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1.
Pan Afr Med J ; 42: 201, 2022.
Artigo em Francês | MEDLINE | ID: mdl-36284568

RESUMO

The combination of erythroblastopenia and primary myelofibrosis is very rare. We here report the unusual case of a 76-year-old Moroccan patient followed up since 2018 for idiopathic erythroblastopenia, initially treated with corticotherapy and then with ciclosporin. Two years later, the patient reported bone pain with splenomegaly. Assessment including myelogram, bone marrow biopsy and molecular biology showed myelofibrosis. Etiological assessment of myelofibrosis was negative confirming its primitive nature. The patient received ruxolitinib with transfusion support. Patient´s outcome was favorable and marked by improvement of general condition, splenomegaly and transfusion rate. The association between erythroblastopenia and myeloproliferative disorder is exceptional and only a few cases have been reported in the literature.


Assuntos
Mielofibrose Primária , Aplasia Pura de Série Vermelha , Humanos , Idoso , Mielofibrose Primária/complicações , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/terapia , Esplenomegalia/etiologia , Ciclosporina , Baço , Medula Óssea/patologia , Transtornos da Insuficiência da Medula Óssea
2.
Tunis Med ; 94(8-9): 541-545, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-28603827

RESUMO

BACKGROUND: Prognostic scores are an indispensable tool in the management of myelodysplastic syndromes. AIM: Identify prognostic factors influencing overall survival of patients with myelodysplastic syndromes. METHODS: We included all patients with myelodysplastic syndromes treated in the clinical hematology department of the military hospital of Rabat (Morocco). RESULTS: Seventy-four patients were included. The sex ratio [M / F] was 1.8. The median age was 65.5 years. The median follow-up was 45 months. The karyotype prognostic was good in 74%. In univariate analysis, prognostic factors influencing overall survival are: Age (p = 0.024), karyotype (p = 0.033), rate of bone marrow blasts (p = 0.002), transfusion dependence (p = 0.002), number of cytopenias (p = 0.001), hemoglobin (p = 0.009) and neutrophil count (p = 0.003). In multivariate analysis the only prognostic factor that influenced overall survival was the karyotype (p = 0.036). Transfusion dependence (p = 0.072) and the rate of marrow blasts (p = 0.107) was close to significance. The WHO Prognostic Scoring System (WPSS), the International Prognostic Scoring System (IPSS) and the Revised International Prognostic Scoring System (RIPSS) influence overall survival with a statistical significance (p <0.001). CONCLUSION: Our study confirms the prognostic value of the karyotype and validated the WPSS, the IPSS and the RIPSS scores as significant predictors of overall survival in Moroccan patients with myelodysplastic syndromes.


Assuntos
Síndromes Mielodisplásicas/mortalidade , Idoso , Análise de Variância , Transfusão de Sangue , Feminino , Seguimentos , Humanos , Cariótipo , Masculino , Marrocos , Síndromes Mielodisplásicas/patologia , Síndromes Mielodisplásicas/terapia , Prognóstico , Taxa de Sobrevida
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