Myelodysplastic syndromes (MDS) include a group of heterogeneous hematological disorders with a variable risk of leukemic evolution and short survival. Around 40-50% of patients show abnormal karyotypes that are mostly characterized by monosomies or deletions. Cytogenetic findings are an independent prognostic factor and the International prognostic scoring system (IPSS) differentiates three cytogenetic categories, despite the Intermediate one being heterogeneous. The aim of this study, including 421 Argentinean patients with primary MDS, is to characterize the cytogenetic profile, to test its prognostic value and to compare partial and monosomal karyotypes against other cytogenetic findings. An abnormal karyotype (median survival: 26 months) was observed in 176 patients. The presence of complex karyotypes, number of alterations, and the IPSS cytogenetic groups showed significant differences for predicting outcome. Behavior of patients with isolated deletions (median survival: 49 months) did not differ from those with normal karyotype (56 months, P 5 0.654) or Good prognostic findings (43 months, P 5 0.371). However, a worse prognosis was observed when another alteration was added (31 months, P 5 0.043). Karyotypes with autosomal monosomies (median survival: 16 months) had a prognostic impact similar to other Poor cytogenetic findings (17 months, P 5 0.626). In our population classified according to French-American-British (FAB) or World Health Organization (WHO), this new categorization of cytogenetic abnormalities, recognizing three different risk groups, showed an independent prognostic impact and a better discriminating power than the IPSS categories. It can be concluded that all isolate deletions (excluding 7q-) are good prognostic findings and all monosomies (excluding Y chromosome loss) are bad indicators. Am. J. Hematol. 86:540-545, 2011. V
This is the first paper to report the incidence density of ATTRwt, AL and AA amyloidosis in Latin America. Our results are consistent with other studies from other regions. Although systemic amyloidosis is a rare disease, it is a major health problem because of its morbi-mortality.
This multicenter, open-label study evaluated the efficacy and safety of decitabine in patients from Argentina and South Korea with myelodysplastic syndromes or chronic myelomonocytic leukemia. Of 106 patients who received decitabine 20 mg/m(2) intravenously over 1 h once daily for 5 days in 4-week cycles, 99 patients were evaluable after receiving at least two cycles. The overall improvement rate was 35% (19% complete response +4% marrow complete response +4% partial response +8% hematologic improvement). Overall survival at 2 years was 71%. Treatment-related adverse events included febrile neutropenia, thrombocytopenia and bleeding, asthenia, fatigue, and eosinophilia. After complete response (CR), three patients received an allogeneic stem cell transplant. Four patients who relapsed after CR responded to decitabine retreatment. Acute myelogenous leukemia developed during follow-up in 21% of patients. Decitabine in a 5-day outpatient administration schedule was effective and well tolerated in typical clinical practice settings in South America and Asia.
Objetivos: Determinar la prevalencia de amiloidosis cardíaca en pacientes con amiloidosis sistémica. Comparar la supervivencia según sea que presenten o no compromiso cardíaco. Métodos: Cohorte retrospectiva de pacientes con amiloidosis sistémica del Registro Institucional de Amiloidosis del Hospital Italiano de Buenos Aires, entre 2010 y 2019. Se consideró como compromiso cardíaco la presencia de síntomas o imágenes consistentes con amiloidosis no explicado por otras causas. Se evaluó la muerte por todas las causas. Se calculó la sobrevida mediante Kaplan-Meier. Los factores relacionados con mortalidad se evaluaron con un modelo de regresión de Cox. Se evaluó el trasplante cardíaco en un modelo de regresión de riesgo competitivo. Resultados: La prevalencia de compromiso cardíaco fue del 63%. La incidencia de muerte fue de 14/1,000 personas-meses para el grupo con compromiso cardíaco y de 5/1,000 personas-meses para los pacientes sin compromiso. Los pacientes con compromiso cardíaco tuvieron una sobrevida global a los cinco años de 44% contra 67% en los que no tenían compromiso (p = 0.02). El HR crudo para el compromiso cardíaco fue de 2.09 (p = 0.02). La edad mostró un HR ajustado de 1.06 (p <0.01). El modelo de regresión de riesgos competitivos estableció un sub-HR de 1.86 (IC95%, 0.99-3.49; p = 0.05). Conclusiones: El compromiso cardíaco es un factor pronóstico importante en pacientes con amiloidosis.
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