Kasi Bharathi Thatikonda1, Manas Kalra1, Arun Danewa1, Pallavi Sachdeva1, Tanusree Paul1, Divij Sachdeva1, Anupam Sachdeva2. 1. Department of Pediatric Hematology Oncology and BMT, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi. 2. Department of Pediatric Hematology Oncology and BMT, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi. Correspondence to: Dr Anupam Sachdeva, Director Pediatric Hematology Oncology and BMT, Institute for Child Health, Sir Ganga Ram Hospital, New Delhi. anupamace@yahoo.co.in.
Abstract
OBJECTIVE: To analyze clinical and laboratory parameters, and treatment outcomes of children with autoimmune hemolytic anemia (AIHA). METHODS: Retrospective analysis of 50 children aged 0-18 years. Monospecific direct antiglobulin test (DAT) and investigations for secondary causes were performed. Disease status was categorized based on Cerevance criteria. RESULTS: Median (range) age at diagnosis was 36 (1.5-204) months. AIHA was categorized as cold (IgM+,C3+/cold agglutinin+) (35%), warm (IgG+ with/without C3+) (28%), mixed (IgG+, IgM+, C3+) (15%) and paroxysmal cold hemoglobinuria (4%). Primary AIHA accounted for 64% cases. Treatment modalities included steroid (66%), intravenous immunoglobulin (IVIg) (4%), steroid+IVIg (4%), and steroid+rituximab (4%). Treatment duration was longer for secondary AIHA than primary (11 vs 6.6 months, P<0.02) and in patients needing polytherapy than steroids only (13.3 vs 7.5 months, P<0.006). During median (range) follow-up period of 73 (1-150) months, 29 (58%) remained in continuous complete remission, 16 (32%) remained in complete remission. CONCLUSIONS: Infants with AIHA have a more severe presentation. Monospecific DAT and a thorough search for an underlying cause help optimize therapy in most patients of AIHA.
OBJECTIVE: To analyze clinical and laboratory parameters, and treatment outcomes of children with autoimmune hemolytic anemia (AIHA). METHODS: Retrospective analysis of 50 children aged 0-18 years. Monospecific direct antiglobulin test (DAT) and investigations for secondary causes were performed. Disease status was categorized based on Cerevance criteria. RESULTS: Median (range) age at diagnosis was 36 (1.5-204) months. AIHA was categorized as cold (IgM+,C3+/cold agglutinin+) (35%), warm (IgG+ with/without C3+) (28%), mixed (IgG+, IgM+, C3+) (15%) and paroxysmal cold hemoglobinuria (4%). Primary AIHA accounted for 64% cases. Treatment modalities included steroid (66%), intravenous immunoglobulin (IVIg) (4%), steroid+IVIg (4%), and steroid+rituximab (4%). Treatment duration was longer for secondary AIHA than primary (11 vs 6.6 months, P<0.02) and in patients needing polytherapy than steroids only (13.3 vs 7.5 months, P<0.006). During median (range) follow-up period of 73 (1-150) months, 29 (58%) remained in continuous complete remission, 16 (32%) remained in complete remission. CONCLUSIONS: Infants with AIHA have a more severe presentation. Monospecific DAT and a thorough search for an underlying cause help optimize therapy in most patients of AIHA.
Authors: Rahul Naithani; Neerja Agrawal; M Mahapatra; Rajat Kumar; H P Pati; V P Choudhry Journal: Pediatr Hematol Oncol Date: 2007-06 Impact factor: 1.969