethasone, which was given for 4 days and switched to oral prednisolone. One unit of PRBC transfusion was also given. In view of elevated Uric acid level, allopurinol was started. In view of deranged RFT and hyperkalemia, adequate hydration was given and hyperkalemia was corrected as per protocol. Child improved with treatment given and was shifted out of PICU on day 3 of admission.
As part of Workup of AIHA, IgM EBV sent was positive, IgM Mycoplasma sent was negative. Respiratory viral panel was positive for human rhinovirus/human enterovirus. C3 was normal and C4 was reduced. ANA (IFA) sent was normal. Cold antibodies in serum were negative. Child improved with treatment given. Blood investigations showed improving trend. He was planned to be continued on oral steroids at 2mg/kg/day for 2 weeks followed tapering dose of steroids. At the time of discharge he had a haemoglobin level of 9.5g/dl, Urea 45.5mg/dl and Creatinine of 0.63mg/dl. He was discharged with plan to repeat complete blood count and GRBS every 3 days and to review after 2 weeks or earlier if haemoglobin is showing decreasing trend.
Autoimmune hemolytic anemia (AIHA) is a condition characterized the premature destruction of red blood cells due to an immune response targeting them. It can be classified into warm and cold AIHA, with the former being more common and associated with various underlying conditions, such as infections (e.g., Epstein-Barr virus), autoimmune diseases, and certain medications. Clinically, patients may present with symptoms like fatigue, pallor, jaundice, dark-colored urine, and splenomegaly. Diagnosis is confirmed through laboratory tests, including a complete blood count, peripheral blood smear, and direct Coombs test. Management typically involves corticosteroids to suppress the immune response, along with supportive measures like blood transfusions in severe cases. The prognosis varies based on the underlying cause and response to treatment, emphasizing the importance of regular follow-up for potential relapses.Early recognition and prompt management of AIHA are crucial in paediatric patients to prevent complications and improve outcomes.