RESUMO
Our objective was to recognize the association of autoimmune diseases (AD) in patients with myelodysplastic syndromes (MDS) and understand how this association could affect prognosis and management of both diseases. We describe our cohort of 10 patients and 34 patients reported in the English literature in addition to ten cohort studies. Interestingly, four cases showed improvement in AD after 5-azacitidine treatment. The mechanism(s) of the association between AD and MDS are discussed. Treatment could be targeted against AD, MDS or both, though based on recent reports, treating MDS with hypomethylating agents alone could improve the associated AD.
Assuntos
Doenças Autoimunes/diagnóstico , Síndromes Mielodisplásicas/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antimetabólitos Antineoplásicos/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Azacitidina/uso terapêutico , Feminino , Humanos , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/tratamento farmacológico , Prognóstico , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVES: Pediatric embryonal heterologous rhabdomyosarcoma of the cervix is a rare tumor. METHODS: We present an interesting clinico-pathologic situation of two sisters presenting with pediatric embryonal heterologous rhabdomyosarcoma of the cervix. RESULTS: Pediatric embryonal heterologous rhabdomyosarcomas of the cervix are relatively uncommon. After a Pubmed search from 1952 to present, to our knowledge, this is the only report involving sisters presenting with this disease. Treatment has been extrapolated from collaborative groups such as Intergroup Rhabdomyosarcoma Study (IRS) Group so that optimal management may be achieved. CONCLUSIONS: We urge continued reporting of these rare tumors to enhance understanding if there may be a genetic component associated with them.
