Abstract
Introduction: The aim of this work is to establish an epidemiological approach of aplastic anemia (AA) in Algeria, to identify the different therapeutic strategy and the patients outcomes.
Material and methods: This is a retrospective multi-center epidemiological study over 10 years (from 01 January 2007 to 31 December 2016). The source of information is represented by the medical files and the consultation sheets. The collection of information is ensured by the fact sheets established and distributed to the departments Hematology and Pediatrics at the national level.
Results: A total of 844 cards were received, concerning 844 patients including 746 adults and 94 children. The overall incidence of the disease is 0.21, it varies from 0.16 to 0.28 / 100000 inhabitants / year depending on the year. The average age is 34.7 years with extremes ranging from 1 month to 91 years, the sex ratio (M / F) is 1 (420/424). In adults there is a slight male predominance: sex ratio is 1.11 (421/378). AA is acquired in 806 patients (95.4%) and congenital in 38 patients (4.6%). Among the acquired AA: idiopathic = 694 (86.1%), toxic = 18 (2.23%), viral: 17 (2.1%): HBV = 13, HCV = 3, HIV = 1, drug = 13 (1.6%), gestational = 9, (1.1%), hematopoietic tuberculosis = 1. The presence of an HPN clone in 55 out of 261 patients studied was 21%, associated with a dysimmunitary disease = 5. In congenital AM, Fanconi anemia = 35, Blackfand-Diamond = 2, congenital dyskeratosis = 1. The prognostic classification according to the Camitta criteria and the EBMT criterion: out of 540 evaluable records found: very severe = 83 (15.3%), severe = 291 (53.8%), moderate = 166 (30.7%). CsA-SAL reference immunosuppressive therapy was instituted in only 49 pts (13.1%) with severe and very severe forms. It resulted in a hematologic response in 18 patients (36.5%), 15 patients died and 16 patients were probably lost to follow-up. Three hundred and twenty-seven pts (38.7%) received allogeneic hematopoietic stem cell transplant at CPMC (n = 316) and EHU (n = 11); = 277), Fanconi anemia (n = 44), amgacaryocytosis in its aplastic form (n = 3), erythroblastopenia in aplastic form (n = 2), untagged constitutional medullary aplasia (n = 1). As of 31/12/2016: 344 pts are alive, 208 died, 194 lost sight of and 98 pts whose fate is not specified on the cards.
Conclusion: AA is a rare , the incidence is 0.2 / 100000 inhabitants / year. It affects young adults with a slight male predominance in adults, idiopathic forms are in the foreground and severe forms predominat
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
