autoimmune hemolytic anemia ( aiha ) is one of the infrequent , but an important causative factor of anemia in infants , affecting one in 80,000 live births . complications including fatal severe hemolysis , acute renal failure are infrequently seen with fulminant cases , which is often refractory to conventional treatment with immunosuppressants . we report a case of aiha in a 4-month - old infant who presented with severe anemia , raised reticulocyte count , positive coombs test along with smear evidence of hemolysis . he has been treated with intravenous immunoglobulins ( iv igs ) , packed red cell transfusion and steroids . he had ill - sustained response to conventional therapy ; hence , he received rituximab , a chimeric monoclonal antibody . a 4-month - old male infant , born to non - consanguineous parents , with uneventful perinatal history and thriving well on breast milk , presented with cough for 10 days and pallor for 2 days . infant was pale with mild icterus , without any fever and any significant lymphadenopathy or organomegaly . hemoglobin ( hb ) , reticulocyte counts were 3.2 g / dl and 6% respectively . liver function test was normal , but with indirect hyperbilirubinemia ( 2.5 mg / dl out of 2.9 mg / dl of total bilirubin ) . high performance liquid chromatography was normal with hb f being elevated as per age . in urine microscopy , no fresh red blood cell ( rbc ) but hb level increased to 7.9 g% after packed rbc transfusion ( 15 ml / kg ) . however , his repeat hb 24 h later dropped to 5.8 g% with agglutination in the smear and repeat dct being again positive ( 4 + ) . immunoglobulin g ( igg ) auto antibodies were high and diagnosis of aiha further confirmed . he was started on iv ig ( 2 g / kg / total ) and on methylprednisolone ( 10 mg / kg / day ) . as hb improved to 9 g% after 4 days , he was discharged with advice to continue oral prednisolone ( 2 mg / kg ) . while tapering steroids , his hb dropped to 6.7 g% , with repeat dct being positive and with evidence of hemolysis in smear . a repeat course of iv igs ( 1 g / kg ) along with stepping up of prednisolone to 1 mg / kg / day was given as parents were not consenting for administration of rituximab . there was a transient improvement in hb to 10 g% , which has dropped later to 7.8 g% on tapering steroids . he was started on rituximab as response to steroids and iv ig was ill sustained , after obtaining the parent 's consent . dose was calculated as per the body weight ( by taking the standard recommendation of 375 mg / m once a week for 4 weeks ) and 85 mg given once a week . seven days after the 3 dose , good hematological response ( hb > 10.5 g% , reticulocytes 3% ) was observed . though the patient was covered prophylactically by co - trimoxazole after 3 dose of rituximab he developed severe pneumocystis carinii pneumonia ( pcp ) . the pcp infection was thought to be secondary to immunosuppressive status because of on - going steroid treatment aggravated by rituximab . after rituximab treatment , steroids were tapered over a month and continued at a very low dose ( 0.2 mg kg / day initially and on alternate day later ) for another 2 months as per the hematological picture . currently , he is off steroids for 6 months from last rituximab dose and maintaining a hb of 9 - 10 g% . hemolytic anemia in early infancy is because of either blood group incompatibility or hereditary red cell morphological disorders such as congenital spherocytosis , elliptocytosis or in certain mediterranean and asian regions it may be due to red cell enzyme abnormalities , including glucose-6-phosphate dehydrogenase deficiency . it is one of the rare clinical conditions in young infants with significant mortality and morbidity rate up to 10% in children . iv igs and steroids for immunosuppression are the mainstay of treatment other than support with red cell transfusion where it is possible to get at least near match compatible blood for red cell transfusion . rituximab , a chimeric anti cd20 monoclonal antibody , has shown to arrest the progress of hemolytic process , through its inhibitory action on b lymphocytes . hence , it is considered in the management of refractory aiha , with variable success rate . zecca et al . have reported a success rate of 87% ( 13/15 patients ) . there are few case reports of rituximab usage in infants with aiha worldwide , yadav and chinnabhandar have reported two cases of refractory aiha treated with rituximab from india . results of laboratory investigations ( positive dct , negative ana and retrovirus , highly positive igg ) suggested that this was a case of primary ( idiopathic ) aiha . we observed a remitting , relapsing course of hemolytic anemia to conventional agents like iv igs and steroids in our patient . he responded promptly to rituximab in terms of maintaining near normal levels of hb for 9 months from the last dose of rituximab . he tolerated the drug well except that he developed infection with pcp , which has responded to high dose co - trimoxazole . it could be due to the effect of corticosteroid immunosuppression and the depletion of circulating b lymphocytes with rituximab . our patient did not require further blood transfusion and the requirement for steroids reduced post rituximab therapy . use of rituximab results in less exposure to steroids and other immunosuppressants in young children and this may replace or shorten the duration of conventional immunosuppressive treatment in the future . pediatricians should weigh the risk and benefits before considering rituximab as a therapeutic option in treatment resistant cases .
autoimmune hemolytic anemia ( aiha ) is an immune mediated destruction of erythrocytes , which has a good prognosis in children . 
 it is known to have chronic , remitting or relapsing course , especially in infants and adolescents . 
 treatment of refractory or relapsing aiha is a challenge as the other aim of the treatment is to avoid prolonged exposure to steroids or other immunosuppressants in small children . 
 rituximab is used in patients who are non - responsive to conventional treatment such as steroids , intravenous immunoglobulins and transfusion therapy . 
 it has varying therapeutic success rate . 
 we report a case of aiha in a 4-month - old infant who had ill - sustained response to conventional therapy , but responded to rituximab .