Heterogeneity of acquired idiopathic sideroblastic anaemia (AISA)
| Autor: | Jacques Gardais, Jean-Louis Bremond, Marie-Paule Callat, Françoise Accard, Marie Bizet, Jean-Etienne Goasguen, Richard Garand, Eric Tron de Bouchony |
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| Rok vydání: | 1992 |
| Předmět: |
Cancer Research
medicine.medical_specialty Pediatrics Anemia Sideroblastic anaemia Gastroenterology Sideroblastic anemia Internal medicine medicine Humans Refractory anaemia Aged Idiopathic sideroblastic anaemia Aged 80 and over business.industry Myelodysplastic syndromes Anemia Refractory Hematology Middle Aged Prognosis medicine.disease Anemia Sideroblastic Oncology Sideroblastosis Outcome data business |
| Zdroj: | Leukemia Research. 16:463-468 |
| ISSN: | 0145-2126 |
| DOI: | 10.1016/0145-2126(92)90171-3 |
| Popis: | Clinical, haematological and outcome data were studied in 84 patients with acquired idiopathic sideroblastic anaemia (AISA) from a registry of 613 consecutive myelodysplastic syndromes (MDS) recorded by five institutions in western France. Two groups could be identified and compared: 'pure' erythroblastic AISA (AISA-E: 59 pts), and AISA with myelodysplastic features, i.e. dysgranulo and/or dysmegakaryopoiesis (AISA-M: 25 pts). Results were also compared to those of a series of 71 cases of refractory anaemia without sideroblastosis (RA) carried out from the same registry. Dyserythropoiesis was present in 90% of all AISA subtypes, dysgranulopoiesis in 88% of the AISA-M cases; dysmegakaryopoiesis was observed in 44% of AISA-M. Ten patients with both forms of AISA showed high platelet counts. These cases appeared particular in that four of them were associated with a splenomegaly and/or a hyperleucocytosis. They had to be distinguished from myeloproliferative syndromes. Outcome comparison of AISA-E with AISA-M showed a significant discrepancy of survival duration (60 vs 38 months respectively). Progression towards refractory anaemia with excess of blasts or acute leukaemia, was significantly higher for AISA-M than for AISA-E. The risk of transformation increased to 24% for the AISA-M group similarly to those of RA patients (17%). We conclude that AISA must be divided into two categories, 'pure' AISA and AISA-M, because survival duration and risk of transformation are different. |
| Databáze: | OpenAIRE |
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