Effect of therapy-related acute myeloid leukemia on the outcome of patients with acute myeloid leukemia.

Therapy-related acute myeloid leukemia (t-AML) is a rare and almost always fatal late side effect of antineoplastic treatment involving chemotherapy, radiotherapy or the two combined. The present retrospective study intended to characterize t-AML patients that were diagnosed and treated in a single referral to an oncological institution in North Portugal. Over the past 10 years, 231 cases of AML were diagnosed and treated at the Portuguese Institute of Oncology of Porto, of which 38 t-AML cases were identified. Data regarding the patient demographics, primary diagnosis and treatment, age at onset of therapy-related myeloid neoplasm, latency time of the neoplasm, cytogenetic characteristics, AML therapy and outcome were collected from medical records. A previous diagnosis with solid tumors was present in 28 patients, and 10 patients possessed a history of hematological conditions, all a lymphoproliferative disorder. Breast cancer was the most frequent solid tumor identified (39.5% of all solid tumors diagnosed). The mean latency time was 3 years. In the present study, t-AML patients were older (P<0.001) and more frequently carried cytogenetic abnormalities (P=0.009) compared with de novo AML patients. The overall survival time was observed to be significantly poorer among individuals with t-AML (P<0.001). However, in younger patients (age, <50 years) there was no difference between the overall survival time of patients with t-AML and those with de novo AML (P=0.983). Additionally, patients with promyelocytic leukemia possess a good prognosis, even when AML occurs as a secondary event (P=0.98). To the best of our knowledge, the present study is the first to evaluate t-AML in Portugal and the results are consistent with the data published previously in other populations. The present study concludes that although t-AML demonstrates a poor prognosis, this is not observed among younger patients or promyelocytic leukemia patients.

Enteropathy-associated T cell lymphoma as a complication of silent celiac disease.

Celiac disease is an autoimmune disorder in which a genetic predisposition and the ingestion of wheat gluten triggers a deleterious immune response. This response is complex and may lead to manifestations other than enteropathyha: hepatitis, dermatitis and neuropathy. There is higher risk for neoplasia. We observed an atypical case, corresponding to a 69-year old female presenting with complicated celiac disease. The patient was referred following the histological examination of an enterectomy specimen, which unexpectedly revealed an enteropathy-associated T cell lymphoma in a background of celiac disease. Patient's previous medical history comprised several abdominal surgical procedures, without other prior symptoms suggestive of celiac disease. Indeed, the patient was obese and no signs of malabsortion were apparent. This case draws our attention to clinically silent celiac disease, which represents a diagnostic challenge. Thus, this should be kept in mind whenever a patient presents with abdominal relapsing complications, otherwise unexplained.

[Adulthood Langerhans cell histiocytosis: experience of two Portuguese hospitals]. / Histiocitose de Largerhans no Adulto: Experiência de Dois Hospitais Portugueses.

INTRODUCTION: Langerhans cell histiocytosis is a heterogeneous disease, more frequently diagnosed during childhood. Between 1/2001 and 12/2013, 20 adult patients were admitted at both Hospitals. This work aimed at characterizing this population. MATERIAL AND METHODS: Retrospective study, review of clinical records. RESULTS: 16 patients were eligible to analysis. The median age at diagnosis was 34 years (15-48); 10 males and 6 females. The referral motive was: respiratory complaints - 37.5%; bone changes - 37.5%; dental complaints - 25%; constitutional symptoms - 19%; mucocutaneous lesions - 6% and one patient (6%) was accidentally diagnosed after a thyroidectomy. The tissue of histological diagnosis was: bone - 50%; pulmonary tissue - 37.5%; liver, genital mucosa and thyroid - 6%, respectively. Staging was: single organ involvement (uni/multifocal) - 69% and multisystem disease in 31%. Clinical re-evaluation of these cases is being done at the moment. The median follow up was 5 years (1 month - 11 years) and the overall survival was 92%. Currently 19% are alive without signs of disease; 44% are alive with disease; 25% are under treatment and 12% died. DISCUSSION: These results agree with published literature. Considering the actual guidelines 56% patients were incompletely staged, which probably lead to suboptimal treatment. There is heterogeneity of clinical procedures aiming at staging and treatment of these patients. CONCLUSION: The diagnosis of adulthood Langerhans cell histiocytosis is difficult considering the diversity of clinical behavior. Frequently this also leads to diagnosis delay. Prospective international clinical trials enrolling adult patients are important.

IntroduçÉo: A histiocitose de células de Langerhans é uma doença heterogénea e mais frequente em crianças. Entre 1/2001 e 12/2013 admitimos 20 doentes com HCL nas duas instituições. O objectivo deste trabalho foi caracterizar esta populaçÉo, avaliando as formas de apresentaçÉo, o estadiamento e tratamento.Material e Métodos: Estudo retrospectivo; consulta do processo clínico.Resultados: Dos 16 doentes analisáveis verificamos uma mediana de idade 34 anos (15-48), 10 mulheres e 6 homens. Os motivos que determinaram a referenciaçÉo dos doentes foram: queixas respiratórias em 37,5%; alterações ósseas em 37,5%; queixas dentárias em 25%; sintomas constitucionais em 19%; lesões mucocut'neas em 6% e outro foi um achado histológico inesperado após tiroidectomia. O diagnóstico histológico foi obtido em: osso em 50%; pulmÉo em 37,5%; fígado, mucosa vulvar e peça de tiroidectomia em 6%, respectivamente. O estadiamento assumido na prática clínica foi: envolvimento de órgÉo único (uni/multifocal) em 69% e doença multissistémica em 31%. A mediana de seguimento foi cinco anos (dois meses-11 anos) e a sobrevivência global 92%. Actualmente: 19% estÉo vivos sem doença; 44% estÉo vivos com doença; 25% estÉo em tratamento e 12% morreram.DiscussÉo: Estes resultados estÉo de acordo com a literatura. No entanto, segundo as recomendações actuais consideramos que 56% doentes efectuaram estudo complementar incompleto condicionando subestadiamento e provavelmente subtratamento. Verifica-se heterogeneidade de procedimentos no estadiamento e tratamento.ConclusÉo: Frequentemente há dificuldades e atraso no diagnóstico desta entidade clínica. SÉo importantes estudos prospectivos internacionais na populaçÉo adulta.