[Treatment of systemic AL amyloidosis: about 25 cases].

Autor: Eddou H; Service de Médecine Interne, Hôpital Militaire Moulay Ismail Méknes, Maroc., Zinebi A; Service de Médecine Interne, Hôpital Militaire Moulay Ismail Méknes, Maroc., Maaroufi HE; Service d'Hématologie Clinique, Hôpital Militaire d'Instruction Mohammed V, Rabat, Maroc., Moudden MK; Service de Médecine Interne, Hôpital Militaire Moulay Ismail Méknes, Maroc., Doghmi K; Service d'Hématologie Clinique, Hôpital Militaire d'Instruction Mohammed V, Rabat, Maroc., Mikdame M; Service d'Hématologie Clinique, Hôpital Militaire d'Instruction Mohammed V, Rabat, Maroc., Baaj ME; Service de Médecine Interne, Hôpital Militaire Moulay Ismail Méknes, Maroc.
Jazyk: francouzština
Zdroj: The Pan African medical journal [Pan Afr Med J] 2017 Oct 19; Vol. 28, pp. 160. Date of Electronic Publication: 2017 Oct 19 (Print Publication: 2017).
DOI: 10.11604/pamj.2017.28.160.11885
Abstrakt: Primarye systemic AL amyloidosis is a rare hematologic disorder. The majority of the therapeutic guidelines are based on phase II studies or on retrospective comparisons and case series. Our study aimed to describe all the cases of primary AL amyloidosis reported in 2 military hospitals and to make a comparison between standard melphalan-dexamethasone protocol and new agents in first-line treatment of patients with this disease. We conducted a retrospective, descriptive and multicentric study of all patients with AL amyloidosis whose data were collected during the period July 2009-June 2016. Twenty five patients were enrolled in the study (12 patients treated with melphalan-dexamethasone and 13 with bortezomib-based protocol or lenalidomide-based protocol). There was no significant difference in the epidemiological, clinical and prognostic features between the 2 groups. After a median follow up of 40 months, median overall survival was 54 months in the melphalan-dexamethasone-treated group and 60 months in the new therapies-treated group (P = 0.98). Progression-free survival was 18 months in the standard treatment group vs 11 months in the 2nd group (p = 0.08). In our small case series we haven't found a superiority of the new therapies compared to the standard protocol. This result should be confirmed by a true prospective study, mainly because of the cost of these new molecules that are not always accessible, especially in developing countries.
Databáze: MEDLINE