J Serrano1. 1. Departamento de Hematología y Hemoterapia, Fundación Jiménez Diaz (Universidad Autónoma), Madrid.
Abstract
PURPOSE: To propose a new aetiologic classification of the patients suffering from autoimmune haemolytic anaemia (AIHA). MATERIAL AND METHODS: Two-hundred cases of AIHA studied and followed-up between 1970 and 1989 are presented. From an aetiological and pathogenetic standpoint the disease was classified into 4 groups: (1) idiopathic, (2) secondary, (3) associated and (4) accompanying. RESULTS: The percentage of the different AIHA types, in accordance with the antibodies behaviour, was: (1) warm-reacting AIHA, 74.5%; (2) cold-reacting, 19%; (3) combined or mixed pattern, 6.5%. The immunoglobulins fixed on red cell surface and the specificity of the antibodies found corresponded to those reported in the literature. No treatment was needed in 27.5% of the cases. Corticosteroids were the therapy used in virtually all the cases. Corticosteroids were favourable effect could be appreciated in about 80% of the patients treated. Splenectomy had to be performed in 13.1% of the patients; 47.4% of them attained complete remission while 56.2 failed to respond. Nonsteroid immunosuppressive agents were used in 16.5% of the cases, with 50% of total or partial responses. There are striking prognostic-evolutive differences when this study is viewed from the patients or the AIHA standpoints. Thus, the mortality reached 57.5% of the cases, but in only 10% it was related with AIHA, and something similar could be said about remissions. CONCLUSIONS: The analysis of this series allows one to establish: (1) four aetiopathogenetic groups of AIHA; (2) a high percentage of cases related with severe underlying diseases; (3) over 50% incidence in people over 50 years of age, and (4) natural ageing of the series is appreciated after a long follow-up (1-20 years).
PURPOSE: To propose a new aetiologic classification of the patients suffering from autoimmune haemolytic anaemia (AIHA). MATERIAL AND METHODS: Two-hundred cases of AIHA studied and followed-up between 1970 and 1989 are presented. From an aetiological and pathogenetic standpoint the disease was classified into 4 groups: (1) idiopathic, (2) secondary, (3) associated and (4) accompanying. RESULTS: The percentage of the different AIHA types, in accordance with the antibodies behaviour, was: (1) warm-reacting AIHA, 74.5%; (2) cold-reacting, 19%; (3) combined or mixed pattern, 6.5%. The immunoglobulins fixed on red cell surface and the specificity of the antibodies found corresponded to those reported in the literature. No treatment was needed in 27.5% of the cases. Corticosteroids were the therapy used in virtually all the cases. Corticosteroids were favourable effect could be appreciated in about 80% of the patients treated. Splenectomy had to be performed in 13.1% of the patients; 47.4% of them attained complete remission while 56.2 failed to respond. Nonsteroid immunosuppressive agents were used in 16.5% of the cases, with 50% of total or partial responses. There are striking prognostic-evolutive differences when this study is viewed from the patients or the AIHA standpoints. Thus, the mortality reached 57.5% of the cases, but in only 10% it was related with AIHA, and something similar could be said about remissions. CONCLUSIONS: The analysis of this series allows one to establish: (1) four aetiopathogenetic groups of AIHA; (2) a high percentage of cases related with severe underlying diseases; (3) over 50% incidence in people over 50 years of age, and (4) natural ageing of the series is appreciated after a long follow-up (1-20 years).